VANCOUVER—Adjunctive treatment with everolimus significantly reduces seizure frequency in patients with treatment-resistant seizures associated with tuberous sclerosis complex (TSC), according to a trial described at the 68th Annual Meeting of the American Academy of Neurology. The drug appears not to be associated with unexpected adverse events in this indication.

“Results from this [study] … suggest that one can use everolimus as an antiepilepsy therapy, which treats the underlying cause of seizures,” said Jacqueline A. French, MD, Professor of Neurology at NYU Langone Comprehensive Epilepsy Center in New York City. “Whether it in fact is also disease-modifying for epilepsy and [whether] continued therapy will produce a continued improvement remain to be seen.”

Comparing Two Drug Concentrations With Placebo

She and her colleagues conducted a randomized, double-blind, placebo-controlled trial of everolimus in patients with TSC. Eligible participants were between ages 2 and 65 and had at least 16 treatment-resistant seizures in the eight-week baseline phase. Participants also had to be taking one to three antiepileptic drugs at stable concentrations for four weeks before enrollment, as well as through the study’s eight-week baseline phase.

Participants were randomized to adjunctive treatment with placebo, a low serum concentration of everolimus (ie, 3 to 7 ng/mL), or a high serum concentration of everolimus (ie, 9 to 15 ng/mL). Concentrations were titrated during the first eight weeks to achieve stable serum concentrations of treatment. A 12-week maintenance phase followed. Seizures were counted during the titration and maintenance phases. At the end of the maintenance phase, patients had the option of continuing in the extension phase of the trial, which is ongoing. The primary end points were percentage seizure reduction and 50% responder rate.

In all, 119 patients received placebo, 117 received low-concentration everolimus, and 130 received high-concentration everolimus. The treatment arms were evenly matched, in terms of age and gender. Approximately half of participants were female. Five patients in the placebo arm discontinued the study, compared with seven in the low-concentration group and eight in the high-concentration group. Five discontinuations in the low-concentration group resulted from adverse events, including stomatitis, anxiety, immunodeficiency, and pyrexia. Four discontinuations in the high-concentration group resulted from adverse events, including mouth ulceration, neutropenia, pneumonia, and stomatitis. Most patients chose to enter the extension phase.

The study population was highly resistant to treatment. Approximately half of participants had failed more than six antiepileptic drugs before enrollment, and about half were taking three antiepileptic drugs at enrollment. Median baseline seizure frequency per 28 days was higher than the requirement for enrollment. Several participants were receiving treatment with vagus nerve stimulation or the ketogenic diet. Patients had various seizure types and syndromes.

Treatment Yielded Clinically Meaningful Improvement

The median percent seizure reduction was significantly greater with low-concentration everolimus (29.3%) and high-concentration everolimus (39.6%), compared with placebo (14.9%). The 50% responder rate also was significantly greater with low-concentration everolimus (28.2%) and high-concentration everolimus (40%), compared with placebo (15.1%).

Approximately 5% of participants in the low-concentration group became seizure-free, along with 3.8% of participants in the high-concentration group versus 0.8% for placebo. Patients receiving placebo had a greater likelihood of seizure worsening, compared with patients receiving everolimus. Participants who received everolimus were more likely to improve, compared with controls. Approximately 15% of the patients in the high-concentration group had a 75% to 100% improvement in seizures.

Approximately 17% of participants in the low-concentration group and 23% of participants in the high-concentration group had severe or life-threatening adverse events versus 10.9% on placebo. The most common adverse events were stomatitis, diarrhea, mouth ulceration, nasal pharyngitis, and upper respiratory tract infection.

“This study demonstrated a statistically significant and clinically meaningful reduction in seizure frequency in patients with treatment-resistant seizures associated with TSC,” said Dr. French. “As we learn more, we find that there are more causes of epilepsy that relate to abnormalities in the mTOR pathway, including … some focal cortical dysplasias,” she continued. Although Dr. French was discussing a cohort of patients with tuberous sclerosis, “it could be that this pathway and interventions in this pathway will be very important for other common forms of epilepsy as well.”

—Erik Greb

VANCOUVER—Adjunctive treatment with everolimus significantly reduces seizure frequency in patients with treatment-resistant seizures associated with tuberous sclerosis complex (TSC), according to a trial described at the 68th Annual Meeting of the American Academy of Neurology. The drug appears not to be associated with unexpected adverse events in this indication.

“Results from this [study] … suggest that one can use everolimus as an antiepilepsy therapy, which treats the underlying cause of seizures,” said Jacqueline A. French, MD, Professor of Neurology at NYU Langone Comprehensive Epilepsy Center in New York City. “Whether it in fact is also disease-modifying for epilepsy and [whether] continued therapy will produce a continued improvement remain to be seen.”

Comparing Two Drug Concentrations With Placebo

She and her colleagues conducted a randomized, double-blind, placebo-controlled trial of everolimus in patients with TSC. Eligible participants were between ages 2 and 65 and had at least 16 treatment-resistant seizures in the eight-week baseline phase. Participants also had to be taking one to three antiepileptic drugs at stable concentrations for four weeks before enrollment, as well as through the study’s eight-week baseline phase.

Participants were randomized to adjunctive treatment with placebo, a low serum concentration of everolimus (ie, 3 to 7 ng/mL), or a high serum concentration of everolimus (ie, 9 to 15 ng/mL). Concentrations were titrated during the first eight weeks to achieve stable serum concentrations of treatment. A 12-week maintenance phase followed. Seizures were counted during the titration and maintenance phases. At the end of the maintenance phase, patients had the option of continuing in the extension phase of the trial, which is ongoing. The primary end points were percentage seizure reduction and 50% responder rate.

In all, 119 patients received placebo, 117 received low-concentration everolimus, and 130 received high-concentration everolimus. The treatment arms were evenly matched, in terms of age and gender. Approximately half of participants were female. Five patients in the placebo arm discontinued the study, compared with seven in the low-concentration group and eight in the high-concentration group. Five discontinuations in the low-concentration group resulted from adverse events, including stomatitis, anxiety, immunodeficiency, and pyrexia. Four discontinuations in the high-concentration group resulted from adverse events, including mouth ulceration, neutropenia, pneumonia, and stomatitis. Most patients chose to enter the extension phase.

The study population was highly resistant to treatment. Approximately half of participants had failed more than six antiepileptic drugs before enrollment, and about half were taking three antiepileptic drugs at enrollment. Median baseline seizure frequency per 28 days was higher than the requirement for enrollment. Several participants were receiving treatment with vagus nerve stimulation or the ketogenic diet. Patients had various seizure types and syndromes.

Treatment Yielded Clinically Meaningful Improvement

The median percent seizure reduction was significantly greater with low-concentration everolimus (29.3%) and high-concentration everolimus (39.6%), compared with placebo (14.9%). The 50% responder rate also was significantly greater with low-concentration everolimus (28.2%) and high-concentration everolimus (40%), compared with placebo (15.1%).

Approximately 5% of participants in the low-concentration group became seizure-free, along with 3.8% of participants in the high-concentration group versus 0.8% for placebo. Patients receiving placebo had a greater likelihood of seizure worsening, compared with patients receiving everolimus. Participants who received everolimus were more likely to improve, compared with controls. Approximately 15% of the patients in the high-concentration group had a 75% to 100% improvement in seizures.

Approximately 17% of participants in the low-concentration group and 23% of participants in the high-concentration group had severe or life-threatening adverse events versus 10.9% on placebo. The most common adverse events were stomatitis, diarrhea, mouth ulceration, nasal pharyngitis, and upper respiratory tract infection.

“This study demonstrated a statistically significant and clinically meaningful reduction in seizure frequency in patients with treatment-resistant seizures associated with TSC,” said Dr. French. “As we learn more, we find that there are more causes of epilepsy that relate to abnormalities in the mTOR pathway, including … some focal cortical dysplasias,” she continued. Although Dr. French was discussing a cohort of patients with tuberous sclerosis, “it could be that this pathway and interventions in this pathway will be very important for other common forms of epilepsy as well.”

—Erik Greb

VANCOUVER—Adjunctive treatment with everolimus significantly reduces seizure frequency in patients with treatment-resistant seizures associated with tuberous sclerosis complex (TSC), according to a trial described at the 68th Annual Meeting of the American Academy of Neurology. The drug appears not to be associated with unexpected adverse events in this indication.

“Results from this [study] … suggest that one can use everolimus as an antiepilepsy therapy, which treats the underlying cause of seizures,” said Jacqueline A. French, MD, Professor of Neurology at NYU Langone Comprehensive Epilepsy Center in New York City. “Whether it in fact is also disease-modifying for epilepsy and [whether] continued therapy will produce a continued improvement remain to be seen.”

Comparing Two Drug Concentrations With Placebo

She and her colleagues conducted a randomized, double-blind, placebo-controlled trial of everolimus in patients with TSC. Eligible participants were between ages 2 and 65 and had at least 16 treatment-resistant seizures in the eight-week baseline phase. Participants also had to be taking one to three antiepileptic drugs at stable concentrations for four weeks before enrollment, as well as through the study’s eight-week baseline phase.

Participants were randomized to adjunctive treatment with placebo, a low serum concentration of everolimus (ie, 3 to 7 ng/mL), or a high serum concentration of everolimus (ie, 9 to 15 ng/mL). Concentrations were titrated during the first eight weeks to achieve stable serum concentrations of treatment. A 12-week maintenance phase followed. Seizures were counted during the titration and maintenance phases. At the end of the maintenance phase, patients had the option of continuing in the extension phase of the trial, which is ongoing. The primary end points were percentage seizure reduction and 50% responder rate.

In all, 119 patients received placebo, 117 received low-concentration everolimus, and 130 received high-concentration everolimus. The treatment arms were evenly matched, in terms of age and gender. Approximately half of participants were female. Five patients in the placebo arm discontinued the study, compared with seven in the low-concentration group and eight in the high-concentration group. Five discontinuations in the low-concentration group resulted from adverse events, including stomatitis, anxiety, immunodeficiency, and pyrexia. Four discontinuations in the high-concentration group resulted from adverse events, including mouth ulceration, neutropenia, pneumonia, and stomatitis. Most patients chose to enter the extension phase.

The study population was highly resistant to treatment. Approximately half of participants had failed more than six antiepileptic drugs before enrollment, and about half were taking three antiepileptic drugs at enrollment. Median baseline seizure frequency per 28 days was higher than the requirement for enrollment. Several participants were receiving treatment with vagus nerve stimulation or the ketogenic diet. Patients had various seizure types and syndromes.

Treatment Yielded Clinically Meaningful Improvement

The median percent seizure reduction was significantly greater with low-concentration everolimus (29.3%) and high-concentration everolimus (39.6%), compared with placebo (14.9%). The 50% responder rate also was significantly greater with low-concentration everolimus (28.2%) and high-concentration everolimus (40%), compared with placebo (15.1%).

Approximately 5% of participants in the low-concentration group became seizure-free, along with 3.8% of participants in the high-concentration group versus 0.8% for placebo. Patients receiving placebo had a greater likelihood of seizure worsening, compared with patients receiving everolimus. Participants who received everolimus were more likely to improve, compared with controls. Approximately 15% of the patients in the high-concentration group had a 75% to 100% improvement in seizures.

Approximately 17% of participants in the low-concentration group and 23% of participants in the high-concentration group had severe or life-threatening adverse events versus 10.9% on placebo. The most common adverse events were stomatitis, diarrhea, mouth ulceration, nasal pharyngitis, and upper respiratory tract infection.

“This study demonstrated a statistically significant and clinically meaningful reduction in seizure frequency in patients with treatment-resistant seizures associated with TSC,” said Dr. French. “As we learn more, we find that there are more causes of epilepsy that relate to abnormalities in the mTOR pathway, including … some focal cortical dysplasias,” she continued. Although Dr. French was discussing a cohort of patients with tuberous sclerosis, “it could be that this pathway and interventions in this pathway will be very important for other common forms of epilepsy as well.”

—Erik Greb

In parts 1 and 2 of this series on atopic dermatitis (AD),^1,2 the current putative pathogenesis, scoring systems for severity grading, and epidemiology were reviewed. Part 3 reviews the differential diagnosis, with an emphasis on the difficulty of differentiation from some rare but notable illnesses, as well as the recently expanding data on comorbidities that identify AD as a multisystem disorder with widespread health implications for the patient.

Differential Diagnosis for Pediatric AD

The differential diagnosis for pediatric AD includes chronic dermatoses (eg, seborrheic dermatitis, psoriasis), congenital disorders (eg, Netherton syndrome), malignant diseases (eg, cutaneous T-cell lymphoma [CTCL]), immunodeficiencies, infections, and metabolic disorders.³ Netherton syndrome must be ruled out to prevent extensive drug absorption when treating with topical calcineurin inhibitors (TCIs).⁴ Due to the presence of bamboo hairs in these patients, a hair mount may aid in the diagnosis of Netherton syndrome. Misdiagnosis of CTCL as AD may complicate the analysis of safety data on TCIs.^4,5 Multiple skin biopsies are essential in cases of suspected CTCL to provide an accurate diagnosis. Biopsy can be considered in AD cases with changing and/or unusual morphology, erythrodermic skin changes, and disease that is poorly responsive to multiple therapeutic modalities.

Comorbidities in Pediatric AD

Psychosocial Comorbidities

Pediatric AD often takes a psychological toll on patients as well as household members. Almost half of children with AD are reported to have a severely impaired quality of life (QOL).⁶ Contributing factors include fatigue, sleep disturbance, activity restriction (eg, inability to participate in sports), and depression.⁷

Chamlin et al⁸ developed the Childhood Atopic Dermatitis Impact Scale (CADIS), a 45-item instrument (refined from a 62-item prototype), to measure QOL in young children with AD and their family members. Responses were evaluated with consideration of 5 domains: symptoms and activity limitations/behaviors in children, as well as family/social function, sleep, and emotions in parents. The top 12 factors that parents found most bothersome about AD included itching/scratching, child’s pain/discomfort, sleep issues, embarrassment or worry about appearance, child’s fussiness/irritability/crying/unhappiness, helplessness/can’t control it/predict it, worry about skin infection, dryness of skin/nonsmooth skin, skin bleeding, worry about damage/scars, stares/comments of strangers and other children, and rashes/redness of skin/discoloration. Parents were asked to respond to items about their emotional health and social functioning, such as “My child’s skin condition has strained my relationship with my spouse or partner,” “My child’s skin condition makes me feel sad or depressed,” and “I am bothered by the reaction of strangers to this skin condition.”⁸

Kiebert et al⁹ found that AD patients had lower scores on the Short Form-36 Health Survey’s vitality, social functioning, and mental health subscales compared to individuals in the general population. The authors noted that anxiety in AD patients is of particular concern, as stress has been found to trigger the itch-scratch cycle, potentially setting off AD flare-ups.⁹ Family impact of AD is aggravated by disease severity. Sleeplessness, relationship stress, and time management can all cause family problems in patients with AD.⁸

In a survey of 3775 older teenagers aged 18 to 19 years (80% response rate out of 4774 prospective participants), 9.7% of participants reported having current AD.¹⁰ Suicidal ideation was higher in those with current AD than those without AD (15.5% vs 9.1%). The prevalence of suicidal ideation rose to 23.8% in those with both AD and itch. Diagnosis of AD (as determined through participant responses to the question, ‘‘Do you have, or have you had eczema?’’) was associated with mental health problems in 16.0% of those with AD compared to 10.1% of those without AD, with an especially reduced likelihood of romantic relationships for adolescent boys with AD, as measured using the Strength and Difficulties Questionnaire, which measures 4 problem domains and assesses presence of mental health issues in the past 6 months, and the Hopkins Symptom Checklist 10, which uses 10 questions to measure anxiety and depression symptoms in the past week.¹⁰

Dalgard et al¹¹ assessed whether the psychological burden of AD persists in adulthood in an international, multicenter, observational, cross-sectional study conducted in 13 European countries. Each dermatology clinic recruited 250 consecutive adult outpatients to complete a questionnaire along with a control group of 125 hospital employees without skin disease from the same institution but from different departments. The study included a total of 4994 participants (3635 patients and 1359 controls). Clinical depression and anxiety were present in 10.1% and 17.6% of patients, respectively, versus 4.3% and 11.1% of controls, respectively. The prevalence of depression and anxiety was highest in patients with leg ulcers, hand eczema, psoriasis, and AD.¹¹ This study demonstrated that the psychological comorbidities of childhood conditions such as AD may persist into adulthood.

Lymphoma

In a systematic review of the literature and a separate meta-analysis, Legendre et al¹² identified a slight increase in lymphoma among AD patients, with an uncertain but potential increase associated with topical corticosteroid application. This finding is similar to trends seen in other systemic inflammatory conditions that involve the skin, such as psoriasis, and is felt to relate to long-term inflammation.

Obesity

Obesity has been associated with a greater risk for moderate to severe AD in children.^13,14

Infections

Children with AD are at a higher risk for cutaneous infections and generalization of these infections. The leading infections would be with Staphylococcus aureus, but group A streptococci infections do occur. Herpes simplex virus, vaccinia virus or Kaposi varicelliform eruption (KVE), molluscum with or without dermatitis, and fungal infections occur less commonly but with greater morbidity, largely due to the impaired barrier and some innate reduction in cutaneous immunity.¹⁵

Atopic dermatitis in children also is associated with a higher prevalence of extracutaneous infections such as influenza, pneumonia, urinary tract infections, varicella-zoster virus, recurrent ear infections, sinus infections, sore throat, and head or chest colds.¹⁶ Children with AD and warts (human papillomavirus infection) have an even greater risk for these comorbidities.¹⁷ Warts and molluscum infections may become more extensive in children with AD.¹⁸ Generalization of herpetic infections occurs more easily in AD patients due to the impaired skin barrier, which includes generalized skin surface extension of herpes simplex virus type 1, varicella-zoster virus, and historically smallpox. A similar clinical appearance of generalized vesiculopustular lesions with fever can be seen when coxsackievirus A6 infections occur in AD patients; these conditions are called eczema herpeticum due to herpes simplex virus, KVE due to varicella-zoster virus and smallpox, and eczema coxsackium due to coxsackievirus A6,¹⁹ though some authors refer to all of these as KVE.²⁰ These generalized viral illnesses overlying AD often result in fever, malaise, pain, and life-threatening skin denudation with risk for dehydration and superinfection with S aureus.^7,18 It has been shown that the occurrence of eczema herpeticum in AD is associated with and may be caused by an inability to induce human β-defensin 2 and 3 as well as cathelicidin.²¹

Staphylococcus aureus colonization has been noted in 90% to 100% of AD cases, which can be associated with a higher eczema area and severity index score.^22-24 The role of S aureus in AD includes flare triggering through release of superantigens, leading to IL-31–induced pruritis.²⁵ Recurrent infection with either methicillin-sensitive or methicillin-resistant S aureus has been noted in AD.^18,26 Skin infections also occur in AD and appear as erosions and pustules, and coinfection with Streptococcus and Staphylococcus does occur; therefore, cultures often are needed to determine the type of bacteria present on the skin in severe cases and when infection is suspected.²⁷ Perianal bacterial dermatitis is a variant of infected AD occurring in the anal/groin area that is associated with S aureus and/or streptococcal superinfection in which topical corticosteroids and topical anti-infectives can be used. In some severe cases, oral antibiotics may be needed.²⁸

Injury/Hyperactivity

Children aged 0 to 5 years with AD carry an increased risk for injuries requiring medical attention, with association in part due to attention deficit disorder, depression, and anxiety. Antihistamines are believed to aggravate this issue by promoting daytime somnolence²⁹; however, pruritus-induced sleep disturbances in AD also may be responsible for daytime somnolence.³⁰

Contact Allergy and Sensitization

Children with AD may become sensitized to environmental allergens through delayed-type hypersensitivity. The presumed mechanism is that these agents include ingredients added into applied medicaments and application occurs over an impaired skin barrier allowing for absorption and greater risk of antigen presentation. Approximately 50% of children with difficult-to-control AD will react to 1 or more epicutaneous allergens, and patch testing can be performed to identify relevant allergens that can improve skin severity.⁷ Severe dermatitis and id generalized hypersensitivity reactions in patients with AD and nickel allergic contact dermatitis have been described and may aggravate underlying AD.³¹

Family Burden of AD

Parents or caregivers of children with moderate and severe AD spend nearly 3 hours a day caring for their child’s skin and experience QOL impairments including lack of sleep and/or privacy, often due to cosleeping; treatment-related financial expenditures; and feelings of hopelessness, guilt, and depression.⁷

Steroid Phobia

Steroid phobia is the fear of topical application of corticosteroids resulting in systemic side effects including unrealistic fears (eg, fear that the child will develop muscles such as an anabolic steroid user) as well as realistic but statistically low-risk fears (eg, fear of systemic absorption). These fears often result in underutilization of prescribed topical corticosteroid therapies and undertreatment of children with AD.^32,33

Financial Burden

The cost of AD can be high in the United States, with adult data demonstrating costs ranging from $371 to $489 per person.³⁴ The last published cost data for pediatric AD was from 2003, with an average cost of $219 per year.³⁵ Costs include time lost from work, household purchases (eg, skin care products), and co-pays for visits and medication, with an estimated average expenditure per person (SE) of $601.06 ($137.26) annually in 2012.³⁶ The cost of ambulatory care and emergency department visits for AD in children in the United States in 1993 was estimated at $364 million.^37-39 In 2002, Ellis et al⁴⁰ estimated the overall cost of AD to be between $900 million and $3.8 billion in the United States (1997-1998) based on projections from claims, prescriptions, and comorbidities reported to a private insurer and Medicaid. Ellis et al⁴¹ further determined that topical tacrolimus was similar in cost to high-potency corticosteroids.

