Case Letter

To the Editor:

Psoriasis is a chronic inflammatory papulosquamous skin disease affecting 2% to 3% of the population.¹ Its pathogenesis is multifactorial, consisting of a disrupted skin barrier and dysregulated immune activation.²

A wide armamentarium of topical and systemic treatments targeting different aspects of the disease pathogenesis have been developed over the years.^3,4 Psoriasis was once considered a skin disease exclusively, but accumulating evidence suggests that it is accompanied by a multitude of systemic inflammatory comorbidities.⁵ This insight supports the concept of systemic treatment for patients with moderate to severe psoriasis. As a chronic disease, psoriasis requires continuous therapy. The treatment approach should focus on achieving efficacy and minimizing side effects. These goals can be achieved by combination, rotational, and sequential treatment approaches.⁶ Many therapeutic combinations have proven effective, using beneficially different mechanisms of action (MOAs) and toxicity profiles.⁷ We present a patient with moderate to severe recalcitrant palmoplantar psoriasis who demonstrated improvement with combination therapy.

A 50-year-old man presented with palmoplantar psoriasis of 7 years’ duration. His medical history included mild hyperlipidemia treated with atorvastatin. Prior topical treatments including calcipotriene, betamethasone dipropionate, and tacrolimus ointment did not result in improvement. Persistent acral involvement required further intervention, and the excimer laser was added to the therapeutic regimen with a minor additive therapeutic value. Acitretin (25 mg/d) was initiated; however, the disease flared up soon after. Acitretin was discontinued, and the patient was treated with apremilast (30 mg twice daily) for 9 months with a slight improvement. Physical examination revealed erythematous, fissured, scaly plaques involving both the palms and soles. Acitretin (25 mg/d) was reintroduced to the therapeutic regimen, and the acitretin-apremilast combination was used for 2 months. With this regimen, the patient experienced 90% improvement (Figures 1 and 2).

Acitretin, the active metabolite of etretinate, modulates epidermal differentiation and has immunomodulating activities.¹⁷ It commonly is used for treating palmoplantar psoriasis.⁸ Until recently, it was the only nonimmunosuppressive systemic treatment for psoriasis, and its combination with other systemic treatments, particularly biologics, has been advocated.¹⁸ Prior reports showed remarkable disease improvement when combining acitretin with alefacept, etanercept, infliximab, adalimumab, and ustekinumab.¹⁹ The optimal combination should include modalities with different MOAs without overlapping toxicities.¹⁹ Apremilast and acitretin have different MOAs and side-effect profiles, but another theoretical advantage is that they both interfere with intracellular signaling on the transcription level rather than affecting extracellular targets.¹³

Our patient with moderate to severe recalcitrant palmoplantar psoriasis demonstrated approximately 90% improvement following apremilast and acitretin combination therapy. This treatment regimen should be considered in cases of persistent acral disease resistant to other therapeutic efforts.

To the Editor:

Psoriasis is a chronic inflammatory papulosquamous skin disease affecting 2% to 3% of the population.¹ Its pathogenesis is multifactorial, consisting of a disrupted skin barrier and dysregulated immune activation.²

A wide armamentarium of topical and systemic treatments targeting different aspects of the disease pathogenesis have been developed over the years.^3,4 Psoriasis was once considered a skin disease exclusively, but accumulating evidence suggests that it is accompanied by a multitude of systemic inflammatory comorbidities.⁵ This insight supports the concept of systemic treatment for patients with moderate to severe psoriasis. As a chronic disease, psoriasis requires continuous therapy. The treatment approach should focus on achieving efficacy and minimizing side effects. These goals can be achieved by combination, rotational, and sequential treatment approaches.⁶ Many therapeutic combinations have proven effective, using beneficially different mechanisms of action (MOAs) and toxicity profiles.⁷ We present a patient with moderate to severe recalcitrant palmoplantar psoriasis who demonstrated improvement with combination therapy.

A 50-year-old man presented with palmoplantar psoriasis of 7 years’ duration. His medical history included mild hyperlipidemia treated with atorvastatin. Prior topical treatments including calcipotriene, betamethasone dipropionate, and tacrolimus ointment did not result in improvement. Persistent acral involvement required further intervention, and the excimer laser was added to the therapeutic regimen with a minor additive therapeutic value. Acitretin (25 mg/d) was initiated; however, the disease flared up soon after. Acitretin was discontinued, and the patient was treated with apremilast (30 mg twice daily) for 9 months with a slight improvement. Physical examination revealed erythematous, fissured, scaly plaques involving both the palms and soles. Acitretin (25 mg/d) was reintroduced to the therapeutic regimen, and the acitretin-apremilast combination was used for 2 months. With this regimen, the patient experienced 90% improvement (Figures 1 and 2).

Acitretin, the active metabolite of etretinate, modulates epidermal differentiation and has immunomodulating activities.¹⁷ It commonly is used for treating palmoplantar psoriasis.⁸ Until recently, it was the only nonimmunosuppressive systemic treatment for psoriasis, and its combination with other systemic treatments, particularly biologics, has been advocated.¹⁸ Prior reports showed remarkable disease improvement when combining acitretin with alefacept, etanercept, infliximab, adalimumab, and ustekinumab.¹⁹ The optimal combination should include modalities with different MOAs without overlapping toxicities.¹⁹ Apremilast and acitretin have different MOAs and side-effect profiles, but another theoretical advantage is that they both interfere with intracellular signaling on the transcription level rather than affecting extracellular targets.¹³

Our patient with moderate to severe recalcitrant palmoplantar psoriasis demonstrated approximately 90% improvement following apremilast and acitretin combination therapy. This treatment regimen should be considered in cases of persistent acral disease resistant to other therapeutic efforts.

To the Editor:

Psoriasis is a chronic inflammatory papulosquamous skin disease affecting 2% to 3% of the population.¹ Its pathogenesis is multifactorial, consisting of a disrupted skin barrier and dysregulated immune activation.²

A wide armamentarium of topical and systemic treatments targeting different aspects of the disease pathogenesis have been developed over the years.^3,4 Psoriasis was once considered a skin disease exclusively, but accumulating evidence suggests that it is accompanied by a multitude of systemic inflammatory comorbidities.⁵ This insight supports the concept of systemic treatment for patients with moderate to severe psoriasis. As a chronic disease, psoriasis requires continuous therapy. The treatment approach should focus on achieving efficacy and minimizing side effects. These goals can be achieved by combination, rotational, and sequential treatment approaches.⁶ Many therapeutic combinations have proven effective, using beneficially different mechanisms of action (MOAs) and toxicity profiles.⁷ We present a patient with moderate to severe recalcitrant palmoplantar psoriasis who demonstrated improvement with combination therapy.