Pediatric AD often progresses to adult hand eczema and leads to further morbidity, especially in health care workers.⁴² Kemp⁴³ reviewed the cost of AD in children and concluded that AD was a condition with major handicap with personal, financial, and social effects. A cost review of studies conducted in 163,700 children with AD showed that costs related to AD totaled $316.7 million per year. The author concluded that there were substantial psychosocial and financial stresses associated with pediatric AD but no clear path to potential reduction in related costs.⁴³

Sleep Disturbances

Sleep disturbances are common in pediatric AD patients. Pruritus usually is exacerbated at bedtime due to reduced humidity and lack of distractions to prevent scratching. Sleep deprivation has a substantial impact on both the patient and his/her household. Parental frustration increases with sleep disturbance.^18,44 Sleep deprivation is associated with greater severity, both because it is one of the most difficult aspects of illness and because the associated pruritus makes for greater damage done to the skin through injurious scratching.

Sleep disturbances also may interfere with growth and overnight release of growth hormones.^18,44 This latter issue can result in reduced linear growth velocity. Furthermore, sleep deprivation can cause increased risk of accidents and poor school performance.^18,44,45

Many children do not outgrow AD. In adults, AD-associated sleep deprivation has been shown to have an association with fatigue, regular daytime sleepiness, and regular insomnia, correlating to number of sick days, doctor visits, and poorer overall health status.⁴⁵

Inadequate Disease Control

Inadequate disease control has been described by Eichenfeld⁴⁶ as an important issue in AD at this time. Untreated, undertreated, and improperly treated AD are important issues affecting long-term AD care. He further cited steroid phobia as a contributor to undertreatment.⁴⁶ Fleischer⁴⁷ has cited the black box warning present on TCIs as a further deterrent to adequate therapeutic control in our current therapeutic paradigm. Undertreatment may result in uncontrolled disease activity, impaired QOL, infections, and sleep disturbances. The role of undertreatment as a driver of the atopic march is unknown.

Conclusion

Atopic dermatitis is a multisystem disorder that has wide-reaching comorbidities and may mimic a variety of skin conditions. The topic of comorbidities is new and emerging and bears further review to define risk factors, prevention strategies, and long-term monitoring requirements.

In parts 1 and 2 of this series on atopic dermatitis (AD),^1,2 the current putative pathogenesis, scoring systems for severity grading, and epidemiology were reviewed. Part 3 reviews the differential diagnosis, with an emphasis on the difficulty of differentiation from some rare but notable illnesses, as well as the recently expanding data on comorbidities that identify AD as a multisystem disorder with widespread health implications for the patient.

Differential Diagnosis for Pediatric AD

The differential diagnosis for pediatric AD includes chronic dermatoses (eg, seborrheic dermatitis, psoriasis), congenital disorders (eg, Netherton syndrome), malignant diseases (eg, cutaneous T-cell lymphoma [CTCL]), immunodeficiencies, infections, and metabolic disorders.³ Netherton syndrome must be ruled out to prevent extensive drug absorption when treating with topical calcineurin inhibitors (TCIs).⁴ Due to the presence of bamboo hairs in these patients, a hair mount may aid in the diagnosis of Netherton syndrome. Misdiagnosis of CTCL as AD may complicate the analysis of safety data on TCIs.^4,5 Multiple skin biopsies are essential in cases of suspected CTCL to provide an accurate diagnosis. Biopsy can be considered in AD cases with changing and/or unusual morphology, erythrodermic skin changes, and disease that is poorly responsive to multiple therapeutic modalities.

Comorbidities in Pediatric AD

Psychosocial Comorbidities

Pediatric AD often takes a psychological toll on patients as well as household members. Almost half of children with AD are reported to have a severely impaired quality of life (QOL).⁶ Contributing factors include fatigue, sleep disturbance, activity restriction (eg, inability to participate in sports), and depression.⁷

Chamlin et al⁸ developed the Childhood Atopic Dermatitis Impact Scale (CADIS), a 45-item instrument (refined from a 62-item prototype), to measure QOL in young children with AD and their family members. Responses were evaluated with consideration of 5 domains: symptoms and activity limitations/behaviors in children, as well as family/social function, sleep, and emotions in parents. The top 12 factors that parents found most bothersome about AD included itching/scratching, child’s pain/discomfort, sleep issues, embarrassment or worry about appearance, child’s fussiness/irritability/crying/unhappiness, helplessness/can’t control it/predict it, worry about skin infection, dryness of skin/nonsmooth skin, skin bleeding, worry about damage/scars, stares/comments of strangers and other children, and rashes/redness of skin/discoloration. Parents were asked to respond to items about their emotional health and social functioning, such as “My child’s skin condition has strained my relationship with my spouse or partner,” “My child’s skin condition makes me feel sad or depressed,” and “I am bothered by the reaction of strangers to this skin condition.”⁸

Kiebert et al⁹ found that AD patients had lower scores on the Short Form-36 Health Survey’s vitality, social functioning, and mental health subscales compared to individuals in the general population. The authors noted that anxiety in AD patients is of particular concern, as stress has been found to trigger the itch-scratch cycle, potentially setting off AD flare-ups.⁹ Family impact of AD is aggravated by disease severity. Sleeplessness, relationship stress, and time management can all cause family problems in patients with AD.⁸

In a survey of 3775 older teenagers aged 18 to 19 years (80% response rate out of 4774 prospective participants), 9.7% of participants reported having current AD.¹⁰ Suicidal ideation was higher in those with current AD than those without AD (15.5% vs 9.1%). The prevalence of suicidal ideation rose to 23.8% in those with both AD and itch. Diagnosis of AD (as determined through participant responses to the question, ‘‘Do you have, or have you had eczema?’’) was associated with mental health problems in 16.0% of those with AD compared to 10.1% of those without AD, with an especially reduced likelihood of romantic relationships for adolescent boys with AD, as measured using the Strength and Difficulties Questionnaire, which measures 4 problem domains and assesses presence of mental health issues in the past 6 months, and the Hopkins Symptom Checklist 10, which uses 10 questions to measure anxiety and depression symptoms in the past week.¹⁰

Dalgard et al¹¹ assessed whether the psychological burden of AD persists in adulthood in an international, multicenter, observational, cross-sectional study conducted in 13 European countries. Each dermatology clinic recruited 250 consecutive adult outpatients to complete a questionnaire along with a control group of 125 hospital employees without skin disease from the same institution but from different departments. The study included a total of 4994 participants (3635 patients and 1359 controls). Clinical depression and anxiety were present in 10.1% and 17.6% of patients, respectively, versus 4.3% and 11.1% of controls, respectively. The prevalence of depression and anxiety was highest in patients with leg ulcers, hand eczema, psoriasis, and AD.¹¹ This study demonstrated that the psychological comorbidities of childhood conditions such as AD may persist into adulthood.

Lymphoma

In a systematic review of the literature and a separate meta-analysis, Legendre et al¹² identified a slight increase in lymphoma among AD patients, with an uncertain but potential increase associated with topical corticosteroid application. This finding is similar to trends seen in other systemic inflammatory conditions that involve the skin, such as psoriasis, and is felt to relate to long-term inflammation.

Obesity

Obesity has been associated with a greater risk for moderate to severe AD in children.^13,14

Infections

Children with AD are at a higher risk for cutaneous infections and generalization of these infections. The leading infections would be with Staphylococcus aureus, but group A streptococci infections do occur. Herpes simplex virus, vaccinia virus or Kaposi varicelliform eruption (KVE), molluscum with or without dermatitis, and fungal infections occur less commonly but with greater morbidity, largely due to the impaired barrier and some innate reduction in cutaneous immunity.¹⁵

Atopic dermatitis in children also is associated with a higher prevalence of extracutaneous infections such as influenza, pneumonia, urinary tract infections, varicella-zoster virus, recurrent ear infections, sinus infections, sore throat, and head or chest colds.¹⁶ Children with AD and warts (human papillomavirus infection) have an even greater risk for these comorbidities.¹⁷ Warts and molluscum infections may become more extensive in children with AD.¹⁸ Generalization of herpetic infections occurs more easily in AD patients due to the impaired skin barrier, which includes generalized skin surface extension of herpes simplex virus type 1, varicella-zoster virus, and historically smallpox. A similar clinical appearance of generalized vesiculopustular lesions with fever can be seen when coxsackievirus A6 infections occur in AD patients; these conditions are called eczema herpeticum due to herpes simplex virus, KVE due to varicella-zoster virus and smallpox, and eczema coxsackium due to coxsackievirus A6,¹⁹ though some authors refer to all of these as KVE.²⁰ These generalized viral illnesses overlying AD often result in fever, malaise, pain, and life-threatening skin denudation with risk for dehydration and superinfection with S aureus.^7,18 It has been shown that the occurrence of eczema herpeticum in AD is associated with and may be caused by an inability to induce human β-defensin 2 and 3 as well as cathelicidin.²¹

Staphylococcus aureus colonization has been noted in 90% to 100% of AD cases, which can be associated with a higher eczema area and severity index score.^22-24 The role of S aureus in AD includes flare triggering through release of superantigens, leading to IL-31–induced pruritis.²⁵ Recurrent infection with either methicillin-sensitive or methicillin-resistant S aureus has been noted in AD.^18,26 Skin infections also occur in AD and appear as erosions and pustules, and coinfection with Streptococcus and Staphylococcus does occur; therefore, cultures often are needed to determine the type of bacteria present on the skin in severe cases and when infection is suspected.²⁷ Perianal bacterial dermatitis is a variant of infected AD occurring in the anal/groin area that is associated with S aureus and/or streptococcal superinfection in which topical corticosteroids and topical anti-infectives can be used. In some severe cases, oral antibiotics may be needed.²⁸

Injury/Hyperactivity

Children aged 0 to 5 years with AD carry an increased risk for injuries requiring medical attention, with association in part due to attention deficit disorder, depression, and anxiety. Antihistamines are believed to aggravate this issue by promoting daytime somnolence²⁹; however, pruritus-induced sleep disturbances in AD also may be responsible for daytime somnolence.³⁰

Contact Allergy and Sensitization

Children with AD may become sensitized to environmental allergens through delayed-type hypersensitivity. The presumed mechanism is that these agents include ingredients added into applied medicaments and application occurs over an impaired skin barrier allowing for absorption and greater risk of antigen presentation. Approximately 50% of children with difficult-to-control AD will react to 1 or more epicutaneous allergens, and patch testing can be performed to identify relevant allergens that can improve skin severity.⁷ Severe dermatitis and id generalized hypersensitivity reactions in patients with AD and nickel allergic contact dermatitis have been described and may aggravate underlying AD.³¹

Family Burden of AD

Parents or caregivers of children with moderate and severe AD spend nearly 3 hours a day caring for their child’s skin and experience QOL impairments including lack of sleep and/or privacy, often due to cosleeping; treatment-related financial expenditures; and feelings of hopelessness, guilt, and depression.⁷

Steroid Phobia

Steroid phobia is the fear of topical application of corticosteroids resulting in systemic side effects including unrealistic fears (eg, fear that the child will develop muscles such as an anabolic steroid user) as well as realistic but statistically low-risk fears (eg, fear of systemic absorption). These fears often result in underutilization of prescribed topical corticosteroid therapies and undertreatment of children with AD.^32,33

Financial Burden

The cost of AD can be high in the United States, with adult data demonstrating costs ranging from $371 to $489 per person.³⁴ The last published cost data for pediatric AD was from 2003, with an average cost of $219 per year.³⁵ Costs include time lost from work, household purchases (eg, skin care products), and co-pays for visits and medication, with an estimated average expenditure per person (SE) of $601.06 ($137.26) annually in 2012.³⁶ The cost of ambulatory care and emergency department visits for AD in children in the United States in 1993 was estimated at $364 million.^37-39 In 2002, Ellis et al⁴⁰ estimated the overall cost of AD to be between $900 million and $3.8 billion in the United States (1997-1998) based on projections from claims, prescriptions, and comorbidities reported to a private insurer and Medicaid. Ellis et al⁴¹ further determined that topical tacrolimus was similar in cost to high-potency corticosteroids.

Pediatric AD often progresses to adult hand eczema and leads to further morbidity, especially in health care workers.⁴² Kemp⁴³ reviewed the cost of AD in children and concluded that AD was a condition with major handicap with personal, financial, and social effects. A cost review of studies conducted in 163,700 children with AD showed that costs related to AD totaled $316.7 million per year. The author concluded that there were substantial psychosocial and financial stresses associated with pediatric AD but no clear path to potential reduction in related costs.⁴³

Sleep Disturbances

Sleep disturbances are common in pediatric AD patients. Pruritus usually is exacerbated at bedtime due to reduced humidity and lack of distractions to prevent scratching. Sleep deprivation has a substantial impact on both the patient and his/her household. Parental frustration increases with sleep disturbance.^18,44 Sleep deprivation is associated with greater severity, both because it is one of the most difficult aspects of illness and because the associated pruritus makes for greater damage done to the skin through injurious scratching.

Sleep disturbances also may interfere with growth and overnight release of growth hormones.^18,44 This latter issue can result in reduced linear growth velocity. Furthermore, sleep deprivation can cause increased risk of accidents and poor school performance.^18,44,45

Many children do not outgrow AD. In adults, AD-associated sleep deprivation has been shown to have an association with fatigue, regular daytime sleepiness, and regular insomnia, correlating to number of sick days, doctor visits, and poorer overall health status.⁴⁵

Inadequate Disease Control

Inadequate disease control has been described by Eichenfeld⁴⁶ as an important issue in AD at this time. Untreated, undertreated, and improperly treated AD are important issues affecting long-term AD care. He further cited steroid phobia as a contributor to undertreatment.⁴⁶ Fleischer⁴⁷ has cited the black box warning present on TCIs as a further deterrent to adequate therapeutic control in our current therapeutic paradigm. Undertreatment may result in uncontrolled disease activity, impaired QOL, infections, and sleep disturbances. The role of undertreatment as a driver of the atopic march is unknown.

Conclusion

Atopic dermatitis is a multisystem disorder that has wide-reaching comorbidities and may mimic a variety of skin conditions. The topic of comorbidities is new and emerging and bears further review to define risk factors, prevention strategies, and long-term monitoring requirements.

In parts 1 and 2 of this series on atopic dermatitis (AD),^1,2 the current putative pathogenesis, scoring systems for severity grading, and epidemiology were reviewed. Part 3 reviews the differential diagnosis, with an emphasis on the difficulty of differentiation from some rare but notable illnesses, as well as the recently expanding data on comorbidities that identify AD as a multisystem disorder with widespread health implications for the patient.

Differential Diagnosis for Pediatric AD

The differential diagnosis for pediatric AD includes chronic dermatoses (eg, seborrheic dermatitis, psoriasis), congenital disorders (eg, Netherton syndrome), malignant diseases (eg, cutaneous T-cell lymphoma [CTCL]), immunodeficiencies, infections, and metabolic disorders.³ Netherton syndrome must be ruled out to prevent extensive drug absorption when treating with topical calcineurin inhibitors (TCIs).⁴ Due to the presence of bamboo hairs in these patients, a hair mount may aid in the diagnosis of Netherton syndrome. Misdiagnosis of CTCL as AD may complicate the analysis of safety data on TCIs.^4,5 Multiple skin biopsies are essential in cases of suspected CTCL to provide an accurate diagnosis. Biopsy can be considered in AD cases with changing and/or unusual morphology, erythrodermic skin changes, and disease that is poorly responsive to multiple therapeutic modalities.

Comorbidities in Pediatric AD

Psychosocial Comorbidities

Pediatric AD often takes a psychological toll on patients as well as household members. Almost half of children with AD are reported to have a severely impaired quality of life (QOL).⁶ Contributing factors include fatigue, sleep disturbance, activity restriction (eg, inability to participate in sports), and depression.⁷

Chamlin et al⁸ developed the Childhood Atopic Dermatitis Impact Scale (CADIS), a 45-item instrument (refined from a 62-item prototype), to measure QOL in young children with AD and their family members. Responses were evaluated with consideration of 5 domains: symptoms and activity limitations/behaviors in children, as well as family/social function, sleep, and emotions in parents. The top 12 factors that parents found most bothersome about AD included itching/scratching, child’s pain/discomfort, sleep issues, embarrassment or worry about appearance, child’s fussiness/irritability/crying/unhappiness, helplessness/can’t control it/predict it, worry about skin infection, dryness of skin/nonsmooth skin, skin bleeding, worry about damage/scars, stares/comments of strangers and other children, and rashes/redness of skin/discoloration. Parents were asked to respond to items about their emotional health and social functioning, such as “My child’s skin condition has strained my relationship with my spouse or partner,” “My child’s skin condition makes me feel sad or depressed,” and “I am bothered by the reaction of strangers to this skin condition.”⁸

Kiebert et al⁹ found that AD patients had lower scores on the Short Form-36 Health Survey’s vitality, social functioning, and mental health subscales compared to individuals in the general population. The authors noted that anxiety in AD patients is of particular concern, as stress has been found to trigger the itch-scratch cycle, potentially setting off AD flare-ups.⁹ Family impact of AD is aggravated by disease severity. Sleeplessness, relationship stress, and time management can all cause family problems in patients with AD.⁸

In a survey of 3775 older teenagers aged 18 to 19 years (80% response rate out of 4774 prospective participants), 9.7% of participants reported having current AD.¹⁰ Suicidal ideation was higher in those with current AD than those without AD (15.5% vs 9.1%). The prevalence of suicidal ideation rose to 23.8% in those with both AD and itch. Diagnosis of AD (as determined through participant responses to the question, ‘‘Do you have, or have you had eczema?’’) was associated with mental health problems in 16.0% of those with AD compared to 10.1% of those without AD, with an especially reduced likelihood of romantic relationships for adolescent boys with AD, as measured using the Strength and Difficulties Questionnaire, which measures 4 problem domains and assesses presence of mental health issues in the past 6 months, and the Hopkins Symptom Checklist 10, which uses 10 questions to measure anxiety and depression symptoms in the past week.¹⁰

Dalgard et al¹¹ assessed whether the psychological burden of AD persists in adulthood in an international, multicenter, observational, cross-sectional study conducted in 13 European countries. Each dermatology clinic recruited 250 consecutive adult outpatients to complete a questionnaire along with a control group of 125 hospital employees without skin disease from the same institution but from different departments. The study included a total of 4994 participants (3635 patients and 1359 controls). Clinical depression and anxiety were present in 10.1% and 17.6% of patients, respectively, versus 4.3% and 11.1% of controls, respectively. The prevalence of depression and anxiety was highest in patients with leg ulcers, hand eczema, psoriasis, and AD.¹¹ This study demonstrated that the psychological comorbidities of childhood conditions such as AD may persist into adulthood.

Lymphoma

In a systematic review of the literature and a separate meta-analysis, Legendre et al¹² identified a slight increase in lymphoma among AD patients, with an uncertain but potential increase associated with topical corticosteroid application. This finding is similar to trends seen in other systemic inflammatory conditions that involve the skin, such as psoriasis, and is felt to relate to long-term inflammation.

Obesity

Obesity has been associated with a greater risk for moderate to severe AD in children.^13,14

Infections

Children with AD are at a higher risk for cutaneous infections and generalization of these infections. The leading infections would be with Staphylococcus aureus, but group A streptococci infections do occur. Herpes simplex virus, vaccinia virus or Kaposi varicelliform eruption (KVE), molluscum with or without dermatitis, and fungal infections occur less commonly but with greater morbidity, largely due to the impaired barrier and some innate reduction in cutaneous immunity.¹⁵

Atopic dermatitis in children also is associated with a higher prevalence of extracutaneous infections such as influenza, pneumonia, urinary tract infections, varicella-zoster virus, recurrent ear infections, sinus infections, sore throat, and head or chest colds.¹⁶ Children with AD and warts (human papillomavirus infection) have an even greater risk for these comorbidities.¹⁷ Warts and molluscum infections may become more extensive in children with AD.¹⁸ Generalization of herpetic infections occurs more easily in AD patients due to the impaired skin barrier, which includes generalized skin surface extension of herpes simplex virus type 1, varicella-zoster virus, and historically smallpox. A similar clinical appearance of generalized vesiculopustular lesions with fever can be seen when coxsackievirus A6 infections occur in AD patients; these conditions are called eczema herpeticum due to herpes simplex virus, KVE due to varicella-zoster virus and smallpox, and eczema coxsackium due to coxsackievirus A6,¹⁹ though some authors refer to all of these as KVE.²⁰ These generalized viral illnesses overlying AD often result in fever, malaise, pain, and life-threatening skin denudation with risk for dehydration and superinfection with S aureus.^7,18 It has been shown that the occurrence of eczema herpeticum in AD is associated with and may be caused by an inability to induce human β-defensin 2 and 3 as well as cathelicidin.²¹

Staphylococcus aureus colonization has been noted in 90% to 100% of AD cases, which can be associated with a higher eczema area and severity index score.^22-24 The role of S aureus in AD includes flare triggering through release of superantigens, leading to IL-31–induced pruritis.²⁵ Recurrent infection with either methicillin-sensitive or methicillin-resistant S aureus has been noted in AD.^18,26 Skin infections also occur in AD and appear as erosions and pustules, and coinfection with Streptococcus and Staphylococcus does occur; therefore, cultures often are needed to determine the type of bacteria present on the skin in severe cases and when infection is suspected.²⁷ Perianal bacterial dermatitis is a variant of infected AD occurring in the anal/groin area that is associated with S aureus and/or streptococcal superinfection in which topical corticosteroids and topical anti-infectives can be used. In some severe cases, oral antibiotics may be needed.²⁸

Injury/Hyperactivity

Children aged 0 to 5 years with AD carry an increased risk for injuries requiring medical attention, with association in part due to attention deficit disorder, depression, and anxiety. Antihistamines are believed to aggravate this issue by promoting daytime somnolence²⁹; however, pruritus-induced sleep disturbances in AD also may be responsible for daytime somnolence.³⁰

Contact Allergy and Sensitization

Children with AD may become sensitized to environmental allergens through delayed-type hypersensitivity. The presumed mechanism is that these agents include ingredients added into applied medicaments and application occurs over an impaired skin barrier allowing for absorption and greater risk of antigen presentation. Approximately 50% of children with difficult-to-control AD will react to 1 or more epicutaneous allergens, and patch testing can be performed to identify relevant allergens that can improve skin severity.⁷ Severe dermatitis and id generalized hypersensitivity reactions in patients with AD and nickel allergic contact dermatitis have been described and may aggravate underlying AD.³¹

Family Burden of AD

Parents or caregivers of children with moderate and severe AD spend nearly 3 hours a day caring for their child’s skin and experience QOL impairments including lack of sleep and/or privacy, often due to cosleeping; treatment-related financial expenditures; and feelings of hopelessness, guilt, and depression.⁷

Steroid Phobia

Steroid phobia is the fear of topical application of corticosteroids resulting in systemic side effects including unrealistic fears (eg, fear that the child will develop muscles such as an anabolic steroid user) as well as realistic but statistically low-risk fears (eg, fear of systemic absorption). These fears often result in underutilization of prescribed topical corticosteroid therapies and undertreatment of children with AD.^32,33

Financial Burden

The cost of AD can be high in the United States, with adult data demonstrating costs ranging from $371 to $489 per person.³⁴ The last published cost data for pediatric AD was from 2003, with an average cost of $219 per year.³⁵ Costs include time lost from work, household purchases (eg, skin care products), and co-pays for visits and medication, with an estimated average expenditure per person (SE) of $601.06 ($137.26) annually in 2012.³⁶ The cost of ambulatory care and emergency department visits for AD in children in the United States in 1993 was estimated at $364 million.^37-39 In 2002, Ellis et al⁴⁰ estimated the overall cost of AD to be between $900 million and $3.8 billion in the United States (1997-1998) based on projections from claims, prescriptions, and comorbidities reported to a private insurer and Medicaid. Ellis et al⁴¹ further determined that topical tacrolimus was similar in cost to high-potency corticosteroids.

Pediatric AD often progresses to adult hand eczema and leads to further morbidity, especially in health care workers.⁴² Kemp⁴³ reviewed the cost of AD in children and concluded that AD was a condition with major handicap with personal, financial, and social effects. A cost review of studies conducted in 163,700 children with AD showed that costs related to AD totaled $316.7 million per year. The author concluded that there were substantial psychosocial and financial stresses associated with pediatric AD but no clear path to potential reduction in related costs.⁴³

Sleep Disturbances

Sleep disturbances are common in pediatric AD patients. Pruritus usually is exacerbated at bedtime due to reduced humidity and lack of distractions to prevent scratching. Sleep deprivation has a substantial impact on both the patient and his/her household. Parental frustration increases with sleep disturbance.^18,44 Sleep deprivation is associated with greater severity, both because it is one of the most difficult aspects of illness and because the associated pruritus makes for greater damage done to the skin through injurious scratching.

Sleep disturbances also may interfere with growth and overnight release of growth hormones.^18,44 This latter issue can result in reduced linear growth velocity. Furthermore, sleep deprivation can cause increased risk of accidents and poor school performance.^18,44,45

Many children do not outgrow AD. In adults, AD-associated sleep deprivation has been shown to have an association with fatigue, regular daytime sleepiness, and regular insomnia, correlating to number of sick days, doctor visits, and poorer overall health status.⁴⁵

Inadequate Disease Control

Inadequate disease control has been described by Eichenfeld⁴⁶ as an important issue in AD at this time. Untreated, undertreated, and improperly treated AD are important issues affecting long-term AD care. He further cited steroid phobia as a contributor to undertreatment.⁴⁶ Fleischer⁴⁷ has cited the black box warning present on TCIs as a further deterrent to adequate therapeutic control in our current therapeutic paradigm. Undertreatment may result in uncontrolled disease activity, impaired QOL, infections, and sleep disturbances. The role of undertreatment as a driver of the atopic march is unknown.

Conclusion

Atopic dermatitis is a multisystem disorder that has wide-reaching comorbidities and may mimic a variety of skin conditions. The topic of comorbidities is new and emerging and bears further review to define risk factors, prevention strategies, and long-term monitoring requirements.

As a hospitalist, patient satisfaction is top of mind. Then there’s another group of people whose satisfaction is also paramount: your family. What do they have to say about life with a hospitalist? You’re about to find out!

The Hospitalist asked family members of David Pressel, MD, PhD, a pediatric hospitalist at A.I. DuPont Hospital for Children in Wilmington, Del., and a former member of Team Hospitalist, for their impressions, and wife Karen and son Rob’s honest answers (and gentle ribbings) show that for whatever ups and downs life may bring, being part of a hospitalist’s family is full of rewards and lots of love. Of course, that’s not to say they didn’t have some suggestions for improvements.

For Hospitalists’ Spouses Everywhere

Marrying a doctor was never on my to-do list. In fact, my list specified quite the opposite; I was never going to marry a physician. My stereotypical perception of the lives of physicians included long hours, too much stress, no family time, guaranteed interruptions at social events, calls at all hours of the day, never enough sleep—you get the picture. I imagined too many headaches to make being a “doctor’s wife” in the slightest bit enticing. I wanted no part of it, and besides, I had my own career to think about.

But then I met my husband, and my list went out the window.

Still, after a couple decades of negotiating a balance between the demands of his job (see above) and the demands of his family, there are things I’d like to say to him. So here goes. Hospitalists, take note.

There Are Only 24 Hours in a Day

How many times have you called to say, “I’ll be leaving the hospital in 10 minutes”? How long did it take for me to realize that relying on that kind of statement was crazy? I’m embarrassed to say that it took me way longer than it should have to come to that understanding. After many overcooked dinners and missed social events, I finally realized that your anticipated departure held no validity and I could only trust that you had left the hospital when you called from the car with the wheels rolling. Fortunately, you were more astute than I and changed your communication habits rather quickly, although the timing of said notifications still does not always take traffic into account and could use some work.

Still, I recognize that “leaving the hospital” is really just a physical indicator of your location, not necessarily a reflection of your state of mind. When you get home, please tell me if you still have work to do (notes, email, patient follow-up) or if you are done for the day. I suppose second-guessing your clinical decisions and calling the hospital to check on patients are unavoidable, but give me a clue whether I should actually expect you fully home to join the rest of the family—or if you will just be working at the home “nursing station” all evening. The burden of healthcare in America doesn’t fall on just you. If you can’t figure out what is wrong with a patient or don’t know what to do, you have many colleagues who can help.

Please remember that you are only one person. Don’t think that if there is a staffing shortage to fill, the responsibility for working is yours. Your colleagues are wonderful and, almost without exception, are happy to pitch in to help carry the extra load. The same goes with holidays; you don’t need to work more than everyone else. I know you are not a slacker. If you try to spread the load when you manage patient care and work schedules, you will have a happier spouse. Remember, a happy wife is a happy life. (I’m sure there is an analogous saying for your colleagues’ husbands and partners.)

Along those same lines, please limit the moonlighting you choose to do. My preconceived idea of a physician’s salary was very different from your reality. You are a pediatrician, an academic pediatrician. Having said that, we lead a wonderful life. We have what we need and have been very happy without the fanciness of some of our neighbors. Although the extra income is nice, I’d rather see more of you than more money. Besides, we just wrote the last check for college tuition, and I’m sure the boys will never ask us for money again.

Being Grumpy (No, Not the Dwarf)

My thoughts on moonlighting lead me perfectly to a discussion of your frame of mind: your mood. By definition, your patients are seriously ill hospitalized children. The bursting hospital census, the acuity of your patients, and the relative craziness of some of their parents invariably elevate your stress level. This, in turn, drives more frequent calls to the hospital and time on the computer all hours of the day or night. This does not allow for a restful sleep, when you sleep at all. I may be biased, but I think you are in the minority of hospitalists who bring their jobs home. Not that I’m complaining too loudly; this is who you are and why I love you, but if you haven’t noticed, when you are on service you tend to get grumpy. Think about this: If you’re not on call, why not turn off your pager, turn off your phone, and leave email alone?

Given the pressures inherent in your job, please tell me again why you would want to moonlight. Moonlighting means even longer hours, more stress, and less sleep for you, all of which make you grumpier and, as a result, tend to make me grumpy.

No, thank you.

Everyone we know has some form of “honey-do” list, whether intended for himself or herself, a spouse, or a professional. I know it makes you feel like a competent husband and man to do things around the house, but here’s a bit of advice: Let me hire someone else. Keep in mind that contractors were invented for good reason. The aggravation you’ll have trying to fit whatever project we’ve contemplated into your schedule will be dwarfed by the aggravation I’ll have when you can’t. I’ve never heard you ruminate about not cutting the lawn after we hired the landscaper and you got rid of the lawnmower.

The same goes for quality. Do you really think you did anywhere near as good a job replacing the leaking toilet as a real plumber? Should we talk about the breakfast room light fixture? Do you want me to continue?

My annoyance probably lessened any satisfaction you derived by completing these projects yourself. You should always keep the Pressel money-management credo forefront in your mind: “You earn it, I spend it.” Please let me do my job.

Let Me See If “The Doctor” Is In

Please leave the professor at the office; don’t talk too much medicine when you are not at work. Your trainees might need to hear all the minute details of whatever medical issue is at hand, but your family and friends do not. Most of those close to us chose careers outside of medicine a long time ago and probably don’t want to change direction now. Why do you think they call me for medical advice? It’s not because I’m a better doctor but because they know they’ll hear one of two things:

1. I’ll tell them I don’t have a clue and they should ask you; or
2. I’ll answer their questions in a tenth of the time that it would have taken you. And we’re talking easy questions because, while I’ve listened to you speak to medical students and residents for the last 20 years, we both know I am not a doctor.

Nevertheless, I do pretty well even with some of the hard questions, if I say so myself. Don’t worry though, there’s no need for concern. Please know that I am not practicing with your license.

Relative to the home practice of medicine, it’s OK to look in our kids’ ears! You must remember the huge fight we had when our son exhibited all of the classic signs of an ear infection and you refused to examine his ears. I know you agonize when you make a clinical error with a patient, but this was just an ear infection. I would have taken him to a real doctor if he was sick enough to merit consideration of what you were worried about missing (brain abscess or meningitis). Really? If I had known how to work your otoscope back then, I would have looked in his ears myself. I’m still not sure how treating minor illnesses in our children is different from the same thing with children of our friends.

You have a perfectly reasonable excuse to be exhausted, yet you are often embarrassed when you fall asleep at our friends’ houses during social events. But the truth is they consider it a mark of true friendship when you go missing before dessert is served. When we were still new in the area and someone would realize that you had disappeared, I was mortified. I quickly realized though that our friends would all rather you and I join them than stay home entirely. No one is offended to find you asleep on the sofa (and your disappearance is now almost expected). To tell you the truth, I’m not sure anyone misses your conversation.

Meetings make the world go round, and your attendance is obligatory at many, even if you’d rather not attend. When I was still working, someone came up with the idea of a stand-up meeting. It was a brilliant idea that made meeting participants use the time more efficiently. Why don’t you propose that some of your administrative meetings be run that way rather than depending on me to page you, “Dr. Pressel, we need you urgently in room 23!”? Sorry I’m calling you out on this, but I’m not always available at the exact time you’ve specified that you want to be interrupted. Besides, it is sometimes amusing to hear that you fell asleep at some senior hospital administrator’s meeting.

I started this by writing that I never wanted to marry a physician, but the last quarter century with you has been the adventure of a lifetime. I just sometimes ask myself, “Why didn’t he become a dermatologist?” TH

Karen Pressel is the wife of David Pressel, MD, PhD, a pediatric hospitalist at A.I. duPont Hospital for Children in Wilmington, Del., and a former member of Team Hospitalist.

What I Want My Hospitalist Father to Know

Let me start out by saying that I think you have a great job and I am proud of you. But there are some things you should know. I’ll begin with the good ones.

We lead a very comfortable life, and I am grateful for all that you do for me. You don’t need to remind me, though, every time you manually scoop poop from some constipated kid that it pays for the roof over my head, clothes on my back, and my expensive university education.

I get it.

Even so, having a parent who is a physician is way better than having a parent who is, say, an accountant. I don’t need help with my taxes, but it sure is nice to get some quick medical advice when I have a rash. I even still trust you after you missed my broken arm when I was in sixth grade. Do me a favor though: Just tell me what it is and how I can fix the problem. Save the lecture on the pathophysiology, epidemiology, and differential diagnosis for your residents and medical students. It’s only poison ivy.

When we were growing up, you always gave us a “case of the week.” There were some consistent themes, and I’ve never been sure if these patients were real or fake. Most were either adolescent girls with belly pain or children experiencing bizarre spells who ended up being intoxicated from some ingestion. Was there supposed to be a not-so-subtle message here not to use drugs and to choose my romantic interests carefully?

I actually enjoy hearing about interesting patients, although maybe you could vary the cases, focusing more on human-interest situations rather than on complex technical patients. Relative to the human-interest stories, shouldn’t some of the names parents give to their children be considered child abuse? You probably don’t know, but in Iceland, there is a government Naming Committee that actually maintains a list of approved children’s names.

I know you have to take both clinical and administrative calls. When you get a medical call while we’re having dinner, would you please go somewhere else to talk? Hearing you ask about a patient’s diarrhea when we are eating sort of ruins my appetite.

Similarly, please let me vet topics before you discuss them with my friends. You have some cool stories, but Dad, I’m not sure my friends want to hear about child abuse or vaginal discharge. I will say that the absolute best phone calls you get occur when, after 22 years of Pressel Medical School, I’m able to make the diagnosis or give the correct advice (sometimes faster than your medical students and residents).

Let’s talk about what you learned from me. Though you may not agree, you should think of all the times you found me annoying, particularly when I was a pain-in-the-ass kid, as CME. Over the years, I gave you regular opportunities to enhance your knowledge of child development and to improve your parenting skills—things that undoubtedly continue to help you as a pediatrician.

I like visiting you in the hospital. I know you enjoy showing me off. When you introduce me to your coworkers, it’s OK if you tell them I’m not going to medical school. Still, you should know that I fully intend to repay all that you have done.

Hearing from you about all that happens in a hospital, I can understand why you never want to be a patient. I’ll do my best to ensure you don’t get admitted to a hospital and are able to die peacefully at home. You can count on your loving son, Dad. I’ll be sure you don’t have a hospitalist with you at the end. TH

Rob Pressel is the son of David Pressel, MD, PhD, a pediatric hospitalist at A.I. duPont Hospital for Children in Wilmington, Del., and a former member of Team Hospitalist.

As a hospitalist, patient satisfaction is top of mind. Then there’s another group of people whose satisfaction is also paramount: your family. What do they have to say about life with a hospitalist? You’re about to find out!

The Hospitalist asked family members of David Pressel, MD, PhD, a pediatric hospitalist at A.I. DuPont Hospital for Children in Wilmington, Del., and a former member of Team Hospitalist, for their impressions, and wife Karen and son Rob’s honest answers (and gentle ribbings) show that for whatever ups and downs life may bring, being part of a hospitalist’s family is full of rewards and lots of love. Of course, that’s not to say they didn’t have some suggestions for improvements.

For Hospitalists’ Spouses Everywhere

Marrying a doctor was never on my to-do list. In fact, my list specified quite the opposite; I was never going to marry a physician. My stereotypical perception of the lives of physicians included long hours, too much stress, no family time, guaranteed interruptions at social events, calls at all hours of the day, never enough sleep—you get the picture. I imagined too many headaches to make being a “doctor’s wife” in the slightest bit enticing. I wanted no part of it, and besides, I had my own career to think about.

But then I met my husband, and my list went out the window.

Still, after a couple decades of negotiating a balance between the demands of his job (see above) and the demands of his family, there are things I’d like to say to him. So here goes. Hospitalists, take note.

There Are Only 24 Hours in a Day

How many times have you called to say, “I’ll be leaving the hospital in 10 minutes”? How long did it take for me to realize that relying on that kind of statement was crazy? I’m embarrassed to say that it took me way longer than it should have to come to that understanding. After many overcooked dinners and missed social events, I finally realized that your anticipated departure held no validity and I could only trust that you had left the hospital when you called from the car with the wheels rolling. Fortunately, you were more astute than I and changed your communication habits rather quickly, although the timing of said notifications still does not always take traffic into account and could use some work.