A 50-year-old man presented with palmoplantar psoriasis of 7 years’ duration. His medical history included mild hyperlipidemia treated with atorvastatin. Prior topical treatments including calcipotriene, betamethasone dipropionate, and tacrolimus ointment did not result in improvement. Persistent acral involvement required further intervention, and the excimer laser was added to the therapeutic regimen with a minor additive therapeutic value. Acitretin (25 mg/d) was initiated; however, the disease flared up soon after. Acitretin was discontinued, and the patient was treated with apremilast (30 mg twice daily) for 9 months with a slight improvement. Physical examination revealed erythematous, fissured, scaly plaques involving both the palms and soles. Acitretin (25 mg/d) was reintroduced to the therapeutic regimen, and the acitretin-apremilast combination was used for 2 months. With this regimen, the patient experienced 90% improvement (Figures 1 and 2).

Acitretin, the active metabolite of etretinate, modulates epidermal differentiation and has immunomodulating activities.¹⁷ It commonly is used for treating palmoplantar psoriasis.⁸ Until recently, it was the only nonimmunosuppressive systemic treatment for psoriasis, and its combination with other systemic treatments, particularly biologics, has been advocated.¹⁸ Prior reports showed remarkable disease improvement when combining acitretin with alefacept, etanercept, infliximab, adalimumab, and ustekinumab.¹⁹ The optimal combination should include modalities with different MOAs without overlapping toxicities.¹⁹ Apremilast and acitretin have different MOAs and side-effect profiles, but another theoretical advantage is that they both interfere with intracellular signaling on the transcription level rather than affecting extracellular targets.¹³

Our patient with moderate to severe recalcitrant palmoplantar psoriasis demonstrated approximately 90% improvement following apremilast and acitretin combination therapy. This treatment regimen should be considered in cases of persistent acral disease resistant to other therapeutic efforts.

To the Editor:

A 2-week-old female infant presented to our dermatology clinic for evaluation of a 4.0×4.5-cm pink-red patch with a 1-cm central nodule and an overlying tuft of hair on the midline occipital region (Figure). The patient was born at 39 weeks’ gestation to nonconsanguineous parents via a normal spontaneous vaginal delivery and had an unremarkable prenatal course with no complications since birth. The red patch and tuft of hair were noted at birth, and the parents reported that the redness varied somewhat in size throughout the day and from day to day. An initial neurologic workup revealed no gross neurologic abnormalities. A head ultrasound revealed a soft-tissue hypervascular nodule that appeared separate from bony structures but showed evidence of a necklike extension from the nodule to the underlying soft tissues. The ultrasound could not definitively rule out intracranial extension; gross brain structures appeared normal. The initial differential diagnosis consisted of a congenital hemangioma (either a rapidly involuting or noninvoluting subtype), meningioma, or cephalocele.

Consultation with the pediatric neurosurgery service was sought, and magnetic resonance imaging of the head was performed, which demonstrated a cystic lesion within the subcutaneous soft tissue in the midline posterior scalp approximately 2 cm above the torcula. There also was a thin stalk extending from the cyst and going through an osseous defect within the occipital bone and attaching to the falx cerebri. There was no evidence of any venous communication with the cerebral sinus tracts or intraparenchymal extension. No intracranial abnormalities were noted. Given the radiographic evidence, a presumptive diagnosis of an atretic cephalocele was made with the plan for surgical repair.

The patient was re-evaluated at 3 and 4 months of age; there were no changes in the size or appearance of the lesion, and she continued to meet all developmental milestones. At 9 months of age the patient underwent uncomplicated neurosurgery to repair the cephalocele. Histopathologic examination of the resected lesion was consistent with an atretic cephalocele and showed positive staining for epithelial membrane antigen, which further confirmed a meningothelial origin; no glial elements were identified. The postoperative course was uncomplicated, and the patient was healing well at a follow-up examination 2 weeks after the procedure.

This case highlights the importance of an extensive workup when a patient presents with a midline lesion and hypertrichosis. The patient’s red patch, excluding the hair tuft, was reminiscent of a vascular malformation or hemangioma precursor lesion given the hypervascularity, the history of the lesion being present since birth, the lack of neurologic symptomatology, and the history of meeting all developmental milestones. The differential diagnosis for this patient was extensive, as many neurologic conditions present with cutaneous findings. Having central nervous system (CNS) and cutaneous comorbidities coincide underscores their common neuroectodermal origin during embryogenesis.^1,2

Atretic cephalocele is a rare diagnosis, with the prevalence of cephaloceles estimated to be 0.8 to 3.0 per 10,000 births.³ It typically occurs in either the parietal or occipital scalp as a skin nodule with a hair tuft or alopecic lesion with or without a hair collar. A cephalocele is defined as a skin-covered protrusion of intracranial contents through a bony defect. Central nervous system tissue, meninges, or cerebrospinal fluid can protrude outside the skull with this condition. An atretic cephalocele refers to a cephalocele that arrested in development and represents approximately 40% to 50% of all cephaloceles.⁴ Various hypotheses have explained the development of atretic cephaloceles: it represents a neural crest remnant, regression of a meningocele in utero, injury of multipotential mesenchymal cells, and either failure of the neural tube to close or reopening of the neural tube after closure.^4-6 There is evidence of developmental defects in skin appendages including sweat and sebaceous glands, arrector pili muscles, and hair follicles in and around the skin overlying the cephalocele, suggesting that there is a developmental abnormality of not only the CNS but also the cutaneous tissue.⁵ Typical radiographic findings include a cystic lesion with underlying defect in the skull. A vertical positioning of the straight sinus also has been demonstrated to be a consistent finding that can aid in diagnosis.⁴

Imaging is of utmost importance when a patient presents with a tuft of hair on the scalp to rule out intracranial extension and associated abnormalities such as gray matter heterotopia, hypogenesis of the corpus callosum, hydrocephalus, and Dandy-Walker and Walker-Warburg syndromes, which have all been associated with atretic cephaloceles.^4,7 The impact of location of the intracranial abnormality on prognosis has been contested, with some reporting a better prognosis with occipital cephalocele vs parietal cephalocele while others have found the opposite to be true.^6,7Surgical intervention typically is recommended to make a definitive diagnosis based on histologic evidence, to prevent ulceration or rupture of the lesion, and overall cosmesis.^4,6

Cutaneous abnormalities presenting with hypertrichosis (ie, hair tuft, hair collar) and/or capillary malformations increase the likelihood of a cranial dysraphism, especially when these findings present together and occur in and around the midline. Clinical examination cannot rule out an underlying connection to the CNS; these findings require appropriate radiographic imaging assessment prior to any procedural intervention.

To the Editor:

A 2-week-old female infant presented to our dermatology clinic for evaluation of a 4.0×4.5-cm pink-red patch with a 1-cm central nodule and an overlying tuft of hair on the midline occipital region (Figure). The patient was born at 39 weeks’ gestation to nonconsanguineous parents via a normal spontaneous vaginal delivery and had an unremarkable prenatal course with no complications since birth. The red patch and tuft of hair were noted at birth, and the parents reported that the redness varied somewhat in size throughout the day and from day to day. An initial neurologic workup revealed no gross neurologic abnormalities. A head ultrasound revealed a soft-tissue hypervascular nodule that appeared separate from bony structures but showed evidence of a necklike extension from the nodule to the underlying soft tissues. The ultrasound could not definitively rule out intracranial extension; gross brain structures appeared normal. The initial differential diagnosis consisted of a congenital hemangioma (either a rapidly involuting or noninvoluting subtype), meningioma, or cephalocele.

Consultation with the pediatric neurosurgery service was sought, and magnetic resonance imaging of the head was performed, which demonstrated a cystic lesion within the subcutaneous soft tissue in the midline posterior scalp approximately 2 cm above the torcula. There also was a thin stalk extending from the cyst and going through an osseous defect within the occipital bone and attaching to the falx cerebri. There was no evidence of any venous communication with the cerebral sinus tracts or intraparenchymal extension. No intracranial abnormalities were noted. Given the radiographic evidence, a presumptive diagnosis of an atretic cephalocele was made with the plan for surgical repair.

The patient was re-evaluated at 3 and 4 months of age; there were no changes in the size or appearance of the lesion, and she continued to meet all developmental milestones. At 9 months of age the patient underwent uncomplicated neurosurgery to repair the cephalocele. Histopathologic examination of the resected lesion was consistent with an atretic cephalocele and showed positive staining for epithelial membrane antigen, which further confirmed a meningothelial origin; no glial elements were identified. The postoperative course was uncomplicated, and the patient was healing well at a follow-up examination 2 weeks after the procedure.

This case highlights the importance of an extensive workup when a patient presents with a midline lesion and hypertrichosis. The patient’s red patch, excluding the hair tuft, was reminiscent of a vascular malformation or hemangioma precursor lesion given the hypervascularity, the history of the lesion being present since birth, the lack of neurologic symptomatology, and the history of meeting all developmental milestones. The differential diagnosis for this patient was extensive, as many neurologic conditions present with cutaneous findings. Having central nervous system (CNS) and cutaneous comorbidities coincide underscores their common neuroectodermal origin during embryogenesis.^1,2

Atretic cephalocele is a rare diagnosis, with the prevalence of cephaloceles estimated to be 0.8 to 3.0 per 10,000 births.³ It typically occurs in either the parietal or occipital scalp as a skin nodule with a hair tuft or alopecic lesion with or without a hair collar. A cephalocele is defined as a skin-covered protrusion of intracranial contents through a bony defect. Central nervous system tissue, meninges, or cerebrospinal fluid can protrude outside the skull with this condition. An atretic cephalocele refers to a cephalocele that arrested in development and represents approximately 40% to 50% of all cephaloceles.⁴ Various hypotheses have explained the development of atretic cephaloceles: it represents a neural crest remnant, regression of a meningocele in utero, injury of multipotential mesenchymal cells, and either failure of the neural tube to close or reopening of the neural tube after closure.^4-6 There is evidence of developmental defects in skin appendages including sweat and sebaceous glands, arrector pili muscles, and hair follicles in and around the skin overlying the cephalocele, suggesting that there is a developmental abnormality of not only the CNS but also the cutaneous tissue.⁵ Typical radiographic findings include a cystic lesion with underlying defect in the skull. A vertical positioning of the straight sinus also has been demonstrated to be a consistent finding that can aid in diagnosis.⁴

Imaging is of utmost importance when a patient presents with a tuft of hair on the scalp to rule out intracranial extension and associated abnormalities such as gray matter heterotopia, hypogenesis of the corpus callosum, hydrocephalus, and Dandy-Walker and Walker-Warburg syndromes, which have all been associated with atretic cephaloceles.^4,7 The impact of location of the intracranial abnormality on prognosis has been contested, with some reporting a better prognosis with occipital cephalocele vs parietal cephalocele while others have found the opposite to be true.^6,7Surgical intervention typically is recommended to make a definitive diagnosis based on histologic evidence, to prevent ulceration or rupture of the lesion, and overall cosmesis.^4,6

Cutaneous abnormalities presenting with hypertrichosis (ie, hair tuft, hair collar) and/or capillary malformations increase the likelihood of a cranial dysraphism, especially when these findings present together and occur in and around the midline. Clinical examination cannot rule out an underlying connection to the CNS; these findings require appropriate radiographic imaging assessment prior to any procedural intervention.

To the Editor:

A 2-week-old female infant presented to our dermatology clinic for evaluation of a 4.0×4.5-cm pink-red patch with a 1-cm central nodule and an overlying tuft of hair on the midline occipital region (Figure). The patient was born at 39 weeks’ gestation to nonconsanguineous parents via a normal spontaneous vaginal delivery and had an unremarkable prenatal course with no complications since birth. The red patch and tuft of hair were noted at birth, and the parents reported that the redness varied somewhat in size throughout the day and from day to day. An initial neurologic workup revealed no gross neurologic abnormalities. A head ultrasound revealed a soft-tissue hypervascular nodule that appeared separate from bony structures but showed evidence of a necklike extension from the nodule to the underlying soft tissues. The ultrasound could not definitively rule out intracranial extension; gross brain structures appeared normal. The initial differential diagnosis consisted of a congenital hemangioma (either a rapidly involuting or noninvoluting subtype), meningioma, or cephalocele.

Consultation with the pediatric neurosurgery service was sought, and magnetic resonance imaging of the head was performed, which demonstrated a cystic lesion within the subcutaneous soft tissue in the midline posterior scalp approximately 2 cm above the torcula. There also was a thin stalk extending from the cyst and going through an osseous defect within the occipital bone and attaching to the falx cerebri. There was no evidence of any venous communication with the cerebral sinus tracts or intraparenchymal extension. No intracranial abnormalities were noted. Given the radiographic evidence, a presumptive diagnosis of an atretic cephalocele was made with the plan for surgical repair.

The patient was re-evaluated at 3 and 4 months of age; there were no changes in the size or appearance of the lesion, and she continued to meet all developmental milestones. At 9 months of age the patient underwent uncomplicated neurosurgery to repair the cephalocele. Histopathologic examination of the resected lesion was consistent with an atretic cephalocele and showed positive staining for epithelial membrane antigen, which further confirmed a meningothelial origin; no glial elements were identified. The postoperative course was uncomplicated, and the patient was healing well at a follow-up examination 2 weeks after the procedure.