Still, I recognize that “leaving the hospital” is really just a physical indicator of your location, not necessarily a reflection of your state of mind. When you get home, please tell me if you still have work to do (notes, email, patient follow-up) or if you are done for the day. I suppose second-guessing your clinical decisions and calling the hospital to check on patients are unavoidable, but give me a clue whether I should actually expect you fully home to join the rest of the family—or if you will just be working at the home “nursing station” all evening. The burden of healthcare in America doesn’t fall on just you. If you can’t figure out what is wrong with a patient or don’t know what to do, you have many colleagues who can help.

Please remember that you are only one person. Don’t think that if there is a staffing shortage to fill, the responsibility for working is yours. Your colleagues are wonderful and, almost without exception, are happy to pitch in to help carry the extra load. The same goes with holidays; you don’t need to work more than everyone else. I know you are not a slacker. If you try to spread the load when you manage patient care and work schedules, you will have a happier spouse. Remember, a happy wife is a happy life. (I’m sure there is an analogous saying for your colleagues’ husbands and partners.)

Along those same lines, please limit the moonlighting you choose to do. My preconceived idea of a physician’s salary was very different from your reality. You are a pediatrician, an academic pediatrician. Having said that, we lead a wonderful life. We have what we need and have been very happy without the fanciness of some of our neighbors. Although the extra income is nice, I’d rather see more of you than more money. Besides, we just wrote the last check for college tuition, and I’m sure the boys will never ask us for money again.

Being Grumpy (No, Not the Dwarf)

My thoughts on moonlighting lead me perfectly to a discussion of your frame of mind: your mood. By definition, your patients are seriously ill hospitalized children. The bursting hospital census, the acuity of your patients, and the relative craziness of some of their parents invariably elevate your stress level. This, in turn, drives more frequent calls to the hospital and time on the computer all hours of the day or night. This does not allow for a restful sleep, when you sleep at all. I may be biased, but I think you are in the minority of hospitalists who bring their jobs home. Not that I’m complaining too loudly; this is who you are and why I love you, but if you haven’t noticed, when you are on service you tend to get grumpy. Think about this: If you’re not on call, why not turn off your pager, turn off your phone, and leave email alone?

Given the pressures inherent in your job, please tell me again why you would want to moonlight. Moonlighting means even longer hours, more stress, and less sleep for you, all of which make you grumpier and, as a result, tend to make me grumpy.

No, thank you.

Everyone we know has some form of “honey-do” list, whether intended for himself or herself, a spouse, or a professional. I know it makes you feel like a competent husband and man to do things around the house, but here’s a bit of advice: Let me hire someone else. Keep in mind that contractors were invented for good reason. The aggravation you’ll have trying to fit whatever project we’ve contemplated into your schedule will be dwarfed by the aggravation I’ll have when you can’t. I’ve never heard you ruminate about not cutting the lawn after we hired the landscaper and you got rid of the lawnmower.

The same goes for quality. Do you really think you did anywhere near as good a job replacing the leaking toilet as a real plumber? Should we talk about the breakfast room light fixture? Do you want me to continue?

My annoyance probably lessened any satisfaction you derived by completing these projects yourself. You should always keep the Pressel money-management credo forefront in your mind: “You earn it, I spend it.” Please let me do my job.

Let Me See If “The Doctor” Is In

Please leave the professor at the office; don’t talk too much medicine when you are not at work. Your trainees might need to hear all the minute details of whatever medical issue is at hand, but your family and friends do not. Most of those close to us chose careers outside of medicine a long time ago and probably don’t want to change direction now. Why do you think they call me for medical advice? It’s not because I’m a better doctor but because they know they’ll hear one of two things:

1. I’ll tell them I don’t have a clue and they should ask you; or
2. I’ll answer their questions in a tenth of the time that it would have taken you. And we’re talking easy questions because, while I’ve listened to you speak to medical students and residents for the last 20 years, we both know I am not a doctor.

Nevertheless, I do pretty well even with some of the hard questions, if I say so myself. Don’t worry though, there’s no need for concern. Please know that I am not practicing with your license.

Relative to the home practice of medicine, it’s OK to look in our kids’ ears! You must remember the huge fight we had when our son exhibited all of the classic signs of an ear infection and you refused to examine his ears. I know you agonize when you make a clinical error with a patient, but this was just an ear infection. I would have taken him to a real doctor if he was sick enough to merit consideration of what you were worried about missing (brain abscess or meningitis). Really? If I had known how to work your otoscope back then, I would have looked in his ears myself. I’m still not sure how treating minor illnesses in our children is different from the same thing with children of our friends.

You have a perfectly reasonable excuse to be exhausted, yet you are often embarrassed when you fall asleep at our friends’ houses during social events. But the truth is they consider it a mark of true friendship when you go missing before dessert is served. When we were still new in the area and someone would realize that you had disappeared, I was mortified. I quickly realized though that our friends would all rather you and I join them than stay home entirely. No one is offended to find you asleep on the sofa (and your disappearance is now almost expected). To tell you the truth, I’m not sure anyone misses your conversation.

Meetings make the world go round, and your attendance is obligatory at many, even if you’d rather not attend. When I was still working, someone came up with the idea of a stand-up meeting. It was a brilliant idea that made meeting participants use the time more efficiently. Why don’t you propose that some of your administrative meetings be run that way rather than depending on me to page you, “Dr. Pressel, we need you urgently in room 23!”? Sorry I’m calling you out on this, but I’m not always available at the exact time you’ve specified that you want to be interrupted. Besides, it is sometimes amusing to hear that you fell asleep at some senior hospital administrator’s meeting.

I started this by writing that I never wanted to marry a physician, but the last quarter century with you has been the adventure of a lifetime. I just sometimes ask myself, “Why didn’t he become a dermatologist?” TH

Karen Pressel is the wife of David Pressel, MD, PhD, a pediatric hospitalist at A.I. duPont Hospital for Children in Wilmington, Del., and a former member of Team Hospitalist.

What I Want My Hospitalist Father to Know

Let me start out by saying that I think you have a great job and I am proud of you. But there are some things you should know. I’ll begin with the good ones.

We lead a very comfortable life, and I am grateful for all that you do for me. You don’t need to remind me, though, every time you manually scoop poop from some constipated kid that it pays for the roof over my head, clothes on my back, and my expensive university education.

I get it.

Even so, having a parent who is a physician is way better than having a parent who is, say, an accountant. I don’t need help with my taxes, but it sure is nice to get some quick medical advice when I have a rash. I even still trust you after you missed my broken arm when I was in sixth grade. Do me a favor though: Just tell me what it is and how I can fix the problem. Save the lecture on the pathophysiology, epidemiology, and differential diagnosis for your residents and medical students. It’s only poison ivy.

When we were growing up, you always gave us a “case of the week.” There were some consistent themes, and I’ve never been sure if these patients were real or fake. Most were either adolescent girls with belly pain or children experiencing bizarre spells who ended up being intoxicated from some ingestion. Was there supposed to be a not-so-subtle message here not to use drugs and to choose my romantic interests carefully?

I actually enjoy hearing about interesting patients, although maybe you could vary the cases, focusing more on human-interest situations rather than on complex technical patients. Relative to the human-interest stories, shouldn’t some of the names parents give to their children be considered child abuse? You probably don’t know, but in Iceland, there is a government Naming Committee that actually maintains a list of approved children’s names.

I know you have to take both clinical and administrative calls. When you get a medical call while we’re having dinner, would you please go somewhere else to talk? Hearing you ask about a patient’s diarrhea when we are eating sort of ruins my appetite.

Similarly, please let me vet topics before you discuss them with my friends. You have some cool stories, but Dad, I’m not sure my friends want to hear about child abuse or vaginal discharge. I will say that the absolute best phone calls you get occur when, after 22 years of Pressel Medical School, I’m able to make the diagnosis or give the correct advice (sometimes faster than your medical students and residents).

Let’s talk about what you learned from me. Though you may not agree, you should think of all the times you found me annoying, particularly when I was a pain-in-the-ass kid, as CME. Over the years, I gave you regular opportunities to enhance your knowledge of child development and to improve your parenting skills—things that undoubtedly continue to help you as a pediatrician.

I like visiting you in the hospital. I know you enjoy showing me off. When you introduce me to your coworkers, it’s OK if you tell them I’m not going to medical school. Still, you should know that I fully intend to repay all that you have done.

Hearing from you about all that happens in a hospital, I can understand why you never want to be a patient. I’ll do my best to ensure you don’t get admitted to a hospital and are able to die peacefully at home. You can count on your loving son, Dad. I’ll be sure you don’t have a hospitalist with you at the end. TH

Rob Pressel is the son of David Pressel, MD, PhD, a pediatric hospitalist at A.I. duPont Hospital for Children in Wilmington, Del., and a former member of Team Hospitalist.

As a hospitalist, patient satisfaction is top of mind. Then there’s another group of people whose satisfaction is also paramount: your family. What do they have to say about life with a hospitalist? You’re about to find out!

The Hospitalist asked family members of David Pressel, MD, PhD, a pediatric hospitalist at A.I. DuPont Hospital for Children in Wilmington, Del., and a former member of Team Hospitalist, for their impressions, and wife Karen and son Rob’s honest answers (and gentle ribbings) show that for whatever ups and downs life may bring, being part of a hospitalist’s family is full of rewards and lots of love. Of course, that’s not to say they didn’t have some suggestions for improvements.

For Hospitalists’ Spouses Everywhere

Marrying a doctor was never on my to-do list. In fact, my list specified quite the opposite; I was never going to marry a physician. My stereotypical perception of the lives of physicians included long hours, too much stress, no family time, guaranteed interruptions at social events, calls at all hours of the day, never enough sleep—you get the picture. I imagined too many headaches to make being a “doctor’s wife” in the slightest bit enticing. I wanted no part of it, and besides, I had my own career to think about.

But then I met my husband, and my list went out the window.

Still, after a couple decades of negotiating a balance between the demands of his job (see above) and the demands of his family, there are things I’d like to say to him. So here goes. Hospitalists, take note.

There Are Only 24 Hours in a Day

How many times have you called to say, “I’ll be leaving the hospital in 10 minutes”? How long did it take for me to realize that relying on that kind of statement was crazy? I’m embarrassed to say that it took me way longer than it should have to come to that understanding. After many overcooked dinners and missed social events, I finally realized that your anticipated departure held no validity and I could only trust that you had left the hospital when you called from the car with the wheels rolling. Fortunately, you were more astute than I and changed your communication habits rather quickly, although the timing of said notifications still does not always take traffic into account and could use some work.

Still, I recognize that “leaving the hospital” is really just a physical indicator of your location, not necessarily a reflection of your state of mind. When you get home, please tell me if you still have work to do (notes, email, patient follow-up) or if you are done for the day. I suppose second-guessing your clinical decisions and calling the hospital to check on patients are unavoidable, but give me a clue whether I should actually expect you fully home to join the rest of the family—or if you will just be working at the home “nursing station” all evening. The burden of healthcare in America doesn’t fall on just you. If you can’t figure out what is wrong with a patient or don’t know what to do, you have many colleagues who can help.

Please remember that you are only one person. Don’t think that if there is a staffing shortage to fill, the responsibility for working is yours. Your colleagues are wonderful and, almost without exception, are happy to pitch in to help carry the extra load. The same goes with holidays; you don’t need to work more than everyone else. I know you are not a slacker. If you try to spread the load when you manage patient care and work schedules, you will have a happier spouse. Remember, a happy wife is a happy life. (I’m sure there is an analogous saying for your colleagues’ husbands and partners.)

Along those same lines, please limit the moonlighting you choose to do. My preconceived idea of a physician’s salary was very different from your reality. You are a pediatrician, an academic pediatrician. Having said that, we lead a wonderful life. We have what we need and have been very happy without the fanciness of some of our neighbors. Although the extra income is nice, I’d rather see more of you than more money. Besides, we just wrote the last check for college tuition, and I’m sure the boys will never ask us for money again.

Being Grumpy (No, Not the Dwarf)

My thoughts on moonlighting lead me perfectly to a discussion of your frame of mind: your mood. By definition, your patients are seriously ill hospitalized children. The bursting hospital census, the acuity of your patients, and the relative craziness of some of their parents invariably elevate your stress level. This, in turn, drives more frequent calls to the hospital and time on the computer all hours of the day or night. This does not allow for a restful sleep, when you sleep at all. I may be biased, but I think you are in the minority of hospitalists who bring their jobs home. Not that I’m complaining too loudly; this is who you are and why I love you, but if you haven’t noticed, when you are on service you tend to get grumpy. Think about this: If you’re not on call, why not turn off your pager, turn off your phone, and leave email alone?

Given the pressures inherent in your job, please tell me again why you would want to moonlight. Moonlighting means even longer hours, more stress, and less sleep for you, all of which make you grumpier and, as a result, tend to make me grumpy.

No, thank you.

Everyone we know has some form of “honey-do” list, whether intended for himself or herself, a spouse, or a professional. I know it makes you feel like a competent husband and man to do things around the house, but here’s a bit of advice: Let me hire someone else. Keep in mind that contractors were invented for good reason. The aggravation you’ll have trying to fit whatever project we’ve contemplated into your schedule will be dwarfed by the aggravation I’ll have when you can’t. I’ve never heard you ruminate about not cutting the lawn after we hired the landscaper and you got rid of the lawnmower.

The same goes for quality. Do you really think you did anywhere near as good a job replacing the leaking toilet as a real plumber? Should we talk about the breakfast room light fixture? Do you want me to continue?

My annoyance probably lessened any satisfaction you derived by completing these projects yourself. You should always keep the Pressel money-management credo forefront in your mind: “You earn it, I spend it.” Please let me do my job.

Let Me See If “The Doctor” Is In

Please leave the professor at the office; don’t talk too much medicine when you are not at work. Your trainees might need to hear all the minute details of whatever medical issue is at hand, but your family and friends do not. Most of those close to us chose careers outside of medicine a long time ago and probably don’t want to change direction now. Why do you think they call me for medical advice? It’s not because I’m a better doctor but because they know they’ll hear one of two things:

1. I’ll tell them I don’t have a clue and they should ask you; or
2. I’ll answer their questions in a tenth of the time that it would have taken you. And we’re talking easy questions because, while I’ve listened to you speak to medical students and residents for the last 20 years, we both know I am not a doctor.

Nevertheless, I do pretty well even with some of the hard questions, if I say so myself. Don’t worry though, there’s no need for concern. Please know that I am not practicing with your license.

Relative to the home practice of medicine, it’s OK to look in our kids’ ears! You must remember the huge fight we had when our son exhibited all of the classic signs of an ear infection and you refused to examine his ears. I know you agonize when you make a clinical error with a patient, but this was just an ear infection. I would have taken him to a real doctor if he was sick enough to merit consideration of what you were worried about missing (brain abscess or meningitis). Really? If I had known how to work your otoscope back then, I would have looked in his ears myself. I’m still not sure how treating minor illnesses in our children is different from the same thing with children of our friends.

You have a perfectly reasonable excuse to be exhausted, yet you are often embarrassed when you fall asleep at our friends’ houses during social events. But the truth is they consider it a mark of true friendship when you go missing before dessert is served. When we were still new in the area and someone would realize that you had disappeared, I was mortified. I quickly realized though that our friends would all rather you and I join them than stay home entirely. No one is offended to find you asleep on the sofa (and your disappearance is now almost expected). To tell you the truth, I’m not sure anyone misses your conversation.

Meetings make the world go round, and your attendance is obligatory at many, even if you’d rather not attend. When I was still working, someone came up with the idea of a stand-up meeting. It was a brilliant idea that made meeting participants use the time more efficiently. Why don’t you propose that some of your administrative meetings be run that way rather than depending on me to page you, “Dr. Pressel, we need you urgently in room 23!”? Sorry I’m calling you out on this, but I’m not always available at the exact time you’ve specified that you want to be interrupted. Besides, it is sometimes amusing to hear that you fell asleep at some senior hospital administrator’s meeting.

I started this by writing that I never wanted to marry a physician, but the last quarter century with you has been the adventure of a lifetime. I just sometimes ask myself, “Why didn’t he become a dermatologist?” TH

Karen Pressel is the wife of David Pressel, MD, PhD, a pediatric hospitalist at A.I. duPont Hospital for Children in Wilmington, Del., and a former member of Team Hospitalist.

What I Want My Hospitalist Father to Know

Let me start out by saying that I think you have a great job and I am proud of you. But there are some things you should know. I’ll begin with the good ones.

We lead a very comfortable life, and I am grateful for all that you do for me. You don’t need to remind me, though, every time you manually scoop poop from some constipated kid that it pays for the roof over my head, clothes on my back, and my expensive university education.

I get it.

Even so, having a parent who is a physician is way better than having a parent who is, say, an accountant. I don’t need help with my taxes, but it sure is nice to get some quick medical advice when I have a rash. I even still trust you after you missed my broken arm when I was in sixth grade. Do me a favor though: Just tell me what it is and how I can fix the problem. Save the lecture on the pathophysiology, epidemiology, and differential diagnosis for your residents and medical students. It’s only poison ivy.

When we were growing up, you always gave us a “case of the week.” There were some consistent themes, and I’ve never been sure if these patients were real or fake. Most were either adolescent girls with belly pain or children experiencing bizarre spells who ended up being intoxicated from some ingestion. Was there supposed to be a not-so-subtle message here not to use drugs and to choose my romantic interests carefully?

I actually enjoy hearing about interesting patients, although maybe you could vary the cases, focusing more on human-interest situations rather than on complex technical patients. Relative to the human-interest stories, shouldn’t some of the names parents give to their children be considered child abuse? You probably don’t know, but in Iceland, there is a government Naming Committee that actually maintains a list of approved children’s names.

I know you have to take both clinical and administrative calls. When you get a medical call while we’re having dinner, would you please go somewhere else to talk? Hearing you ask about a patient’s diarrhea when we are eating sort of ruins my appetite.

Similarly, please let me vet topics before you discuss them with my friends. You have some cool stories, but Dad, I’m not sure my friends want to hear about child abuse or vaginal discharge. I will say that the absolute best phone calls you get occur when, after 22 years of Pressel Medical School, I’m able to make the diagnosis or give the correct advice (sometimes faster than your medical students and residents).

Let’s talk about what you learned from me. Though you may not agree, you should think of all the times you found me annoying, particularly when I was a pain-in-the-ass kid, as CME. Over the years, I gave you regular opportunities to enhance your knowledge of child development and to improve your parenting skills—things that undoubtedly continue to help you as a pediatrician.

I like visiting you in the hospital. I know you enjoy showing me off. When you introduce me to your coworkers, it’s OK if you tell them I’m not going to medical school. Still, you should know that I fully intend to repay all that you have done.

Hearing from you about all that happens in a hospital, I can understand why you never want to be a patient. I’ll do my best to ensure you don’t get admitted to a hospital and are able to die peacefully at home. You can count on your loving son, Dad. I’ll be sure you don’t have a hospitalist with you at the end. TH

Rob Pressel is the son of David Pressel, MD, PhD, a pediatric hospitalist at A.I. duPont Hospital for Children in Wilmington, Del., and a former member of Team Hospitalist.

Editor’s note: “Everything We Say and Do” is an informational series developed by SHM’s Patient Experience Committee to provide readers with thoughtful and actionable communication tactics that have great potential to positively impact patients’ experience of care. Each article will focus on how the contributor applies one or more of the “key communication” tactics in practice to maintain provider accountability for “everything we say and do that affects our patients’ thoughts, feelings, and well-being.”

View a chart outlining key communication tactics

What I Say and Do

I explain my role as a hospitalist and my connection to the patient’s primary care physician (PCP) on first meeting the patient. I look for ways to reinforce this throughout the hospitalization.