This case highlights the importance of an extensive workup when a patient presents with a midline lesion and hypertrichosis. The patient’s red patch, excluding the hair tuft, was reminiscent of a vascular malformation or hemangioma precursor lesion given the hypervascularity, the history of the lesion being present since birth, the lack of neurologic symptomatology, and the history of meeting all developmental milestones. The differential diagnosis for this patient was extensive, as many neurologic conditions present with cutaneous findings. Having central nervous system (CNS) and cutaneous comorbidities coincide underscores their common neuroectodermal origin during embryogenesis.^1,2

Atretic cephalocele is a rare diagnosis, with the prevalence of cephaloceles estimated to be 0.8 to 3.0 per 10,000 births.³ It typically occurs in either the parietal or occipital scalp as a skin nodule with a hair tuft or alopecic lesion with or without a hair collar. A cephalocele is defined as a skin-covered protrusion of intracranial contents through a bony defect. Central nervous system tissue, meninges, or cerebrospinal fluid can protrude outside the skull with this condition. An atretic cephalocele refers to a cephalocele that arrested in development and represents approximately 40% to 50% of all cephaloceles.⁴ Various hypotheses have explained the development of atretic cephaloceles: it represents a neural crest remnant, regression of a meningocele in utero, injury of multipotential mesenchymal cells, and either failure of the neural tube to close or reopening of the neural tube after closure.^4-6 There is evidence of developmental defects in skin appendages including sweat and sebaceous glands, arrector pili muscles, and hair follicles in and around the skin overlying the cephalocele, suggesting that there is a developmental abnormality of not only the CNS but also the cutaneous tissue.⁵ Typical radiographic findings include a cystic lesion with underlying defect in the skull. A vertical positioning of the straight sinus also has been demonstrated to be a consistent finding that can aid in diagnosis.⁴

Imaging is of utmost importance when a patient presents with a tuft of hair on the scalp to rule out intracranial extension and associated abnormalities such as gray matter heterotopia, hypogenesis of the corpus callosum, hydrocephalus, and Dandy-Walker and Walker-Warburg syndromes, which have all been associated with atretic cephaloceles.^4,7 The impact of location of the intracranial abnormality on prognosis has been contested, with some reporting a better prognosis with occipital cephalocele vs parietal cephalocele while others have found the opposite to be true.^6,7Surgical intervention typically is recommended to make a definitive diagnosis based on histologic evidence, to prevent ulceration or rupture of the lesion, and overall cosmesis.^4,6

Cutaneous abnormalities presenting with hypertrichosis (ie, hair tuft, hair collar) and/or capillary malformations increase the likelihood of a cranial dysraphism, especially when these findings present together and occur in and around the midline. Clinical examination cannot rule out an underlying connection to the CNS; these findings require appropriate radiographic imaging assessment prior to any procedural intervention.

To the Editor: 

A 49-year-old black woman presented with multiple hyperkeratotic papules that progressed over the last 2 months to circular plaques with central thick black crust resembling eschar. She first noticed these lesions as firm, small, black papules on the legs and continued to develop new lesions that eventually evolved into large, coin-shaped, hyperkeratotic plaques. Her medical history was notable for stage III non-Hodgkin follicular lymphoma in remission after treatment with rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, and prednisone 7 months earlier, and chronic hepatitis B infection being treated with entecavir. Her family history was not remarkable for psoriasis or inflammatory arthritis. 

She initially was seen by internal medicine and was started on topical triamcinolone with no improvement of the lesions. At presentation to dermatology, physical examination revealed firm, small, black, hyperkeratotic papules (Figure 1A) and circular plaques with a rim of erythema and central thick, smooth, black crust resembling eschar (Figure 1B). No other skin changes were noted at the time. The bilateral metacarpophalangeal, bilateral proximal interphalangeal, left wrist, and bilateral ankle joints were remarkable for tenderness, swelling, and reduced range of motion. She noted concomitant arthralgia and stiffness but denied fever. She had no other systemic symptoms including night sweats, weight loss, fatigue, malaise, sun sensitivity, oral ulcers, or hair loss. A radiograph of the hand was negative for erosive changes but showed mild periarticular osteopenia and fusiform soft tissue swelling of the third digit. Given the central appearance of eschar in the larger lesions, the initial differential diagnosis included Sweet syndrome, invasive fungal infection, vasculitis, and recurrent lymphoma. 

A 4-mm punch biopsy specimen of a representative lesion on the right leg revealed psoriasiform epidermal hyperplasia, parakeratosis, neutrophils in the stratum corneum and spinosum, elongation of the rete ridges, and superficial vascular ectasia, which favored a diagnosis of psoriasis (Figure 2). A periodic acid-Schiff stain was negative for fungal hyphae. Fungal culture, bacterial tissue culture, and acid-fast bacilli smear were negative. Absence of deep dermal inflammation precluded a diagnosis of Sweet syndrome. Further notable laboratory studies included negative human immunodeficiency virus (HIV) antibody, rapid plasma reagin, hepatitis C antibody, and rheumatoid factor. 

At follow-up 2 weeks later, the initial lesions were still present, and she had developed new widespread, well-demarcated, erythematous plaques with silver scale along the scalp, back, chest, and abdomen that were more typical of psoriasis. Oil spots were noted on several fingernails and toenails. Based on the clinicopathologic findings, nail changes, and asymmetric inflammatory arthritis, a diagnosis of rupioid psoriasis with psoriatic arthritis (PsA) was established. Treatment with clobetasol ointment 0.05% twice daily to active lesions was started. Initiation of systemic therapy with a steroid-sparing agent was deferred in anticipation of care coordination with rheumatology, hepatology, and hematology/oncology due to the patient's history of follicular lymphoma and chronic hepatitis B. Although attempts were made to avoid systemic corticosteroids due to the risk for a psoriasis flare upon discontinuation, because of the severity of arthralgia she was started on oral prednisone 20 mg daily by rheumatology with plans for a slow taper once an alternative systemic agent was started.¹ 

At 10-week follow-up, the patient had marked improvement of psoriatic plaques with no active lesions while only on prednisone 20 mg daily. In consultation with her care team, she subsequently was started on methotrexate 10 mg weekly for 2 weeks followed by titration to 15 mg weekly. Plans were to start a prednisone taper after a month of methotrexate to allow her new treatment time for therapeutic effect. Notably, the patient chose to discontinue prednisone 2 weeks into methotrexate therapy after only two 10-mg doses of methotrexate weekly and well before therapeutic levels were achieved. Despite stopping prednisone early and without a taper, she did not experience a relapse in psoriatic skin lesions. Three months following initiation of methotrexate, she sustained resolution of the cutaneous lesions with only residual postinflammatory hyperpigmentation. 

Psoriasis is a common chronic inflammatory skin disorder with multiple clinical presentations. There are several variants of psoriasis that are classified by their morphologic appearance including chronic plaque, guttate, erythrodermic, and pustular, with more than 90% of cases representing the plaque variant. Less common clinical presentations of psoriasis include rupioid, ostraceous, inverse, elephantine, and HIV associated.² Rupioid psoriasis is a rare variant that presents with cone-shaped, limpetlike lesions.^3,4 Similar to the limited epidemiological and clinical data pertaining to psoriasis in nonwhite racial groups, there also is a paucity of documented reports of rupioid psoriasis in skin of color. 