Why I Do It

Even when I was hospitalized at my own institution, it was difficult for me to remember all of the providers involved in my care and their roles. My injuries and the large number of doctors caring for me interfered with my ability to absorb this information. I imagine that this is amplified for patients who have little or no experience with the medical system and are unfamiliar with the role that we play in their care.

During a recent initiative to improve the patient experience at my institution, we found it difficult to collect specific feedback on individual providers because many patients did not know their inpatient doctors’ names, frequently referencing their PCPs when asked for feedback on their care. This is common: A 2009 study showed that 75% of patients were unable to name the inpatient physician in charge of their care. Of those who could identify a name, only 40% correctly identified a member of their primary inpatient team, often identifying the PCP or a specialist instead.1

Clarifying our role on the care team, identifying ourselves as the point person for questions or concerns, and reinforcing our relationship with the PCP can help engender trust in the relationship, eliminate confusion, and improve the patient experience.

How I Do It

After introducing myself, I explain to patients that I will notify their PCP of the admission, and I state that I will be acting as the head of the inpatient team on behalf of their PCP. I often explain that most PCPs do not see their own patients in the hospital.

When multiple teams or house staff are involved in care, I clarify my role in relation to other team members. I look for opportunities throughout the hospitalization to reinforce this. For example, I tell patients when I have updated their PCP on significant events, and I clarify my role in simple terms, such as “quarterback,” when there are multiple subspecialists involved in care. I try to avoid terms like “attending,” which are often meaningless to patients.

In my hospitalist group, we help to reinforce our role and identity by providing a business card that includes a headshot. TH

Dr. Moore is a hospitalist at Beth Israel Deaconess Medical Center and an instructor of medicine at Harvard Medical School, both in Boston. She is a member of SHM’s Patient Experience Committee.

Reference

1. Arora V, Gangireddy S, Mehrotra A, Ginde R, Tormey M, Meltzer D. Ability of hospitalized patients to identify their in-hospital physicians. Arch Intern Med. 2009;169(2):199-201.

Editor’s note: “Everything We Say and Do” is an informational series developed by SHM’s Patient Experience Committee to provide readers with thoughtful and actionable communication tactics that have great potential to positively impact patients’ experience of care. Each article will focus on how the contributor applies one or more of the “key communication” tactics in practice to maintain provider accountability for “everything we say and do that affects our patients’ thoughts, feelings, and well-being.”

View a chart outlining key communication tactics

What I Say and Do

I explain my role as a hospitalist and my connection to the patient’s primary care physician (PCP) on first meeting the patient. I look for ways to reinforce this throughout the hospitalization.

Why I Do It

Even when I was hospitalized at my own institution, it was difficult for me to remember all of the providers involved in my care and their roles. My injuries and the large number of doctors caring for me interfered with my ability to absorb this information. I imagine that this is amplified for patients who have little or no experience with the medical system and are unfamiliar with the role that we play in their care.

During a recent initiative to improve the patient experience at my institution, we found it difficult to collect specific feedback on individual providers because many patients did not know their inpatient doctors’ names, frequently referencing their PCPs when asked for feedback on their care. This is common: A 2009 study showed that 75% of patients were unable to name the inpatient physician in charge of their care. Of those who could identify a name, only 40% correctly identified a member of their primary inpatient team, often identifying the PCP or a specialist instead.1

Clarifying our role on the care team, identifying ourselves as the point person for questions or concerns, and reinforcing our relationship with the PCP can help engender trust in the relationship, eliminate confusion, and improve the patient experience.

How I Do It

After introducing myself, I explain to patients that I will notify their PCP of the admission, and I state that I will be acting as the head of the inpatient team on behalf of their PCP. I often explain that most PCPs do not see their own patients in the hospital.

When multiple teams or house staff are involved in care, I clarify my role in relation to other team members. I look for opportunities throughout the hospitalization to reinforce this. For example, I tell patients when I have updated their PCP on significant events, and I clarify my role in simple terms, such as “quarterback,” when there are multiple subspecialists involved in care. I try to avoid terms like “attending,” which are often meaningless to patients.

In my hospitalist group, we help to reinforce our role and identity by providing a business card that includes a headshot. TH

Dr. Moore is a hospitalist at Beth Israel Deaconess Medical Center and an instructor of medicine at Harvard Medical School, both in Boston. She is a member of SHM’s Patient Experience Committee.

Reference

1. Arora V, Gangireddy S, Mehrotra A, Ginde R, Tormey M, Meltzer D. Ability of hospitalized patients to identify their in-hospital physicians. Arch Intern Med. 2009;169(2):199-201.

Editor’s note: “Everything We Say and Do” is an informational series developed by SHM’s Patient Experience Committee to provide readers with thoughtful and actionable communication tactics that have great potential to positively impact patients’ experience of care. Each article will focus on how the contributor applies one or more of the “key communication” tactics in practice to maintain provider accountability for “everything we say and do that affects our patients’ thoughts, feelings, and well-being.”

View a chart outlining key communication tactics

What I Say and Do

I explain my role as a hospitalist and my connection to the patient’s primary care physician (PCP) on first meeting the patient. I look for ways to reinforce this throughout the hospitalization.

Why I Do It

Even when I was hospitalized at my own institution, it was difficult for me to remember all of the providers involved in my care and their roles. My injuries and the large number of doctors caring for me interfered with my ability to absorb this information. I imagine that this is amplified for patients who have little or no experience with the medical system and are unfamiliar with the role that we play in their care.

During a recent initiative to improve the patient experience at my institution, we found it difficult to collect specific feedback on individual providers because many patients did not know their inpatient doctors’ names, frequently referencing their PCPs when asked for feedback on their care. This is common: A 2009 study showed that 75% of patients were unable to name the inpatient physician in charge of their care. Of those who could identify a name, only 40% correctly identified a member of their primary inpatient team, often identifying the PCP or a specialist instead.1

Clarifying our role on the care team, identifying ourselves as the point person for questions or concerns, and reinforcing our relationship with the PCP can help engender trust in the relationship, eliminate confusion, and improve the patient experience.

How I Do It

After introducing myself, I explain to patients that I will notify their PCP of the admission, and I state that I will be acting as the head of the inpatient team on behalf of their PCP. I often explain that most PCPs do not see their own patients in the hospital.

When multiple teams or house staff are involved in care, I clarify my role in relation to other team members. I look for opportunities throughout the hospitalization to reinforce this. For example, I tell patients when I have updated their PCP on significant events, and I clarify my role in simple terms, such as “quarterback,” when there are multiple subspecialists involved in care. I try to avoid terms like “attending,” which are often meaningless to patients.

In my hospitalist group, we help to reinforce our role and identity by providing a business card that includes a headshot. TH

Dr. Moore is a hospitalist at Beth Israel Deaconess Medical Center and an instructor of medicine at Harvard Medical School, both in Boston. She is a member of SHM’s Patient Experience Committee.

Reference

1. Arora V, Gangireddy S, Mehrotra A, Ginde R, Tormey M, Meltzer D. Ability of hospitalized patients to identify their in-hospital physicians. Arch Intern Med. 2009;169(2):199-201.

Doctor consults with cancer

patient and her father

Photo by Rhoda Baer

Adolescents and young adults (AYAs) are less likely than children to survive 8 relatively common types of cancer, according to a long-running study of cancer survival across Europe.

The study showed that AYAs had significantly worse survival rates than children if they were diagnosed with acute lymphoblastic leukemia (ALL), acute myeloid leukemia (AML), Hodgkin or non-Hodgkin lymphoma (NHL), and 4 types of solid tumor malignancies.

The study’s authors say that variations in survival between age groups are due to a number of factors, including delays in diagnosis and treatment, a lack of treatment guidelines and clinical trials specifically for AYAs, and differences in the biology of some cancers.

“The good news is that the number of children, adolescents, and young adults surviving for at least 5 years after diagnosis has risen steadily over time in Europe,” said author Annalisa Trama, PhD, of The National Institute of Cancer (Istituto Nazionale dei Tumori: Fondazione IRCCS) in Milan, Italy.

“Across all cancers, the level of improvement is similar in these age groups. This contrasts with earlier results that adolescents and young adults diagnosed up to the 1990s were lagging behind children in terms of survival.”

“However, we found that adolescents and young adults still tend to die earlier than children for several cancers common to these age groups, particularly blood cancers like leukemias and non-Hodgkin’s lymphoma.”

Dr Trama and her colleagues reported these findings in The Lancet Oncology.

The researchers compared survival between AYAs (ages 15 to 39), children (ages 0 to 14), and adults (ages 40 to 69) who were diagnosed from 2000 to 2007 and followed up to at least 2008.

The team analyzed data from population-based cancer registries covering all or part of 27 European countries* and estimated 5-year survival for 56,505 cancer cases in children; 312,483 in AYAs; and 3,567,383 in adults. The researchers also analyzed changes in survival over time from 1999 to 2007.

For AYAs, survival at 5 years from diagnosis for all cancers combined was 82% for 2005-2007, which is up from 79% for 1999-2001 (P<0.0001). In children, survival improved from 76% to 79% over the same time period (P<0.0001).

Survival improved significantly in children and AYAs for ALL (P<0.0001) and NHL (P<0.0001 in AYAs and P=0.023 in children). On the other hand, between 1999 and 2007, survival rates remained unchanged for AYAs with AML (around 50%).

Overall, AYAs had slightly better 5-year survival than children because they were diagnosed more often with cancers with fairly good prognoses—Hodgkin lymphoma, NHL, germ cell tumors, melanoma, thyroid cancer, and breast cancer.

However, the overall survival rates conceal differences between specific cancers. Survival was significantly worse for AYAs than for children when it came to 8 relatively common cancers affecting both age groups:

• ALL—55.6% for AYAs and 85.8% for children (P<0.0001)
• AML—49.8% and 60.5%, respectively (P<0.0001)
• Hodgkin lymphoma—92.9% and 95.1%, respectively (P<0.0001)
• NHL—77.4% and 83.0%, respectively (P<0.0001)
• Astrocytomas—46.4% and 61.9%, respectively (P<0.0001)
• Ewing’s sarcoma of bone—49.3% and 66.6%, respectively (P<0.0001)
• Rhabdomyosarcoma—37.8% and 66.6%, respectively (P<0.0001)
• Osteosarcoma—61.5% and 66.8%, respectively (P=0.011).

AYAs had a survival advantage over adults for almost all major cancers affecting both age groups, supporting the idea that younger patients with few other illnesses are likely to fare better than older patients.

There are only 2 types of cancer for which AYAs were at a survival disadvantage—breast (83.5% vs 87.0%) and prostate (79.9% vs 89.8%).

Dr Trama and her colleagues pointed out that this analysis pre-dates recent initiatives to improve outcomes for AYAs that have been implemented in several European countries.

“The European Network for Teenagers and Young Adults with Cancer is advocating collaboration between pediatric and adult oncologists, greater access to clinical trials and research to improve treatments for this specific age group, as well as developing adolescent and young adult-specific practice guidelines, encouraging healthier lifestyles and the greater involvement of patients and patients support groups,” Dr Trama said.

“This study will provide an important starting point from which to evaluate whether these initiatives will reduce the gulf in survival between European adolescents and young adults and children with cancer.”

*Finland, Iceland, Norway, Sweden, England, Ireland, Northern Ireland, Scotland, Wales, Austria, Belgium, France, Germany, Netherlands, Switzerland, Croatia, Italy, Malta, Portugal, Slovenia, Spain, Bulgaria, Estonia, Latvia, Lithuania, Poland, and Slovakia

Doctor consults with cancer

patient and her father

Photo by Rhoda Baer

Adolescents and young adults (AYAs) are less likely than children to survive 8 relatively common types of cancer, according to a long-running study of cancer survival across Europe.

The study showed that AYAs had significantly worse survival rates than children if they were diagnosed with acute lymphoblastic leukemia (ALL), acute myeloid leukemia (AML), Hodgkin or non-Hodgkin lymphoma (NHL), and 4 types of solid tumor malignancies.

The study’s authors say that variations in survival between age groups are due to a number of factors, including delays in diagnosis and treatment, a lack of treatment guidelines and clinical trials specifically for AYAs, and differences in the biology of some cancers.

“The good news is that the number of children, adolescents, and young adults surviving for at least 5 years after diagnosis has risen steadily over time in Europe,” said author Annalisa Trama, PhD, of The National Institute of Cancer (Istituto Nazionale dei Tumori: Fondazione IRCCS) in Milan, Italy.

“Across all cancers, the level of improvement is similar in these age groups. This contrasts with earlier results that adolescents and young adults diagnosed up to the 1990s were lagging behind children in terms of survival.”

“However, we found that adolescents and young adults still tend to die earlier than children for several cancers common to these age groups, particularly blood cancers like leukemias and non-Hodgkin’s lymphoma.”

Dr Trama and her colleagues reported these findings in The Lancet Oncology.

The researchers compared survival between AYAs (ages 15 to 39), children (ages 0 to 14), and adults (ages 40 to 69) who were diagnosed from 2000 to 2007 and followed up to at least 2008.

The team analyzed data from population-based cancer registries covering all or part of 27 European countries* and estimated 5-year survival for 56,505 cancer cases in children; 312,483 in AYAs; and 3,567,383 in adults. The researchers also analyzed changes in survival over time from 1999 to 2007.

For AYAs, survival at 5 years from diagnosis for all cancers combined was 82% for 2005-2007, which is up from 79% for 1999-2001 (P<0.0001). In children, survival improved from 76% to 79% over the same time period (P<0.0001).

Survival improved significantly in children and AYAs for ALL (P<0.0001) and NHL (P<0.0001 in AYAs and P=0.023 in children). On the other hand, between 1999 and 2007, survival rates remained unchanged for AYAs with AML (around 50%).

Overall, AYAs had slightly better 5-year survival than children because they were diagnosed more often with cancers with fairly good prognoses—Hodgkin lymphoma, NHL, germ cell tumors, melanoma, thyroid cancer, and breast cancer.

However, the overall survival rates conceal differences between specific cancers. Survival was significantly worse for AYAs than for children when it came to 8 relatively common cancers affecting both age groups:

• ALL—55.6% for AYAs and 85.8% for children (P<0.0001)
• AML—49.8% and 60.5%, respectively (P<0.0001)
• Hodgkin lymphoma—92.9% and 95.1%, respectively (P<0.0001)
• NHL—77.4% and 83.0%, respectively (P<0.0001)
• Astrocytomas—46.4% and 61.9%, respectively (P<0.0001)
• Ewing’s sarcoma of bone—49.3% and 66.6%, respectively (P<0.0001)
• Rhabdomyosarcoma—37.8% and 66.6%, respectively (P<0.0001)
• Osteosarcoma—61.5% and 66.8%, respectively (P=0.011).

AYAs had a survival advantage over adults for almost all major cancers affecting both age groups, supporting the idea that younger patients with few other illnesses are likely to fare better than older patients.

There are only 2 types of cancer for which AYAs were at a survival disadvantage—breast (83.5% vs 87.0%) and prostate (79.9% vs 89.8%).

Dr Trama and her colleagues pointed out that this analysis pre-dates recent initiatives to improve outcomes for AYAs that have been implemented in several European countries.

“The European Network for Teenagers and Young Adults with Cancer is advocating collaboration between pediatric and adult oncologists, greater access to clinical trials and research to improve treatments for this specific age group, as well as developing adolescent and young adult-specific practice guidelines, encouraging healthier lifestyles and the greater involvement of patients and patients support groups,” Dr Trama said.

“This study will provide an important starting point from which to evaluate whether these initiatives will reduce the gulf in survival between European adolescents and young adults and children with cancer.”

*Finland, Iceland, Norway, Sweden, England, Ireland, Northern Ireland, Scotland, Wales, Austria, Belgium, France, Germany, Netherlands, Switzerland, Croatia, Italy, Malta, Portugal, Slovenia, Spain, Bulgaria, Estonia, Latvia, Lithuania, Poland, and Slovakia

Doctor consults with cancer

patient and her father

Photo by Rhoda Baer

Adolescents and young adults (AYAs) are less likely than children to survive 8 relatively common types of cancer, according to a long-running study of cancer survival across Europe.

The study showed that AYAs had significantly worse survival rates than children if they were diagnosed with acute lymphoblastic leukemia (ALL), acute myeloid leukemia (AML), Hodgkin or non-Hodgkin lymphoma (NHL), and 4 types of solid tumor malignancies.

The study’s authors say that variations in survival between age groups are due to a number of factors, including delays in diagnosis and treatment, a lack of treatment guidelines and clinical trials specifically for AYAs, and differences in the biology of some cancers.

“The good news is that the number of children, adolescents, and young adults surviving for at least 5 years after diagnosis has risen steadily over time in Europe,” said author Annalisa Trama, PhD, of The National Institute of Cancer (Istituto Nazionale dei Tumori: Fondazione IRCCS) in Milan, Italy.

“Across all cancers, the level of improvement is similar in these age groups. This contrasts with earlier results that adolescents and young adults diagnosed up to the 1990s were lagging behind children in terms of survival.”

“However, we found that adolescents and young adults still tend to die earlier than children for several cancers common to these age groups, particularly blood cancers like leukemias and non-Hodgkin’s lymphoma.”

Dr Trama and her colleagues reported these findings in The Lancet Oncology.

The researchers compared survival between AYAs (ages 15 to 39), children (ages 0 to 14), and adults (ages 40 to 69) who were diagnosed from 2000 to 2007 and followed up to at least 2008.

The team analyzed data from population-based cancer registries covering all or part of 27 European countries* and estimated 5-year survival for 56,505 cancer cases in children; 312,483 in AYAs; and 3,567,383 in adults. The researchers also analyzed changes in survival over time from 1999 to 2007.

For AYAs, survival at 5 years from diagnosis for all cancers combined was 82% for 2005-2007, which is up from 79% for 1999-2001 (P<0.0001). In children, survival improved from 76% to 79% over the same time period (P<0.0001).

Survival improved significantly in children and AYAs for ALL (P<0.0001) and NHL (P<0.0001 in AYAs and P=0.023 in children). On the other hand, between 1999 and 2007, survival rates remained unchanged for AYAs with AML (around 50%).

Overall, AYAs had slightly better 5-year survival than children because they were diagnosed more often with cancers with fairly good prognoses—Hodgkin lymphoma, NHL, germ cell tumors, melanoma, thyroid cancer, and breast cancer.

However, the overall survival rates conceal differences between specific cancers. Survival was significantly worse for AYAs than for children when it came to 8 relatively common cancers affecting both age groups:

• ALL—55.6% for AYAs and 85.8% for children (P<0.0001)
• AML—49.8% and 60.5%, respectively (P<0.0001)
• Hodgkin lymphoma—92.9% and 95.1%, respectively (P<0.0001)
• NHL—77.4% and 83.0%, respectively (P<0.0001)
• Astrocytomas—46.4% and 61.9%, respectively (P<0.0001)
• Ewing’s sarcoma of bone—49.3% and 66.6%, respectively (P<0.0001)
• Rhabdomyosarcoma—37.8% and 66.6%, respectively (P<0.0001)
• Osteosarcoma—61.5% and 66.8%, respectively (P=0.011).

AYAs had a survival advantage over adults for almost all major cancers affecting both age groups, supporting the idea that younger patients with few other illnesses are likely to fare better than older patients.

There are only 2 types of cancer for which AYAs were at a survival disadvantage—breast (83.5% vs 87.0%) and prostate (79.9% vs 89.8%).

Dr Trama and her colleagues pointed out that this analysis pre-dates recent initiatives to improve outcomes for AYAs that have been implemented in several European countries.

“The European Network for Teenagers and Young Adults with Cancer is advocating collaboration between pediatric and adult oncologists, greater access to clinical trials and research to improve treatments for this specific age group, as well as developing adolescent and young adult-specific practice guidelines, encouraging healthier lifestyles and the greater involvement of patients and patients support groups,” Dr Trama said.

“This study will provide an important starting point from which to evaluate whether these initiatives will reduce the gulf in survival between European adolescents and young adults and children with cancer.”