Rupioid comes from the Greek word rhupos, meaning dirt or filth, and is used to describe well-demarcated lesions with thick, yellow, dirty-appearing, adherent crusts resembling oyster shells with a surrounding rim of erythema.⁵ Rupioid psoriasis initially was reported in 1948 and remains an uncommon and infrequently reported variant.⁶ The majority of reported cases have been associated with arthropathy, similar to our patient.^3,4 Rupioid lesions also have been observed in an array of other diseases, such as secondary syphilis, crusted scabies, disseminated histoplasmosis, HIV, reactive arthritis, and aminoaciduria.^7-11 

Diagnosis of rupioid psoriasis can be confirmed with a skin biopsy, which demonstrates characteristic histopathologic findings of psoriasis.³ Laboratory analysis should be performed to rule out other causes of rupioid lesions, and PsA should be differentiated from rheumatoid arthritis if arthropathy is present. In our case, serum rapid plasma reagin, anti-HIV antibody, rheumatoid factor, and fungal cultures were negative. Usin0)g clinical findings, histopathology, laboratory analyses, and radiograph findings, the diagnosis of rupioid psoriasis with PsA was confirmed in our patient. 

Psoriasis was not originally suspected in our patient due to the noncharacteristic lesions with smooth black crust--similar appearing to eschar--and the patient's complicated medical history. Variations in the presentation of psoriasis among white individuals and those with skin of color have been reported in the literature.^12,13 Psoriatic lesions in darker skin tones may appear more violaceous or hyperpigmented with more conspicuous erythema and thicker plaques. Our patient lacked the classic rupioid appearance of concentric circular layers of dirty, yellow, oysterlike scale, and instead had thick, lamellate, black crust. A PubMed search of articles indexed for MEDLINE using the terms rupioid, coral reef psoriasis, rupioides, and rhupus revealed no other cases of rupioid psoriasis reported in black patients and no cases detailing the variations of rupioid lesions in skin of color. A case of rupioid psoriasis has been reported in a Hispanic patient, but the described psoriatic lesions were more characteristic of the dirty-appearing, conic plaques previously reported.¹⁴ Our case highlights a unique example of the variable presentations of cutaneous disorders in skin of color and black patients. 

Our patient's case of rupioid psoriasis with PsA presented unique challenges for systemic treatment due to her multiple comorbidities. Rupioid psoriasis most often is treated with combination topical and systemic therapy, with agents such as methotrexate and cyclosporine having prior success.^3,4 This variant of psoriasis is highly responsive to treatment, and marked improvement of lesions has been achieved with topical steroids alone with proper adherence.¹⁵ Our patient was started on clobetasol ointment 0.05% while a systemic agent was debated for her PsA. Although she did not have improvement with topical therapy alone, she experienced rapid resolution of the skin lesions after initiation of low-dose prednisone 20 mg daily. Interestingly, our patient did not experience a flare of the skin lesions upon discontinuation of systemic steroids despite the lack of an appropriate taper and methotrexate not having reached therapeutic levels. 

The clinical nuances of rupioid psoriasis in skin of color have not yet been described and remain an important diagnostic consideration. Our patient achieved remission of skin lesions with sequential treatment of topical clobetasol, a low-dose systemic steroid, and methotrexate. Based on available reports, rupioid psoriasis may represent a variant of psoriasis that is highly responsive to treatment.  

To the Editor: 

A 49-year-old black woman presented with multiple hyperkeratotic papules that progressed over the last 2 months to circular plaques with central thick black crust resembling eschar. She first noticed these lesions as firm, small, black papules on the legs and continued to develop new lesions that eventually evolved into large, coin-shaped, hyperkeratotic plaques. Her medical history was notable for stage III non-Hodgkin follicular lymphoma in remission after treatment with rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, and prednisone 7 months earlier, and chronic hepatitis B infection being treated with entecavir. Her family history was not remarkable for psoriasis or inflammatory arthritis. 

She initially was seen by internal medicine and was started on topical triamcinolone with no improvement of the lesions. At presentation to dermatology, physical examination revealed firm, small, black, hyperkeratotic papules (Figure 1A) and circular plaques with a rim of erythema and central thick, smooth, black crust resembling eschar (Figure 1B). No other skin changes were noted at the time. The bilateral metacarpophalangeal, bilateral proximal interphalangeal, left wrist, and bilateral ankle joints were remarkable for tenderness, swelling, and reduced range of motion. She noted concomitant arthralgia and stiffness but denied fever. She had no other systemic symptoms including night sweats, weight loss, fatigue, malaise, sun sensitivity, oral ulcers, or hair loss. A radiograph of the hand was negative for erosive changes but showed mild periarticular osteopenia and fusiform soft tissue swelling of the third digit. Given the central appearance of eschar in the larger lesions, the initial differential diagnosis included Sweet syndrome, invasive fungal infection, vasculitis, and recurrent lymphoma. 

A 4-mm punch biopsy specimen of a representative lesion on the right leg revealed psoriasiform epidermal hyperplasia, parakeratosis, neutrophils in the stratum corneum and spinosum, elongation of the rete ridges, and superficial vascular ectasia, which favored a diagnosis of psoriasis (Figure 2). A periodic acid-Schiff stain was negative for fungal hyphae. Fungal culture, bacterial tissue culture, and acid-fast bacilli smear were negative. Absence of deep dermal inflammation precluded a diagnosis of Sweet syndrome. Further notable laboratory studies included negative human immunodeficiency virus (HIV) antibody, rapid plasma reagin, hepatitis C antibody, and rheumatoid factor. 

At follow-up 2 weeks later, the initial lesions were still present, and she had developed new widespread, well-demarcated, erythematous plaques with silver scale along the scalp, back, chest, and abdomen that were more typical of psoriasis. Oil spots were noted on several fingernails and toenails. Based on the clinicopathologic findings, nail changes, and asymmetric inflammatory arthritis, a diagnosis of rupioid psoriasis with psoriatic arthritis (PsA) was established. Treatment with clobetasol ointment 0.05% twice daily to active lesions was started. Initiation of systemic therapy with a steroid-sparing agent was deferred in anticipation of care coordination with rheumatology, hepatology, and hematology/oncology due to the patient's history of follicular lymphoma and chronic hepatitis B. Although attempts were made to avoid systemic corticosteroids due to the risk for a psoriasis flare upon discontinuation, because of the severity of arthralgia she was started on oral prednisone 20 mg daily by rheumatology with plans for a slow taper once an alternative systemic agent was started.¹ 

At 10-week follow-up, the patient had marked improvement of psoriatic plaques with no active lesions while only on prednisone 20 mg daily. In consultation with her care team, she subsequently was started on methotrexate 10 mg weekly for 2 weeks followed by titration to 15 mg weekly. Plans were to start a prednisone taper after a month of methotrexate to allow her new treatment time for therapeutic effect. Notably, the patient chose to discontinue prednisone 2 weeks into methotrexate therapy after only two 10-mg doses of methotrexate weekly and well before therapeutic levels were achieved. Despite stopping prednisone early and without a taper, she did not experience a relapse in psoriatic skin lesions. Three months following initiation of methotrexate, she sustained resolution of the cutaneous lesions with only residual postinflammatory hyperpigmentation. 