*Finland, Iceland, Norway, Sweden, England, Ireland, Northern Ireland, Scotland, Wales, Austria, Belgium, France, Germany, Netherlands, Switzerland, Croatia, Italy, Malta, Portugal, Slovenia, Spain, Bulgaria, Estonia, Latvia, Lithuania, Poland, and Slovakia

THE CASE

A 17-year-old boy presented to the emergency department (ED) with a headache, dizziness, lethargy, and weakness that he’d had for 2 weeks. The patient was taking a selective serotonin reuptake inhibitor (SSRI) for depression (sertraline 25 mg/d). He had been vomiting twice daily for the past 3 years. (Although he had been seen multiple times in urgent care clinics, he did not have regular medical care.) The boy was fatigued and had dark yellow urine. His father indicated that his son’s skin had darkened over the last 5 to 6 years and that he had been adding salt, in large quantities, to nearly all of his meals for 10 years.

The boy’s health issues were impacting his school life. He was dismissed from school often because his teachers felt he was skipping class and using the excuse of needing to urinate or vomit. He had traveled back and forth to Mexico about 2 times a year, with the last time being about 3 months before his trip to the ED.

The patient’s vitals included a temperature of 96.3º F, heart rate (HR) of 77 beats/min, respiratory rate of 16 breaths/min, and a supine blood pressure (BP) of 102/58 mm Hg. (The patient’s BP was not obtained when sitting or standing, because he felt dizzy when trying to stand or sit up and the HR monitor increased to 100 beats/min.) His weight was 106.9 pounds and height was 5 feet 8 inches. The teen was ill-appearing and somnolent. No jugular vein distention, murmurs, or gallops were noted on exam. The patient’s lips were dry and cracked, gums were darkened, and his skin was clammy to the touch. His abdomen was soft with hypoactive bowel sounds and no ascites. His extremities were non-edematous.

A chemistry panel showed a low sodium level of 99 mEq/L, a somewhat high potassium level of 5.2 mmol/L, low chloride (69 mEq/L) and CO₂ (5 mEq/L) levels, a high glucose level (124 mg/dL), and normal creatinine (0.79 mg/dL), albumin (5.2 g/dL), and thyroid stimulating hormone (2.4 mIU/L) levels. A tuberculosis (TB) test, acute hepatitis panel, human immunodeficiency test, and urine drug screen were all negative. Liver enzymes and lipase levels were normal.

The patient was admitted to the pediatric intensive care unit (PICU) on 200 mL/hr normal saline (twice the normal maintenance rate) and we took over his care.

THE DIAGNOSIS

Because of the patient’s severe hyponatremia, the differential diagnosis included heart failure, cirrhosis, syndrome of inappropriate antidiuretic hormone secretion (SIADH), SSRI-induced SIADH, cerebral salt wasting, severe hypothyroidism, adrenal insufficiency, malignancies, ecstasy use, renal failure, low dietary solute intake, and psychogenic polydipsia.

A random cortisol test taken in the ED returned and was noted to be very low (<1 mcg/dL). This information, plus the signs of aldosterone deficiency (low sodium and elevated potassium levels) and adrenocorticotropic hormone (ACTH) excess (skin darkening), prompted us to perform a 250-mcg ACTH stimulation test. Results at 30 and 60 minutes both showed cortisol at <1 mcg/dL, which led us to suspect adrenal insufficiency. The diagnosis of autoimmune adrenalitis, or Addison’s disease, was confirmed after inpatient lab work returned with positive 21-hydroxylase antibodies and an elevated ACTH (1117 pg/mL; normal, 10-65 pg/mL).

We noted that the patient’s sodium level was gradually increasing while he was receiving the intravenous (IV) fluids. We were concerned, though, that too rapid a sodium correction would put the patient at risk for central pontine myelinolysis (CPM). So we held off on steroids until 24 hours after he was admitted to the PICU, when his sodium level reached 110 mEq/L.

DISCUSSION

The first evidence of Addison’s disease is usually an increase in plasma renin activity with low serum aldosterone.

Primary adrenal insufficiency in the developed world is commonly caused by autoimmune adrenalitis, also known as Addison’s disease. Addison’s disease is the cause of primary adrenal insufficiency in 70% to 90% of cases, with the remainder caused by TB, adrenal hemorrhage, infarction, lymphoma, cytomegalovirus, adrenoleukodystrophy, or metastatic cancer. We also considered adrenoleukodystrophy in our patient, but felt it unlikely in a 17-year-old with normal mental status and positive adrenal antibodies.

The first evidence of Addison’s disease is usually an increase in plasma renin activity with low serum aldosterone. This might explain our patient’s years of salt cravings prior to presentation. There is typically a decrease in serum cortisol response to ACTH stimulation several months to years after the onset of salt cravings. The next sign of deterioration in adrenal function is an increase in basal serum ACTH; the process concludes with a decreased basal serum cortisol level.^1-3 By the time our patient presented to the ED, his ACTH was very high, his cortisol was low, and his ACTH stimulation response was low.

Acute adrenal insufficiency crisis usually occurs after a prolonged period of nonspecific complaints due to a loss of both glucocorticoids and mineralocorticoids; by the time overt symptoms occur, 90% of the adrenal gland may be destroyed.³ Patients (such as ours) may present with symptoms such as abdominal pain, weakness, vomiting, fever, and decreased responsiveness. Hyponatremia and hyperkalemia are commonly seen at initial diagnosis. BP can be compromised in some patients due to loss of vascular tone; our patient did not present with this finding.

Treatment includes hydrocortisone and fludrocortisone for life

Initial management focuses on rehydration, maintenance of BP, cardiac monitoring, and electrolyte monitoring with a focus on slow normalization of electrolyte abnormalities. Patients should be treated with hydrocortisone (approximately 10 mg/m²/d) and fludrocortisone (usually 0.1 mg/d), and they will be maintained on this regimen for life.^1,3

During acute illness, the doses of hydrocortisone are usually tripled and given 3 times per day to address the increased cortisol needs of the stress response. Lack of stress dose steroids in the setting of illness can lead to repeat adrenal crisis events.

Patients should be taught about intramuscular (IM) hydrocortisone use (100 mg IM) for emergencies and should have medical identification. In many states, emergency medical technicians (EMTs) are now able to administer the patient’s own supply of hydrocortisone. EMTs have even begun carrying hydrocortisone in some states in response to a campaign by the CARES Foundation, a nonprofit organization dedicated to helping families and individuals affected by congenital adrenal hyperplasia.

We started our patient on 100 mg/m²/d hydrocortisone 24 hours after he was admitted to the PICU. (At that time, his sodium level was 110 mEq/L.) Forty-eight hours after admission, we started the patient on fludrocortisone for mineralocorticoid effect at 0.1 mg/d. (The patient’s sodium level was 122 mEq/L). At 72 hours after admission, the patient’s sodium level was 137 mEq/L and his mental status was normal. Normal saline was discontinued when sodium normalized. He was discharged 2 days later. He was informed he should continue these medications for life, though doses might be adjusted slightly with time.

Two weeks later, our patient’s sodium level had reached 141 mEq/L and his weight loss, depression, vomiting, and fatigue had resolved. He stopped taking his SSRI. He was still craving extra salt, but not as much, and his urine was no longer a very dark yellow.

In retrospect, starting this patient on steroids earlier may not have resulted in any more of a rapid sodium rise than that which occurred otherwise, but we believe that our concern for CPM at that time justified the delay in steroid use. We felt it was safe to delay steroids because the patient’s BP was stable and his clinical picture was rapidly improving. In most cases, however, delaying steroids is not advisable.

THE TAKEAWAY

Adrenal insufficiency can be clearly diagnosed via labs and clinical presentation, and is potentially lethal if unrecognized. The predominant manifestations of adrenal crisis are hypotension and shock, usually with hyponatremia and hyperkalemia. During stressful events or illness, patients should increase their glucocorticoid dose. If they are on hydrocortisone, instructions are usually to triple the dose, and give the medication 3 times a day. Patients require instruction beforehand on how and when to increase doses for illness so that they can handle this on their own. Patients should carry a medical identification card so that their condition is evident to anyone caring for them in the ED.

THE CASE

A 17-year-old boy presented to the emergency department (ED) with a headache, dizziness, lethargy, and weakness that he’d had for 2 weeks. The patient was taking a selective serotonin reuptake inhibitor (SSRI) for depression (sertraline 25 mg/d). He had been vomiting twice daily for the past 3 years. (Although he had been seen multiple times in urgent care clinics, he did not have regular medical care.) The boy was fatigued and had dark yellow urine. His father indicated that his son’s skin had darkened over the last 5 to 6 years and that he had been adding salt, in large quantities, to nearly all of his meals for 10 years.

The boy’s health issues were impacting his school life. He was dismissed from school often because his teachers felt he was skipping class and using the excuse of needing to urinate or vomit. He had traveled back and forth to Mexico about 2 times a year, with the last time being about 3 months before his trip to the ED.

The patient’s vitals included a temperature of 96.3º F, heart rate (HR) of 77 beats/min, respiratory rate of 16 breaths/min, and a supine blood pressure (BP) of 102/58 mm Hg. (The patient’s BP was not obtained when sitting or standing, because he felt dizzy when trying to stand or sit up and the HR monitor increased to 100 beats/min.) His weight was 106.9 pounds and height was 5 feet 8 inches. The teen was ill-appearing and somnolent. No jugular vein distention, murmurs, or gallops were noted on exam. The patient’s lips were dry and cracked, gums were darkened, and his skin was clammy to the touch. His abdomen was soft with hypoactive bowel sounds and no ascites. His extremities were non-edematous.

A chemistry panel showed a low sodium level of 99 mEq/L, a somewhat high potassium level of 5.2 mmol/L, low chloride (69 mEq/L) and CO₂ (5 mEq/L) levels, a high glucose level (124 mg/dL), and normal creatinine (0.79 mg/dL), albumin (5.2 g/dL), and thyroid stimulating hormone (2.4 mIU/L) levels. A tuberculosis (TB) test, acute hepatitis panel, human immunodeficiency test, and urine drug screen were all negative. Liver enzymes and lipase levels were normal.

The patient was admitted to the pediatric intensive care unit (PICU) on 200 mL/hr normal saline (twice the normal maintenance rate) and we took over his care.

THE DIAGNOSIS

Because of the patient’s severe hyponatremia, the differential diagnosis included heart failure, cirrhosis, syndrome of inappropriate antidiuretic hormone secretion (SIADH), SSRI-induced SIADH, cerebral salt wasting, severe hypothyroidism, adrenal insufficiency, malignancies, ecstasy use, renal failure, low dietary solute intake, and psychogenic polydipsia.

A random cortisol test taken in the ED returned and was noted to be very low (<1 mcg/dL). This information, plus the signs of aldosterone deficiency (low sodium and elevated potassium levels) and adrenocorticotropic hormone (ACTH) excess (skin darkening), prompted us to perform a 250-mcg ACTH stimulation test. Results at 30 and 60 minutes both showed cortisol at <1 mcg/dL, which led us to suspect adrenal insufficiency. The diagnosis of autoimmune adrenalitis, or Addison’s disease, was confirmed after inpatient lab work returned with positive 21-hydroxylase antibodies and an elevated ACTH (1117 pg/mL; normal, 10-65 pg/mL).

We noted that the patient’s sodium level was gradually increasing while he was receiving the intravenous (IV) fluids. We were concerned, though, that too rapid a sodium correction would put the patient at risk for central pontine myelinolysis (CPM). So we held off on steroids until 24 hours after he was admitted to the PICU, when his sodium level reached 110 mEq/L.

DISCUSSION

The first evidence of Addison’s disease is usually an increase in plasma renin activity with low serum aldosterone.

Primary adrenal insufficiency in the developed world is commonly caused by autoimmune adrenalitis, also known as Addison’s disease. Addison’s disease is the cause of primary adrenal insufficiency in 70% to 90% of cases, with the remainder caused by TB, adrenal hemorrhage, infarction, lymphoma, cytomegalovirus, adrenoleukodystrophy, or metastatic cancer. We also considered adrenoleukodystrophy in our patient, but felt it unlikely in a 17-year-old with normal mental status and positive adrenal antibodies.

The first evidence of Addison’s disease is usually an increase in plasma renin activity with low serum aldosterone. This might explain our patient’s years of salt cravings prior to presentation. There is typically a decrease in serum cortisol response to ACTH stimulation several months to years after the onset of salt cravings. The next sign of deterioration in adrenal function is an increase in basal serum ACTH; the process concludes with a decreased basal serum cortisol level.^1-3 By the time our patient presented to the ED, his ACTH was very high, his cortisol was low, and his ACTH stimulation response was low.

Acute adrenal insufficiency crisis usually occurs after a prolonged period of nonspecific complaints due to a loss of both glucocorticoids and mineralocorticoids; by the time overt symptoms occur, 90% of the adrenal gland may be destroyed.³ Patients (such as ours) may present with symptoms such as abdominal pain, weakness, vomiting, fever, and decreased responsiveness. Hyponatremia and hyperkalemia are commonly seen at initial diagnosis. BP can be compromised in some patients due to loss of vascular tone; our patient did not present with this finding.

Treatment includes hydrocortisone and fludrocortisone for life

Initial management focuses on rehydration, maintenance of BP, cardiac monitoring, and electrolyte monitoring with a focus on slow normalization of electrolyte abnormalities. Patients should be treated with hydrocortisone (approximately 10 mg/m²/d) and fludrocortisone (usually 0.1 mg/d), and they will be maintained on this regimen for life.^1,3

During acute illness, the doses of hydrocortisone are usually tripled and given 3 times per day to address the increased cortisol needs of the stress response. Lack of stress dose steroids in the setting of illness can lead to repeat adrenal crisis events.

Patients should be taught about intramuscular (IM) hydrocortisone use (100 mg IM) for emergencies and should have medical identification. In many states, emergency medical technicians (EMTs) are now able to administer the patient’s own supply of hydrocortisone. EMTs have even begun carrying hydrocortisone in some states in response to a campaign by the CARES Foundation, a nonprofit organization dedicated to helping families and individuals affected by congenital adrenal hyperplasia.

We started our patient on 100 mg/m²/d hydrocortisone 24 hours after he was admitted to the PICU. (At that time, his sodium level was 110 mEq/L.) Forty-eight hours after admission, we started the patient on fludrocortisone for mineralocorticoid effect at 0.1 mg/d. (The patient’s sodium level was 122 mEq/L). At 72 hours after admission, the patient’s sodium level was 137 mEq/L and his mental status was normal. Normal saline was discontinued when sodium normalized. He was discharged 2 days later. He was informed he should continue these medications for life, though doses might be adjusted slightly with time.

Two weeks later, our patient’s sodium level had reached 141 mEq/L and his weight loss, depression, vomiting, and fatigue had resolved. He stopped taking his SSRI. He was still craving extra salt, but not as much, and his urine was no longer a very dark yellow.

In retrospect, starting this patient on steroids earlier may not have resulted in any more of a rapid sodium rise than that which occurred otherwise, but we believe that our concern for CPM at that time justified the delay in steroid use. We felt it was safe to delay steroids because the patient’s BP was stable and his clinical picture was rapidly improving. In most cases, however, delaying steroids is not advisable.

THE TAKEAWAY

Adrenal insufficiency can be clearly diagnosed via labs and clinical presentation, and is potentially lethal if unrecognized. The predominant manifestations of adrenal crisis are hypotension and shock, usually with hyponatremia and hyperkalemia. During stressful events or illness, patients should increase their glucocorticoid dose. If they are on hydrocortisone, instructions are usually to triple the dose, and give the medication 3 times a day. Patients require instruction beforehand on how and when to increase doses for illness so that they can handle this on their own. Patients should carry a medical identification card so that their condition is evident to anyone caring for them in the ED.

THE CASE

A 17-year-old boy presented to the emergency department (ED) with a headache, dizziness, lethargy, and weakness that he’d had for 2 weeks. The patient was taking a selective serotonin reuptake inhibitor (SSRI) for depression (sertraline 25 mg/d). He had been vomiting twice daily for the past 3 years. (Although he had been seen multiple times in urgent care clinics, he did not have regular medical care.) The boy was fatigued and had dark yellow urine. His father indicated that his son’s skin had darkened over the last 5 to 6 years and that he had been adding salt, in large quantities, to nearly all of his meals for 10 years.

The boy’s health issues were impacting his school life. He was dismissed from school often because his teachers felt he was skipping class and using the excuse of needing to urinate or vomit. He had traveled back and forth to Mexico about 2 times a year, with the last time being about 3 months before his trip to the ED.

The patient’s vitals included a temperature of 96.3º F, heart rate (HR) of 77 beats/min, respiratory rate of 16 breaths/min, and a supine blood pressure (BP) of 102/58 mm Hg. (The patient’s BP was not obtained when sitting or standing, because he felt dizzy when trying to stand or sit up and the HR monitor increased to 100 beats/min.) His weight was 106.9 pounds and height was 5 feet 8 inches. The teen was ill-appearing and somnolent. No jugular vein distention, murmurs, or gallops were noted on exam. The patient’s lips were dry and cracked, gums were darkened, and his skin was clammy to the touch. His abdomen was soft with hypoactive bowel sounds and no ascites. His extremities were non-edematous.

A chemistry panel showed a low sodium level of 99 mEq/L, a somewhat high potassium level of 5.2 mmol/L, low chloride (69 mEq/L) and CO₂ (5 mEq/L) levels, a high glucose level (124 mg/dL), and normal creatinine (0.79 mg/dL), albumin (5.2 g/dL), and thyroid stimulating hormone (2.4 mIU/L) levels. A tuberculosis (TB) test, acute hepatitis panel, human immunodeficiency test, and urine drug screen were all negative. Liver enzymes and lipase levels were normal.

The patient was admitted to the pediatric intensive care unit (PICU) on 200 mL/hr normal saline (twice the normal maintenance rate) and we took over his care.

THE DIAGNOSIS

Because of the patient’s severe hyponatremia, the differential diagnosis included heart failure, cirrhosis, syndrome of inappropriate antidiuretic hormone secretion (SIADH), SSRI-induced SIADH, cerebral salt wasting, severe hypothyroidism, adrenal insufficiency, malignancies, ecstasy use, renal failure, low dietary solute intake, and psychogenic polydipsia.

A random cortisol test taken in the ED returned and was noted to be very low (<1 mcg/dL). This information, plus the signs of aldosterone deficiency (low sodium and elevated potassium levels) and adrenocorticotropic hormone (ACTH) excess (skin darkening), prompted us to perform a 250-mcg ACTH stimulation test. Results at 30 and 60 minutes both showed cortisol at <1 mcg/dL, which led us to suspect adrenal insufficiency. The diagnosis of autoimmune adrenalitis, or Addison’s disease, was confirmed after inpatient lab work returned with positive 21-hydroxylase antibodies and an elevated ACTH (1117 pg/mL; normal, 10-65 pg/mL).

We noted that the patient’s sodium level was gradually increasing while he was receiving the intravenous (IV) fluids. We were concerned, though, that too rapid a sodium correction would put the patient at risk for central pontine myelinolysis (CPM). So we held off on steroids until 24 hours after he was admitted to the PICU, when his sodium level reached 110 mEq/L.

DISCUSSION

The first evidence of Addison’s disease is usually an increase in plasma renin activity with low serum aldosterone.

Primary adrenal insufficiency in the developed world is commonly caused by autoimmune adrenalitis, also known as Addison’s disease. Addison’s disease is the cause of primary adrenal insufficiency in 70% to 90% of cases, with the remainder caused by TB, adrenal hemorrhage, infarction, lymphoma, cytomegalovirus, adrenoleukodystrophy, or metastatic cancer. We also considered adrenoleukodystrophy in our patient, but felt it unlikely in a 17-year-old with normal mental status and positive adrenal antibodies.