Psoriasis is a common chronic inflammatory skin disorder with multiple clinical presentations. There are several variants of psoriasis that are classified by their morphologic appearance including chronic plaque, guttate, erythrodermic, and pustular, with more than 90% of cases representing the plaque variant. Less common clinical presentations of psoriasis include rupioid, ostraceous, inverse, elephantine, and HIV associated.² Rupioid psoriasis is a rare variant that presents with cone-shaped, limpetlike lesions.^3,4 Similar to the limited epidemiological and clinical data pertaining to psoriasis in nonwhite racial groups, there also is a paucity of documented reports of rupioid psoriasis in skin of color. 

Rupioid comes from the Greek word rhupos, meaning dirt or filth, and is used to describe well-demarcated lesions with thick, yellow, dirty-appearing, adherent crusts resembling oyster shells with a surrounding rim of erythema.⁵ Rupioid psoriasis initially was reported in 1948 and remains an uncommon and infrequently reported variant.⁶ The majority of reported cases have been associated with arthropathy, similar to our patient.^3,4 Rupioid lesions also have been observed in an array of other diseases, such as secondary syphilis, crusted scabies, disseminated histoplasmosis, HIV, reactive arthritis, and aminoaciduria.^7-11 

Diagnosis of rupioid psoriasis can be confirmed with a skin biopsy, which demonstrates characteristic histopathologic findings of psoriasis.³ Laboratory analysis should be performed to rule out other causes of rupioid lesions, and PsA should be differentiated from rheumatoid arthritis if arthropathy is present. In our case, serum rapid plasma reagin, anti-HIV antibody, rheumatoid factor, and fungal cultures were negative. Usin0)g clinical findings, histopathology, laboratory analyses, and radiograph findings, the diagnosis of rupioid psoriasis with PsA was confirmed in our patient. 

Psoriasis was not originally suspected in our patient due to the noncharacteristic lesions with smooth black crust--similar appearing to eschar--and the patient's complicated medical history. Variations in the presentation of psoriasis among white individuals and those with skin of color have been reported in the literature.^12,13 Psoriatic lesions in darker skin tones may appear more violaceous or hyperpigmented with more conspicuous erythema and thicker plaques. Our patient lacked the classic rupioid appearance of concentric circular layers of dirty, yellow, oysterlike scale, and instead had thick, lamellate, black crust. A PubMed search of articles indexed for MEDLINE using the terms rupioid, coral reef psoriasis, rupioides, and rhupus revealed no other cases of rupioid psoriasis reported in black patients and no cases detailing the variations of rupioid lesions in skin of color. A case of rupioid psoriasis has been reported in a Hispanic patient, but the described psoriatic lesions were more characteristic of the dirty-appearing, conic plaques previously reported.¹⁴ Our case highlights a unique example of the variable presentations of cutaneous disorders in skin of color and black patients. 

Our patient's case of rupioid psoriasis with PsA presented unique challenges for systemic treatment due to her multiple comorbidities. Rupioid psoriasis most often is treated with combination topical and systemic therapy, with agents such as methotrexate and cyclosporine having prior success.^3,4 This variant of psoriasis is highly responsive to treatment, and marked improvement of lesions has been achieved with topical steroids alone with proper adherence.¹⁵ Our patient was started on clobetasol ointment 0.05% while a systemic agent was debated for her PsA. Although she did not have improvement with topical therapy alone, she experienced rapid resolution of the skin lesions after initiation of low-dose prednisone 20 mg daily. Interestingly, our patient did not experience a flare of the skin lesions upon discontinuation of systemic steroids despite the lack of an appropriate taper and methotrexate not having reached therapeutic levels. 

The clinical nuances of rupioid psoriasis in skin of color have not yet been described and remain an important diagnostic consideration. Our patient achieved remission of skin lesions with sequential treatment of topical clobetasol, a low-dose systemic steroid, and methotrexate. Based on available reports, rupioid psoriasis may represent a variant of psoriasis that is highly responsive to treatment.  

To the Editor: 

A 49-year-old black woman presented with multiple hyperkeratotic papules that progressed over the last 2 months to circular plaques with central thick black crust resembling eschar. She first noticed these lesions as firm, small, black papules on the legs and continued to develop new lesions that eventually evolved into large, coin-shaped, hyperkeratotic plaques. Her medical history was notable for stage III non-Hodgkin follicular lymphoma in remission after treatment with rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, and prednisone 7 months earlier, and chronic hepatitis B infection being treated with entecavir. Her family history was not remarkable for psoriasis or inflammatory arthritis. 

She initially was seen by internal medicine and was started on topical triamcinolone with no improvement of the lesions. At presentation to dermatology, physical examination revealed firm, small, black, hyperkeratotic papules (Figure 1A) and circular plaques with a rim of erythema and central thick, smooth, black crust resembling eschar (Figure 1B). No other skin changes were noted at the time. The bilateral metacarpophalangeal, bilateral proximal interphalangeal, left wrist, and bilateral ankle joints were remarkable for tenderness, swelling, and reduced range of motion. She noted concomitant arthralgia and stiffness but denied fever. She had no other systemic symptoms including night sweats, weight loss, fatigue, malaise, sun sensitivity, oral ulcers, or hair loss. A radiograph of the hand was negative for erosive changes but showed mild periarticular osteopenia and fusiform soft tissue swelling of the third digit. Given the central appearance of eschar in the larger lesions, the initial differential diagnosis included Sweet syndrome, invasive fungal infection, vasculitis, and recurrent lymphoma. 

A 4-mm punch biopsy specimen of a representative lesion on the right leg revealed psoriasiform epidermal hyperplasia, parakeratosis, neutrophils in the stratum corneum and spinosum, elongation of the rete ridges, and superficial vascular ectasia, which favored a diagnosis of psoriasis (Figure 2). A periodic acid-Schiff stain was negative for fungal hyphae. Fungal culture, bacterial tissue culture, and acid-fast bacilli smear were negative. Absence of deep dermal inflammation precluded a diagnosis of Sweet syndrome. Further notable laboratory studies included negative human immunodeficiency virus (HIV) antibody, rapid plasma reagin, hepatitis C antibody, and rheumatoid factor. 

At follow-up 2 weeks later, the initial lesions were still present, and she had developed new widespread, well-demarcated, erythematous plaques with silver scale along the scalp, back, chest, and abdomen that were more typical of psoriasis. Oil spots were noted on several fingernails and toenails. Based on the clinicopathologic findings, nail changes, and asymmetric inflammatory arthritis, a diagnosis of rupioid psoriasis with psoriatic arthritis (PsA) was established. Treatment with clobetasol ointment 0.05% twice daily to active lesions was started. Initiation of systemic therapy with a steroid-sparing agent was deferred in anticipation of care coordination with rheumatology, hepatology, and hematology/oncology due to the patient's history of follicular lymphoma and chronic hepatitis B. Although attempts were made to avoid systemic corticosteroids due to the risk for a psoriasis flare upon discontinuation, because of the severity of arthralgia she was started on oral prednisone 20 mg daily by rheumatology with plans for a slow taper once an alternative systemic agent was started.¹ 

At 10-week follow-up, the patient had marked improvement of psoriatic plaques with no active lesions while only on prednisone 20 mg daily. In consultation with her care team, she subsequently was started on methotrexate 10 mg weekly for 2 weeks followed by titration to 15 mg weekly. Plans were to start a prednisone taper after a month of methotrexate to allow her new treatment time for therapeutic effect. Notably, the patient chose to discontinue prednisone 2 weeks into methotrexate therapy after only two 10-mg doses of methotrexate weekly and well before therapeutic levels were achieved. Despite stopping prednisone early and without a taper, she did not experience a relapse in psoriatic skin lesions. Three months following initiation of methotrexate, she sustained resolution of the cutaneous lesions with only residual postinflammatory hyperpigmentation. 

Psoriasis is a common chronic inflammatory skin disorder with multiple clinical presentations. There are several variants of psoriasis that are classified by their morphologic appearance including chronic plaque, guttate, erythrodermic, and pustular, with more than 90% of cases representing the plaque variant. Less common clinical presentations of psoriasis include rupioid, ostraceous, inverse, elephantine, and HIV associated.² Rupioid psoriasis is a rare variant that presents with cone-shaped, limpetlike lesions.^3,4 Similar to the limited epidemiological and clinical data pertaining to psoriasis in nonwhite racial groups, there also is a paucity of documented reports of rupioid psoriasis in skin of color. 

Rupioid comes from the Greek word rhupos, meaning dirt or filth, and is used to describe well-demarcated lesions with thick, yellow, dirty-appearing, adherent crusts resembling oyster shells with a surrounding rim of erythema.⁵ Rupioid psoriasis initially was reported in 1948 and remains an uncommon and infrequently reported variant.⁶ The majority of reported cases have been associated with arthropathy, similar to our patient.^3,4 Rupioid lesions also have been observed in an array of other diseases, such as secondary syphilis, crusted scabies, disseminated histoplasmosis, HIV, reactive arthritis, and aminoaciduria.^7-11 

Diagnosis of rupioid psoriasis can be confirmed with a skin biopsy, which demonstrates characteristic histopathologic findings of psoriasis.³ Laboratory analysis should be performed to rule out other causes of rupioid lesions, and PsA should be differentiated from rheumatoid arthritis if arthropathy is present. In our case, serum rapid plasma reagin, anti-HIV antibody, rheumatoid factor, and fungal cultures were negative. Usin0)g clinical findings, histopathology, laboratory analyses, and radiograph findings, the diagnosis of rupioid psoriasis with PsA was confirmed in our patient. 

Psoriasis was not originally suspected in our patient due to the noncharacteristic lesions with smooth black crust--similar appearing to eschar--and the patient's complicated medical history. Variations in the presentation of psoriasis among white individuals and those with skin of color have been reported in the literature.^12,13 Psoriatic lesions in darker skin tones may appear more violaceous or hyperpigmented with more conspicuous erythema and thicker plaques. Our patient lacked the classic rupioid appearance of concentric circular layers of dirty, yellow, oysterlike scale, and instead had thick, lamellate, black crust. A PubMed search of articles indexed for MEDLINE using the terms rupioid, coral reef psoriasis, rupioides, and rhupus revealed no other cases of rupioid psoriasis reported in black patients and no cases detailing the variations of rupioid lesions in skin of color. A case of rupioid psoriasis has been reported in a Hispanic patient, but the described psoriatic lesions were more characteristic of the dirty-appearing, conic plaques previously reported.¹⁴ Our case highlights a unique example of the variable presentations of cutaneous disorders in skin of color and black patients. 

Our patient's case of rupioid psoriasis with PsA presented unique challenges for systemic treatment due to her multiple comorbidities. Rupioid psoriasis most often is treated with combination topical and systemic therapy, with agents such as methotrexate and cyclosporine having prior success.^3,4 This variant of psoriasis is highly responsive to treatment, and marked improvement of lesions has been achieved with topical steroids alone with proper adherence.¹⁵ Our patient was started on clobetasol ointment 0.05% while a systemic agent was debated for her PsA. Although she did not have improvement with topical therapy alone, she experienced rapid resolution of the skin lesions after initiation of low-dose prednisone 20 mg daily. Interestingly, our patient did not experience a flare of the skin lesions upon discontinuation of systemic steroids despite the lack of an appropriate taper and methotrexate not having reached therapeutic levels. 

The clinical nuances of rupioid psoriasis in skin of color have not yet been described and remain an important diagnostic consideration. Our patient achieved remission of skin lesions with sequential treatment of topical clobetasol, a low-dose systemic steroid, and methotrexate. Based on available reports, rupioid psoriasis may represent a variant of psoriasis that is highly responsive to treatment.  

To the Editor:

A 46-year-old overweight woman presented with a rash in the axillae of 2 months’ duration. She did not report any additional symptoms such as pruritus or pain. She reported changing her deodorant recently from Secret Original to Secret Clinical Strength (both Procter & Gamble). Her medical history was remarkable for asthma and gastroesophageal reflux disease. Clinical examination revealed erythematous-brown, stuccolike, hyperkeratotic papules coalescing into plaques in recently shaved axillae, affecting the left axilla more than the right axilla (Figure 1). The clinical differential diagnosis included granular parakeratosis, intertrigo, Hailey-Hailey disease, Darier disease, pemphigus vegetans, confluent and reticulated papillomatosis, acanthosis nigricans, seborrheic keratoses, and irritant or allergic contact dermatitis. A punch biopsy revealed a marked compact parakeratotic horn with retention of keratohyalin granules (Figure 2). The subjacent epidermis showed some acanthosis and spongiosis with mild chronic inflammation of the dermal rim. Based on histopathology, granular parakeratosis was diagnosed.