The first evidence of Addison’s disease is usually an increase in plasma renin activity with low serum aldosterone. This might explain our patient’s years of salt cravings prior to presentation. There is typically a decrease in serum cortisol response to ACTH stimulation several months to years after the onset of salt cravings. The next sign of deterioration in adrenal function is an increase in basal serum ACTH; the process concludes with a decreased basal serum cortisol level.^1-3 By the time our patient presented to the ED, his ACTH was very high, his cortisol was low, and his ACTH stimulation response was low.

Acute adrenal insufficiency crisis usually occurs after a prolonged period of nonspecific complaints due to a loss of both glucocorticoids and mineralocorticoids; by the time overt symptoms occur, 90% of the adrenal gland may be destroyed.³ Patients (such as ours) may present with symptoms such as abdominal pain, weakness, vomiting, fever, and decreased responsiveness. Hyponatremia and hyperkalemia are commonly seen at initial diagnosis. BP can be compromised in some patients due to loss of vascular tone; our patient did not present with this finding.

Treatment includes hydrocortisone and fludrocortisone for life

Initial management focuses on rehydration, maintenance of BP, cardiac monitoring, and electrolyte monitoring with a focus on slow normalization of electrolyte abnormalities. Patients should be treated with hydrocortisone (approximately 10 mg/m²/d) and fludrocortisone (usually 0.1 mg/d), and they will be maintained on this regimen for life.^1,3

During acute illness, the doses of hydrocortisone are usually tripled and given 3 times per day to address the increased cortisol needs of the stress response. Lack of stress dose steroids in the setting of illness can lead to repeat adrenal crisis events.

Patients should be taught about intramuscular (IM) hydrocortisone use (100 mg IM) for emergencies and should have medical identification. In many states, emergency medical technicians (EMTs) are now able to administer the patient’s own supply of hydrocortisone. EMTs have even begun carrying hydrocortisone in some states in response to a campaign by the CARES Foundation, a nonprofit organization dedicated to helping families and individuals affected by congenital adrenal hyperplasia.

We started our patient on 100 mg/m²/d hydrocortisone 24 hours after he was admitted to the PICU. (At that time, his sodium level was 110 mEq/L.) Forty-eight hours after admission, we started the patient on fludrocortisone for mineralocorticoid effect at 0.1 mg/d. (The patient’s sodium level was 122 mEq/L). At 72 hours after admission, the patient’s sodium level was 137 mEq/L and his mental status was normal. Normal saline was discontinued when sodium normalized. He was discharged 2 days later. He was informed he should continue these medications for life, though doses might be adjusted slightly with time.

Two weeks later, our patient’s sodium level had reached 141 mEq/L and his weight loss, depression, vomiting, and fatigue had resolved. He stopped taking his SSRI. He was still craving extra salt, but not as much, and his urine was no longer a very dark yellow.

In retrospect, starting this patient on steroids earlier may not have resulted in any more of a rapid sodium rise than that which occurred otherwise, but we believe that our concern for CPM at that time justified the delay in steroid use. We felt it was safe to delay steroids because the patient’s BP was stable and his clinical picture was rapidly improving. In most cases, however, delaying steroids is not advisable.

THE TAKEAWAY

Adrenal insufficiency can be clearly diagnosed via labs and clinical presentation, and is potentially lethal if unrecognized. The predominant manifestations of adrenal crisis are hypotension and shock, usually with hyponatremia and hyperkalemia. During stressful events or illness, patients should increase their glucocorticoid dose. If they are on hydrocortisone, instructions are usually to triple the dose, and give the medication 3 times a day. Patients require instruction beforehand on how and when to increase doses for illness so that they can handle this on their own. Patients should carry a medical identification card so that their condition is evident to anyone caring for them in the ED.

THE CASE

The mother of a 13-year-old girl brought her daughter to our family medicine clinic for follow-up after being seen in the emergency department (ED) 3 days earlier. The girl had presented to the ED with a one-day history of back, chest, and vaginal pain. She was diagnosed with a urinary tract infection and treated empirically with phenazopyridine and cephalexin pending a urine culture.

During the follow-up appointment, the patient complained of worsening vaginal pain and increased vaginal discharge, but reported resolution of her back and chest pain. She also said that a week earlier, she’d had a fever that reached 104° F and a sore throat. She denied urinary frequency/urgency, sexual activity, or sexual abuse. The result of the urine culture performed in the ED was <10,000 col/mL (normal urogenital flora).

A genitourinary (GU) exam revealed erythematous patches with small amounts of crusting at the inner labia bilaterally. The labia were also swollen and diffusely tender to palpation. The patient had a white/gray discharge, but no vesicles or papules. The physician was unable to place a speculum due to pain.

The differential diagnosis at the time included candidal vaginitis and cellulitis. Since the patient’s skin was non-erythematous and she had vaginal discharge, she was treated for presumed severe candidal vaginitis with fluconazole and clotrimazole 1% cream. (The antibiotics were stopped because the patient reported worsening symptoms after they were prescribed in the ED.) The patient was told to return to the ED if she experienced signs and symptoms such as worsening vaginal pain or discharge, fever, or chills. A repeat urine culture was performed and the results came back normal.

Worsening symptoms. Six days later, the patient returned to the ED with urinary hesitation and persistent dysuria; she was admitted for pain control. She also complained of worsening labial swelling and increased vaginal discharge despite adherence to the fluconazole and clotrimazole cream regimen, which were discontinued on admission to the ED. She continued to deny being sexually active or abused.

A GU exam showed a 1-cm shallow ulcer on the right labium and a copious amount of foul-smelling white discharge. An Ob/Gyn resident and attending physician examined the patient; their differential diagnosis at this point included herpes simplex virus (HSV), Epstein-Barr virus (EBV), gonorrhea/chlamydia, and trauma. The patient was given topical lidocaine for pain control and started on acyclovir for presumed HSV while awaiting the HSV test results. A pelvic ultrasound and laboratory work-up were ordered at this time as well.

THE DIAGNOSIS

One review found only 13 instances of genital ulceration in females attributable to Epstein-Barr virus. The exact method by which EBV causes the ulcers is unclear.

The pelvic ultrasound showed that the uterus was a normal size and that there was no gross mass or significant pelvic fluid. The patient’s right ovary measured 2.8 × 1.6 cm; the left ovary was not seen.

The patient’s laboratory work-up included an unremarkable comprehensive metabolic panel. A complete blood count was within normal limits, except for the patient’s monocyte level, which was at 12.9% (reference range: 0%-12%). The patient had a negative urinary human chorionic gonadotropin test, and was negative for HSV, chlamydia, gonorrhea, and trichomoniasis. A rapid plasma reagin test and human immunodeficiency virus antibody (1+2) tests were nonreactive. A wet prep was negative. A mononuclear spot test (monospot), however, was positive.

Results from the monospot testing took several days to return. By the time the results arrived, the patient had been transferred to a local children’s hospital for assessment in their pediatric urology department, as she was experiencing urinary hesitation and required catheterization. The diagnosis of infectious mononucleosis presenting with genital ulcer was made. EBV cultures were never obtained, but seemed to be the likely cause of the patient’s infectious mononucleosis given her clinical symptoms and lab results.

DISCUSSION

Approximately 95% of adults worldwide are infected with EBV.¹ While the infection is often asymptomatic, some patients will develop infectious mononucleosis.¹ EBV is the most common cause of infectious mononucleosis, mainly affecting teenagers and young adults (especially college students). At least 25% of teenagers and young adults who become infected with EBV will develop infectious mononucleosis.²

Typical symptoms of infectious mononucleosis include extreme fatigue, fever, sore throat, and head and body aches.² In this case, the patient did have a fever and sore throat one week prior to presentation at our clinic, but she never complained of fatigue.

The association between mononucleosis and genital ulcers is not well known,^3,4 and the exact method by which EBV causes genital ulcers is unclear.⁵ One review found that only 13 instances of genital ulceration in females attributable to EBV infection had been reported.⁵ When ulceration does occur, the majority of cases have involved young females who presented with only mild symptoms of mononucleosis.^3,6 EBV has been found to present in the cervix, which suggests direct inoculation.^3,6

Our patient remained catheterized for 2 days while in the children’s hospital. Her ulcer started to heal and she was sent home in stable condition. No additional follow-up was required and the ulcer did not recur.

THE TAKEAWAY

Include infectious mononucleosis in the differential for patients presenting with vaginal ulcers—especially those who deny sexual activity. Including testing for EBV and mononucleosis antibodies in the work-up can aid in the diagnosis. Cases such as this one are also a good reminder of the need to question young people while their parents/guardians are not in the examroom to foster an open and honest patient-physician relationship.

THE CASE

The mother of a 13-year-old girl brought her daughter to our family medicine clinic for follow-up after being seen in the emergency department (ED) 3 days earlier. The girl had presented to the ED with a one-day history of back, chest, and vaginal pain. She was diagnosed with a urinary tract infection and treated empirically with phenazopyridine and cephalexin pending a urine culture.

During the follow-up appointment, the patient complained of worsening vaginal pain and increased vaginal discharge, but reported resolution of her back and chest pain. She also said that a week earlier, she’d had a fever that reached 104° F and a sore throat. She denied urinary frequency/urgency, sexual activity, or sexual abuse. The result of the urine culture performed in the ED was <10,000 col/mL (normal urogenital flora).

A genitourinary (GU) exam revealed erythematous patches with small amounts of crusting at the inner labia bilaterally. The labia were also swollen and diffusely tender to palpation. The patient had a white/gray discharge, but no vesicles or papules. The physician was unable to place a speculum due to pain.

The differential diagnosis at the time included candidal vaginitis and cellulitis. Since the patient’s skin was non-erythematous and she had vaginal discharge, she was treated for presumed severe candidal vaginitis with fluconazole and clotrimazole 1% cream. (The antibiotics were stopped because the patient reported worsening symptoms after they were prescribed in the ED.) The patient was told to return to the ED if she experienced signs and symptoms such as worsening vaginal pain or discharge, fever, or chills. A repeat urine culture was performed and the results came back normal.

Worsening symptoms. Six days later, the patient returned to the ED with urinary hesitation and persistent dysuria; she was admitted for pain control. She also complained of worsening labial swelling and increased vaginal discharge despite adherence to the fluconazole and clotrimazole cream regimen, which were discontinued on admission to the ED. She continued to deny being sexually active or abused.

A GU exam showed a 1-cm shallow ulcer on the right labium and a copious amount of foul-smelling white discharge. An Ob/Gyn resident and attending physician examined the patient; their differential diagnosis at this point included herpes simplex virus (HSV), Epstein-Barr virus (EBV), gonorrhea/chlamydia, and trauma. The patient was given topical lidocaine for pain control and started on acyclovir for presumed HSV while awaiting the HSV test results. A pelvic ultrasound and laboratory work-up were ordered at this time as well.

THE DIAGNOSIS

One review found only 13 instances of genital ulceration in females attributable to Epstein-Barr virus. The exact method by which EBV causes the ulcers is unclear.

The pelvic ultrasound showed that the uterus was a normal size and that there was no gross mass or significant pelvic fluid. The patient’s right ovary measured 2.8 × 1.6 cm; the left ovary was not seen.

The patient’s laboratory work-up included an unremarkable comprehensive metabolic panel. A complete blood count was within normal limits, except for the patient’s monocyte level, which was at 12.9% (reference range: 0%-12%). The patient had a negative urinary human chorionic gonadotropin test, and was negative for HSV, chlamydia, gonorrhea, and trichomoniasis. A rapid plasma reagin test and human immunodeficiency virus antibody (1+2) tests were nonreactive. A wet prep was negative. A mononuclear spot test (monospot), however, was positive.

Results from the monospot testing took several days to return. By the time the results arrived, the patient had been transferred to a local children’s hospital for assessment in their pediatric urology department, as she was experiencing urinary hesitation and required catheterization. The diagnosis of infectious mononucleosis presenting with genital ulcer was made. EBV cultures were never obtained, but seemed to be the likely cause of the patient’s infectious mononucleosis given her clinical symptoms and lab results.

DISCUSSION

Approximately 95% of adults worldwide are infected with EBV.¹ While the infection is often asymptomatic, some patients will develop infectious mononucleosis.¹ EBV is the most common cause of infectious mononucleosis, mainly affecting teenagers and young adults (especially college students). At least 25% of teenagers and young adults who become infected with EBV will develop infectious mononucleosis.²

Typical symptoms of infectious mononucleosis include extreme fatigue, fever, sore throat, and head and body aches.² In this case, the patient did have a fever and sore throat one week prior to presentation at our clinic, but she never complained of fatigue.

The association between mononucleosis and genital ulcers is not well known,^3,4 and the exact method by which EBV causes genital ulcers is unclear.⁵ One review found that only 13 instances of genital ulceration in females attributable to EBV infection had been reported.⁵ When ulceration does occur, the majority of cases have involved young females who presented with only mild symptoms of mononucleosis.^3,6 EBV has been found to present in the cervix, which suggests direct inoculation.^3,6

Our patient remained catheterized for 2 days while in the children’s hospital. Her ulcer started to heal and she was sent home in stable condition. No additional follow-up was required and the ulcer did not recur.

THE TAKEAWAY

Include infectious mononucleosis in the differential for patients presenting with vaginal ulcers—especially those who deny sexual activity. Including testing for EBV and mononucleosis antibodies in the work-up can aid in the diagnosis. Cases such as this one are also a good reminder of the need to question young people while their parents/guardians are not in the examroom to foster an open and honest patient-physician relationship.

THE CASE

The mother of a 13-year-old girl brought her daughter to our family medicine clinic for follow-up after being seen in the emergency department (ED) 3 days earlier. The girl had presented to the ED with a one-day history of back, chest, and vaginal pain. She was diagnosed with a urinary tract infection and treated empirically with phenazopyridine and cephalexin pending a urine culture.

During the follow-up appointment, the patient complained of worsening vaginal pain and increased vaginal discharge, but reported resolution of her back and chest pain. She also said that a week earlier, she’d had a fever that reached 104° F and a sore throat. She denied urinary frequency/urgency, sexual activity, or sexual abuse. The result of the urine culture performed in the ED was <10,000 col/mL (normal urogenital flora).

A genitourinary (GU) exam revealed erythematous patches with small amounts of crusting at the inner labia bilaterally. The labia were also swollen and diffusely tender to palpation. The patient had a white/gray discharge, but no vesicles or papules. The physician was unable to place a speculum due to pain.

The differential diagnosis at the time included candidal vaginitis and cellulitis. Since the patient’s skin was non-erythematous and she had vaginal discharge, she was treated for presumed severe candidal vaginitis with fluconazole and clotrimazole 1% cream. (The antibiotics were stopped because the patient reported worsening symptoms after they were prescribed in the ED.) The patient was told to return to the ED if she experienced signs and symptoms such as worsening vaginal pain or discharge, fever, or chills. A repeat urine culture was performed and the results came back normal.

Worsening symptoms. Six days later, the patient returned to the ED with urinary hesitation and persistent dysuria; she was admitted for pain control. She also complained of worsening labial swelling and increased vaginal discharge despite adherence to the fluconazole and clotrimazole cream regimen, which were discontinued on admission to the ED. She continued to deny being sexually active or abused.

A GU exam showed a 1-cm shallow ulcer on the right labium and a copious amount of foul-smelling white discharge. An Ob/Gyn resident and attending physician examined the patient; their differential diagnosis at this point included herpes simplex virus (HSV), Epstein-Barr virus (EBV), gonorrhea/chlamydia, and trauma. The patient was given topical lidocaine for pain control and started on acyclovir for presumed HSV while awaiting the HSV test results. A pelvic ultrasound and laboratory work-up were ordered at this time as well.

THE DIAGNOSIS

One review found only 13 instances of genital ulceration in females attributable to Epstein-Barr virus. The exact method by which EBV causes the ulcers is unclear.

The pelvic ultrasound showed that the uterus was a normal size and that there was no gross mass or significant pelvic fluid. The patient’s right ovary measured 2.8 × 1.6 cm; the left ovary was not seen.

The patient’s laboratory work-up included an unremarkable comprehensive metabolic panel. A complete blood count was within normal limits, except for the patient’s monocyte level, which was at 12.9% (reference range: 0%-12%). The patient had a negative urinary human chorionic gonadotropin test, and was negative for HSV, chlamydia, gonorrhea, and trichomoniasis. A rapid plasma reagin test and human immunodeficiency virus antibody (1+2) tests were nonreactive. A wet prep was negative. A mononuclear spot test (monospot), however, was positive.

Results from the monospot testing took several days to return. By the time the results arrived, the patient had been transferred to a local children’s hospital for assessment in their pediatric urology department, as she was experiencing urinary hesitation and required catheterization. The diagnosis of infectious mononucleosis presenting with genital ulcer was made. EBV cultures were never obtained, but seemed to be the likely cause of the patient’s infectious mononucleosis given her clinical symptoms and lab results.

DISCUSSION

Approximately 95% of adults worldwide are infected with EBV.¹ While the infection is often asymptomatic, some patients will develop infectious mononucleosis.¹ EBV is the most common cause of infectious mononucleosis, mainly affecting teenagers and young adults (especially college students). At least 25% of teenagers and young adults who become infected with EBV will develop infectious mononucleosis.²

Typical symptoms of infectious mononucleosis include extreme fatigue, fever, sore throat, and head and body aches.² In this case, the patient did have a fever and sore throat one week prior to presentation at our clinic, but she never complained of fatigue.

The association between mononucleosis and genital ulcers is not well known,^3,4 and the exact method by which EBV causes genital ulcers is unclear.⁵ One review found that only 13 instances of genital ulceration in females attributable to EBV infection had been reported.⁵ When ulceration does occur, the majority of cases have involved young females who presented with only mild symptoms of mononucleosis.^3,6 EBV has been found to present in the cervix, which suggests direct inoculation.^3,6

Our patient remained catheterized for 2 days while in the children’s hospital. Her ulcer started to heal and she was sent home in stable condition. No additional follow-up was required and the ulcer did not recur.

THE TAKEAWAY

Include infectious mononucleosis in the differential for patients presenting with vaginal ulcers—especially those who deny sexual activity. Including testing for EBV and mononucleosis antibodies in the work-up can aid in the diagnosis. Cases such as this one are also a good reminder of the need to question young people while their parents/guardians are not in the examroom to foster an open and honest patient-physician relationship.

A 25-year-old G2P1 mother gave birth to a boy at 40 and 6/7 weeks by vaginal delivery. Labor was induced because of oligohydramnios complicated by chorioamnionitis. The mother was treated with vancomycin and gentamicin. Prenatal lab work and delivery were otherwise unremarkable.

The delivering physician (CG) noted that the neonate had numerous brown, red, and black plaques distributed over his abdomen, lower back, groin, and thighs (FIGURE). Some plaques were hypertrichotic and other areas, apart from the plaques, were thinly desquamated. Apgar scores were 8 and 9 and the remainder of the exam, including the neurologic exam, was normal. The Dermatology Service (JK) was consulted.

WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?

Diagnosis: Giant congenital nevus

Congenital melanocytic nevi (CMN) are pigmented lesions that are present at birth and created by the abnormal migration of neural crest cells during embryogenesis.¹ Nevi are categorized by size as small (<1.5 cm), medium (1.5-20 cm), large (>20 cm), and giant (>40 cm).² Congenital nevi tend to start out flat, with uniform pigmentation, but can become more variegated in texture and color as normal growth and development continue. Giant congenital nevi are likely to thicken, darken, and enlarge as the patient grows. Some nevi may develop very coarse or dark hair.

CMN can cover any part of the body and occur independent of skin color and other ethnic factors.³ Giant congenital nevi are rare, with an incidence of approximately one in 50,000 live births and with males and females equally affected.^3,4 The condition is diagnosed at birth, based on the appearance of the lesions.

The differential diagnosis for CMN includes café au lait macules, blue-gray spots (aka Mongolian spots), nevus of Ota, nevus spilus, and vascular malformations (TABLE).⁵ CMN may present in almost any location and may be brown, black, pink, or purple in color. Café au lait macules, blue-gray spots, nevus of Ota, nevus spilus, and vascular malformations have individual location and color characteristics that set them apart clinically.

Monitor patients for melanoma, CNS complications

Patients with CMN are at increased risk of neurocutaneous melanosis (NCM) and cutaneous melanoma.

Neurocutaneous melanosis, a complication of giant congenital nevi, is a melanocyte proliferation in the central nervous system (CNS). Between 6% and 11% of patients with giant congenital nevi develop symptomatic NCM in childhood. Thus, any CNS symptoms should be fully evaluated.^4,6 NCM can result in seizures, cranial nerve palsy, hydrocephalus, and leptomeningeal melanoma.

Besides giant congenital nevi, risk factors for NCM include male sex, large numbers of satellite nevi, and the presence of nevi over the posterior midline or head and neck.⁷ The prognosis is poor for patients who develop neurologic symptoms. NCM is associated with other malignancies, including rhadomyosarcoma, liposarcoma, and malignant peripheral nerve sheath tumors.⁴

By age 10, up to 8% of patients with giant congenital nevi will develop melanoma within the nevi.

Magnetic resonance imaging (MRI) is helpful to exclude NCM. Ideally, an MRI should be ordered before 4 months of age, at which time myelination begins to make the identification of melanin deposits in the CNS more challenging.⁷ Not all patients with imaging findings that are consistent with NCM will develop symptoms.⁸

Melanoma. By age 10, up to 8% of patients with giant congenital nevi will develop melanoma within the nevi; most of these cases occur during the first 2 years of life.^7,9 Patients with NCM are at even greater risk: their rate of malignant melanoma is between 40% and 60%.⁶ As a result, patients should be monitored closely for any signs of the disease. Total body photography, serial clinical photos, and patient self-exam are helpful to detect changes and de novo lesions. New lesions or ulcerations superimposed on existing nevi may indicate malignancy.⁷ Sun protection is critical to reduce the risk of melanogenesis.

Should patients pursue surgery? It’s debatable

For children with congenital nevi and other skin conditions, the American Academy of Dermatology offers summer camps across the country.

Options for patients with large and giant CMN include early curettage (prior to 2 weeks of life), local excision (often with tissue expansion), dermabrasion, and laser therapy.² There is considerable debate about surgery. Advocates of surgery cite psychosocial relief as a major treatment benefit and speculate about prevention of melanoma. Opponents worry that excessive surgical intervention may cause melanogenesis in a scar or deep in an area of treatment. And, while smaller congenital nevi are easier to surgically remove, they have a low associated risk of developing melanoma and are typically monitored clinically.

Children with congenital nevi will need support

Several nonprofit organizations offer resources for children with congenital nevi and their families. Nevus Outreach (www.nevus.org) is an organization devoted to improving awareness and providing support for people with CMN and NCM. The group maintains a registry of patients with large nevi in an effort to help researchers improve treatment and identify a cure.

For children with congenital nevi and other skin conditions, the American Academy of Dermatology offers its “Camp Discovery” at locations across the country (https://www.aad.org/public/kids/camp-discovery). Camp Discovery provides full scholarships and includes transportation to each of the individual camps for attendees.

Our patient underwent an MRI on his fifth day of life. The results were normal and he hadn’t developed any neurologic symptoms at 4 months of age. The child sees his family physician for routine well-child visits and a dermatologist annually. The dermatologist is carefully monitoring the nevi, which continue to grow.

CORRESPONDENCE
Jonathan Karnes, MD, 6 East Chestnut Street, Suite 340, Augusta, ME 04330; [email protected].

A 25-year-old G2P1 mother gave birth to a boy at 40 and 6/7 weeks by vaginal delivery. Labor was induced because of oligohydramnios complicated by chorioamnionitis. The mother was treated with vancomycin and gentamicin. Prenatal lab work and delivery were otherwise unremarkable.

The delivering physician (CG) noted that the neonate had numerous brown, red, and black plaques distributed over his abdomen, lower back, groin, and thighs (FIGURE). Some plaques were hypertrichotic and other areas, apart from the plaques, were thinly desquamated. Apgar scores were 8 and 9 and the remainder of the exam, including the neurologic exam, was normal. The Dermatology Service (JK) was consulted.

WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?

Diagnosis: Giant congenital nevus

Congenital melanocytic nevi (CMN) are pigmented lesions that are present at birth and created by the abnormal migration of neural crest cells during embryogenesis.¹ Nevi are categorized by size as small (<1.5 cm), medium (1.5-20 cm), large (>20 cm), and giant (>40 cm).² Congenital nevi tend to start out flat, with uniform pigmentation, but can become more variegated in texture and color as normal growth and development continue. Giant congenital nevi are likely to thicken, darken, and enlarge as the patient grows. Some nevi may develop very coarse or dark hair.

CMN can cover any part of the body and occur independent of skin color and other ethnic factors.³ Giant congenital nevi are rare, with an incidence of approximately one in 50,000 live births and with males and females equally affected.^3,4 The condition is diagnosed at birth, based on the appearance of the lesions.

The differential diagnosis for CMN includes café au lait macules, blue-gray spots (aka Mongolian spots), nevus of Ota, nevus spilus, and vascular malformations (TABLE).⁵ CMN may present in almost any location and may be brown, black, pink, or purple in color. Café au lait macules, blue-gray spots, nevus of Ota, nevus spilus, and vascular malformations have individual location and color characteristics that set them apart clinically.

Monitor patients for melanoma, CNS complications

Patients with CMN are at increased risk of neurocutaneous melanosis (NCM) and cutaneous melanoma.

Neurocutaneous melanosis, a complication of giant congenital nevi, is a melanocyte proliferation in the central nervous system (CNS). Between 6% and 11% of patients with giant congenital nevi develop symptomatic NCM in childhood. Thus, any CNS symptoms should be fully evaluated.^4,6 NCM can result in seizures, cranial nerve palsy, hydrocephalus, and leptomeningeal melanoma.

Besides giant congenital nevi, risk factors for NCM include male sex, large numbers of satellite nevi, and the presence of nevi over the posterior midline or head and neck.⁷ The prognosis is poor for patients who develop neurologic symptoms. NCM is associated with other malignancies, including rhadomyosarcoma, liposarcoma, and malignant peripheral nerve sheath tumors.⁴

By age 10, up to 8% of patients with giant congenital nevi will develop melanoma within the nevi.

Magnetic resonance imaging (MRI) is helpful to exclude NCM. Ideally, an MRI should be ordered before 4 months of age, at which time myelination begins to make the identification of melanin deposits in the CNS more challenging.⁷ Not all patients with imaging findings that are consistent with NCM will develop symptoms.⁸

Melanoma. By age 10, up to 8% of patients with giant congenital nevi will develop melanoma within the nevi; most of these cases occur during the first 2 years of life.^7,9 Patients with NCM are at even greater risk: their rate of malignant melanoma is between 40% and 60%.⁶ As a result, patients should be monitored closely for any signs of the disease. Total body photography, serial clinical photos, and patient self-exam are helpful to detect changes and de novo lesions. New lesions or ulcerations superimposed on existing nevi may indicate malignancy.⁷ Sun protection is critical to reduce the risk of melanogenesis.

Should patients pursue surgery? It’s debatable

For children with congenital nevi and other skin conditions, the American Academy of Dermatology offers summer camps across the country.

Options for patients with large and giant CMN include early curettage (prior to 2 weeks of life), local excision (often with tissue expansion), dermabrasion, and laser therapy.² There is considerable debate about surgery. Advocates of surgery cite psychosocial relief as a major treatment benefit and speculate about prevention of melanoma. Opponents worry that excessive surgical intervention may cause melanogenesis in a scar or deep in an area of treatment. And, while smaller congenital nevi are easier to surgically remove, they have a low associated risk of developing melanoma and are typically monitored clinically.

Children with congenital nevi will need support

Several nonprofit organizations offer resources for children with congenital nevi and their families. Nevus Outreach (www.nevus.org) is an organization devoted to improving awareness and providing support for people with CMN and NCM. The group maintains a registry of patients with large nevi in an effort to help researchers improve treatment and identify a cure.

For children with congenital nevi and other skin conditions, the American Academy of Dermatology offers its “Camp Discovery” at locations across the country (https://www.aad.org/public/kids/camp-discovery). Camp Discovery provides full scholarships and includes transportation to each of the individual camps for attendees.

Our patient underwent an MRI on his fifth day of life. The results were normal and he hadn’t developed any neurologic symptoms at 4 months of age. The child sees his family physician for routine well-child visits and a dermatologist annually. The dermatologist is carefully monitoring the nevi, which continue to grow.

CORRESPONDENCE
Jonathan Karnes, MD, 6 East Chestnut Street, Suite 340, Augusta, ME 04330; [email protected].

A 25-year-old G2P1 mother gave birth to a boy at 40 and 6/7 weeks by vaginal delivery. Labor was induced because of oligohydramnios complicated by chorioamnionitis. The mother was treated with vancomycin and gentamicin. Prenatal lab work and delivery were otherwise unremarkable.

The delivering physician (CG) noted that the neonate had numerous brown, red, and black plaques distributed over his abdomen, lower back, groin, and thighs (FIGURE). Some plaques were hypertrichotic and other areas, apart from the plaques, were thinly desquamated. Apgar scores were 8 and 9 and the remainder of the exam, including the neurologic exam, was normal. The Dermatology Service (JK) was consulted.

WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?

Diagnosis: Giant congenital nevus

Congenital melanocytic nevi (CMN) are pigmented lesions that are present at birth and created by the abnormal migration of neural crest cells during embryogenesis.¹ Nevi are categorized by size as small (<1.5 cm), medium (1.5-20 cm), large (>20 cm), and giant (>40 cm).² Congenital nevi tend to start out flat, with uniform pigmentation, but can become more variegated in texture and color as normal growth and development continue. Giant congenital nevi are likely to thicken, darken, and enlarge as the patient grows. Some nevi may develop very coarse or dark hair.

CMN can cover any part of the body and occur independent of skin color and other ethnic factors.³ Giant congenital nevi are rare, with an incidence of approximately one in 50,000 live births and with males and females equally affected.^3,4 The condition is diagnosed at birth, based on the appearance of the lesions.

The differential diagnosis for CMN includes café au lait macules, blue-gray spots (aka Mongolian spots), nevus of Ota, nevus spilus, and vascular malformations (TABLE).⁵ CMN may present in almost any location and may be brown, black, pink, or purple in color. Café au lait macules, blue-gray spots, nevus of Ota, nevus spilus, and vascular malformations have individual location and color characteristics that set them apart clinically.

Monitor patients for melanoma, CNS complications

Patients with CMN are at increased risk of neurocutaneous melanosis (NCM) and cutaneous melanoma.

Neurocutaneous melanosis, a complication of giant congenital nevi, is a melanocyte proliferation in the central nervous system (CNS). Between 6% and 11% of patients with giant congenital nevi develop symptomatic NCM in childhood. Thus, any CNS symptoms should be fully evaluated.^4,6 NCM can result in seizures, cranial nerve palsy, hydrocephalus, and leptomeningeal melanoma.

Besides giant congenital nevi, risk factors for NCM include male sex, large numbers of satellite nevi, and the presence of nevi over the posterior midline or head and neck.⁷ The prognosis is poor for patients who develop neurologic symptoms. NCM is associated with other malignancies, including rhadomyosarcoma, liposarcoma, and malignant peripheral nerve sheath tumors.⁴

By age 10, up to 8% of patients with giant congenital nevi will develop melanoma within the nevi.

Magnetic resonance imaging (MRI) is helpful to exclude NCM. Ideally, an MRI should be ordered before 4 months of age, at which time myelination begins to make the identification of melanin deposits in the CNS more challenging.⁷ Not all patients with imaging findings that are consistent with NCM will develop symptoms.⁸

Melanoma. By age 10, up to 8% of patients with giant congenital nevi will develop melanoma within the nevi; most of these cases occur during the first 2 years of life.^7,9 Patients with NCM are at even greater risk: their rate of malignant melanoma is between 40% and 60%.⁶ As a result, patients should be monitored closely for any signs of the disease. Total body photography, serial clinical photos, and patient self-exam are helpful to detect changes and de novo lesions. New lesions or ulcerations superimposed on existing nevi may indicate malignancy.⁷ Sun protection is critical to reduce the risk of melanogenesis.

Should patients pursue surgery? It’s debatable

For children with congenital nevi and other skin conditions, the American Academy of Dermatology offers summer camps across the country.

Options for patients with large and giant CMN include early curettage (prior to 2 weeks of life), local excision (often with tissue expansion), dermabrasion, and laser therapy.² There is considerable debate about surgery. Advocates of surgery cite psychosocial relief as a major treatment benefit and speculate about prevention of melanoma. Opponents worry that excessive surgical intervention may cause melanogenesis in a scar or deep in an area of treatment. And, while smaller congenital nevi are easier to surgically remove, they have a low associated risk of developing melanoma and are typically monitored clinically.

Children with congenital nevi will need support

Several nonprofit organizations offer resources for children with congenital nevi and their families. Nevus Outreach (www.nevus.org) is an organization devoted to improving awareness and providing support for people with CMN and NCM. The group maintains a registry of patients with large nevi in an effort to help researchers improve treatment and identify a cure.

For children with congenital nevi and other skin conditions, the American Academy of Dermatology offers its “Camp Discovery” at locations across the country (https://www.aad.org/public/kids/camp-discovery). Camp Discovery provides full scholarships and includes transportation to each of the individual camps for attendees.

Our patient underwent an MRI on his fifth day of life. The results were normal and he hadn’t developed any neurologic symptoms at 4 months of age. The child sees his family physician for routine well-child visits and a dermatologist annually. The dermatologist is carefully monitoring the nevi, which continue to grow.

CORRESPONDENCE
Jonathan Karnes, MD, 6 East Chestnut Street, Suite 340, Augusta, ME 04330; [email protected].

It’s been about 2 years since I had my first e-cigarette discussion with a patient. He was a smoker in his 30s and, since we routinely screen for tobacco use in our practice, I asked him if he was interested in quitting. He said he was cutting down by using e-cigarettes, but had not yet stopped smoking.

According to the 2 articles on e-cigarettes in this issue—one original research study about the prevalence of e-cigarette use in rural Illinois and one review of the safety of e-cigarettes—my experience with this patient is typical of e-cigarette users. Many are “dual users” who turn to e-cigarettes to try to cut down on their tobacco use.

As these 2 articles discuss, we still have a great deal to learn about the potential harms and benefits of e-cigarettes. What chemicals are people taking into their bodies and how dangerous are they? And even if they pose health risks, do e-cigarettes have value as smoking cessation aids if they are less harmful than tobacco?

One could simply take a “just say No” approach, as does my wife who says, “Any chemical you inhale into your lungs can’t be good for you!” Or, one can assume the more moderate lesser-of-two-evils stance of the British health system, which posits that there may be some benefit to e-cigarettes if they help people cut down or stop using tobacco products.

Are e-cigarettes a "gateway" to the use of tobacco and other substances of abuse? Or do they represent an effective way to quit smoking?

In writing this editorial, I conducted a quick literature search that yielded only 5 legitimate randomized trials of e-cigarettes to reduce or eliminate tobacco use, and the results were underwhelming. At best, e-cigarettes appear to be as effective as other forms of nicotine replacement, such as patches, which do not have chemical additives.

Fortunately, researchers are taking e-cigarettes seriously, and research is ongoing. Using the search term “e-cigarette” yielded 2058 references, indicating a respectable amount of e-cigarette research conducted over the past 6 years. Most of the research so far has been about the chemical constituents of the vapor people inhale or about use patterns. There is still a lack of definitive research on whether e-cigarettes are an effective smoking cessation method or a “gateway” to the use of tobacco and other substances of abuse.

Or perhaps they are both.

Hopefully, in 5 years we will know a great deal more, but until we do, I am happy to see that the US Food and Drug Administration has decided to regulate e-cigarettes like tobacco.

It’s been about 2 years since I had my first e-cigarette discussion with a patient. He was a smoker in his 30s and, since we routinely screen for tobacco use in our practice, I asked him if he was interested in quitting. He said he was cutting down by using e-cigarettes, but had not yet stopped smoking.

According to the 2 articles on e-cigarettes in this issue—one original research study about the prevalence of e-cigarette use in rural Illinois and one review of the safety of e-cigarettes—my experience with this patient is typical of e-cigarette users. Many are “dual users” who turn to e-cigarettes to try to cut down on their tobacco use.

As these 2 articles discuss, we still have a great deal to learn about the potential harms and benefits of e-cigarettes. What chemicals are people taking into their bodies and how dangerous are they? And even if they pose health risks, do e-cigarettes have value as smoking cessation aids if they are less harmful than tobacco?

One could simply take a “just say No” approach, as does my wife who says, “Any chemical you inhale into your lungs can’t be good for you!” Or, one can assume the more moderate lesser-of-two-evils stance of the British health system, which posits that there may be some benefit to e-cigarettes if they help people cut down or stop using tobacco products.

Are e-cigarettes a "gateway" to the use of tobacco and other substances of abuse? Or do they represent an effective way to quit smoking?

In writing this editorial, I conducted a quick literature search that yielded only 5 legitimate randomized trials of e-cigarettes to reduce or eliminate tobacco use, and the results were underwhelming. At best, e-cigarettes appear to be as effective as other forms of nicotine replacement, such as patches, which do not have chemical additives.

Fortunately, researchers are taking e-cigarettes seriously, and research is ongoing. Using the search term “e-cigarette” yielded 2058 references, indicating a respectable amount of e-cigarette research conducted over the past 6 years. Most of the research so far has been about the chemical constituents of the vapor people inhale or about use patterns. There is still a lack of definitive research on whether e-cigarettes are an effective smoking cessation method or a “gateway” to the use of tobacco and other substances of abuse.

Or perhaps they are both.

Hopefully, in 5 years we will know a great deal more, but until we do, I am happy to see that the US Food and Drug Administration has decided to regulate e-cigarettes like tobacco.

It’s been about 2 years since I had my first e-cigarette discussion with a patient. He was a smoker in his 30s and, since we routinely screen for tobacco use in our practice, I asked him if he was interested in quitting. He said he was cutting down by using e-cigarettes, but had not yet stopped smoking.

According to the 2 articles on e-cigarettes in this issue—one original research study about the prevalence of e-cigarette use in rural Illinois and one review of the safety of e-cigarettes—my experience with this patient is typical of e-cigarette users. Many are “dual users” who turn to e-cigarettes to try to cut down on their tobacco use.

As these 2 articles discuss, we still have a great deal to learn about the potential harms and benefits of e-cigarettes. What chemicals are people taking into their bodies and how dangerous are they? And even if they pose health risks, do e-cigarettes have value as smoking cessation aids if they are less harmful than tobacco?

One could simply take a “just say No” approach, as does my wife who says, “Any chemical you inhale into your lungs can’t be good for you!” Or, one can assume the more moderate lesser-of-two-evils stance of the British health system, which posits that there may be some benefit to e-cigarettes if they help people cut down or stop using tobacco products.

Are e-cigarettes a "gateway" to the use of tobacco and other substances of abuse? Or do they represent an effective way to quit smoking?

In writing this editorial, I conducted a quick literature search that yielded only 5 legitimate randomized trials of e-cigarettes to reduce or eliminate tobacco use, and the results were underwhelming. At best, e-cigarettes appear to be as effective as other forms of nicotine replacement, such as patches, which do not have chemical additives.

Fortunately, researchers are taking e-cigarettes seriously, and research is ongoing. Using the search term “e-cigarette” yielded 2058 references, indicating a respectable amount of e-cigarette research conducted over the past 6 years. Most of the research so far has been about the chemical constituents of the vapor people inhale or about use patterns. There is still a lack of definitive research on whether e-cigarettes are an effective smoking cessation method or a “gateway” to the use of tobacco and other substances of abuse.

Or perhaps they are both.

Hopefully, in 5 years we will know a great deal more, but until we do, I am happy to see that the US Food and Drug Administration has decided to regulate e-cigarettes like tobacco.