At a subsequent visit 2 weeks later, we prescribed glycolic acid lotion 10% applied to the axillae twice daily, plus tretinoin gel 0.05% applied to the axillae each evening. She reported clearing after 1 week of therapy. She also had changed her deodorant from Secret Clinical Strength back to the usual Secret Original. The patient discontinued topical treatment after clearing of the lesions. Three weeks later, clinical examination revealed postinflammatory hyperpigmentation in the axillae, and the prior lesions had resolved (Figure 3).

The cause is unknown but possibly related to irritation from rubbing, occlusion, sweating, or deodorants.^5,7 Cases indicate a link to obesity. Hypotheses as to the etiology include the disruption of cornification. Normally, filaggrin maintains the keratohyaline granules in the stratum corneum during cornification. Therefore, the retention of keratohyaline granules in granular parakeratosis may be due to a defect in processing profilaggrin to filaggrin, which has been proposed based on ultrastructural and immunohistochemical studies.⁸

The differential diagnosis includes granular parakeratosis, intertrigo (caused by seborrheic dermatitis, candidiasis, inverse psoriasis, or erythrasma), Hailey-Hailey disease, Darier disease, pemphigus vegetans, confluent and reticulated papillomatosis, and irritant or allergic contact dermatitis. The papules may resemble seborrheic keratoses, while the plaques can be mistaken for acanthosis nigricans.

Therapeutic success has been reported with topical corticosteroids, vitamin D analogues, topical or oral retinoids, ammonium lactate, calcineurin inhibitors, topical or oral antifungals, cryotherapy, and botulinum toxin injections.^3,9-11 In addition, parakeratosis has decreased in biopsies from psoriatic patients after acitretin, methotrexate, and phototherapy, which may be alternative treatments for unusually difficult or recalcitrant cases of granular parakeratosis. To minimize side effects and resolve the papules quickly, we combined 2 synergistic agents—glycolic acid and tretinoin—each with different mechanisms of action, and we observed excellent clinical response.

Granular parakeratosis is possibly related to a combination of topical products that potentiate irritation, rubbing, and occlusion of sweat. Multiple treatment modalities likely contribute to clearing, the most important being removal of any triggering topical products. Our patient’s change in deodorant may have been the inciting factor for the disease. Withdrawal of the Secret Clinical Strength deodorant prompted clearing, though topical retinoid and glycolic acid acted as facilitating therapies for timely results. A thorough history, as highlighted by this case, may help pinpoint etiologic factors. By identifying a seemingly innocuous change in hygienic routine, we were able to minimize the need for ongoing therapy.

To the Editor:

A 46-year-old overweight woman presented with a rash in the axillae of 2 months’ duration. She did not report any additional symptoms such as pruritus or pain. She reported changing her deodorant recently from Secret Original to Secret Clinical Strength (both Procter & Gamble). Her medical history was remarkable for asthma and gastroesophageal reflux disease. Clinical examination revealed erythematous-brown, stuccolike, hyperkeratotic papules coalescing into plaques in recently shaved axillae, affecting the left axilla more than the right axilla (Figure 1). The clinical differential diagnosis included granular parakeratosis, intertrigo, Hailey-Hailey disease, Darier disease, pemphigus vegetans, confluent and reticulated papillomatosis, acanthosis nigricans, seborrheic keratoses, and irritant or allergic contact dermatitis. A punch biopsy revealed a marked compact parakeratotic horn with retention of keratohyalin granules (Figure 2). The subjacent epidermis showed some acanthosis and spongiosis with mild chronic inflammation of the dermal rim. Based on histopathology, granular parakeratosis was diagnosed.

At a subsequent visit 2 weeks later, we prescribed glycolic acid lotion 10% applied to the axillae twice daily, plus tretinoin gel 0.05% applied to the axillae each evening. She reported clearing after 1 week of therapy. She also had changed her deodorant from Secret Clinical Strength back to the usual Secret Original. The patient discontinued topical treatment after clearing of the lesions. Three weeks later, clinical examination revealed postinflammatory hyperpigmentation in the axillae, and the prior lesions had resolved (Figure 3).

The cause is unknown but possibly related to irritation from rubbing, occlusion, sweating, or deodorants.^5,7 Cases indicate a link to obesity. Hypotheses as to the etiology include the disruption of cornification. Normally, filaggrin maintains the keratohyaline granules in the stratum corneum during cornification. Therefore, the retention of keratohyaline granules in granular parakeratosis may be due to a defect in processing profilaggrin to filaggrin, which has been proposed based on ultrastructural and immunohistochemical studies.⁸

The differential diagnosis includes granular parakeratosis, intertrigo (caused by seborrheic dermatitis, candidiasis, inverse psoriasis, or erythrasma), Hailey-Hailey disease, Darier disease, pemphigus vegetans, confluent and reticulated papillomatosis, and irritant or allergic contact dermatitis. The papules may resemble seborrheic keratoses, while the plaques can be mistaken for acanthosis nigricans.

Therapeutic success has been reported with topical corticosteroids, vitamin D analogues, topical or oral retinoids, ammonium lactate, calcineurin inhibitors, topical or oral antifungals, cryotherapy, and botulinum toxin injections.^3,9-11 In addition, parakeratosis has decreased in biopsies from psoriatic patients after acitretin, methotrexate, and phototherapy, which may be alternative treatments for unusually difficult or recalcitrant cases of granular parakeratosis. To minimize side effects and resolve the papules quickly, we combined 2 synergistic agents—glycolic acid and tretinoin—each with different mechanisms of action, and we observed excellent clinical response.

Granular parakeratosis is possibly related to a combination of topical products that potentiate irritation, rubbing, and occlusion of sweat. Multiple treatment modalities likely contribute to clearing, the most important being removal of any triggering topical products. Our patient’s change in deodorant may have been the inciting factor for the disease. Withdrawal of the Secret Clinical Strength deodorant prompted clearing, though topical retinoid and glycolic acid acted as facilitating therapies for timely results. A thorough history, as highlighted by this case, may help pinpoint etiologic factors. By identifying a seemingly innocuous change in hygienic routine, we were able to minimize the need for ongoing therapy.

To the Editor:

A 46-year-old overweight woman presented with a rash in the axillae of 2 months’ duration. She did not report any additional symptoms such as pruritus or pain. She reported changing her deodorant recently from Secret Original to Secret Clinical Strength (both Procter & Gamble). Her medical history was remarkable for asthma and gastroesophageal reflux disease. Clinical examination revealed erythematous-brown, stuccolike, hyperkeratotic papules coalescing into plaques in recently shaved axillae, affecting the left axilla more than the right axilla (Figure 1). The clinical differential diagnosis included granular parakeratosis, intertrigo, Hailey-Hailey disease, Darier disease, pemphigus vegetans, confluent and reticulated papillomatosis, acanthosis nigricans, seborrheic keratoses, and irritant or allergic contact dermatitis. A punch biopsy revealed a marked compact parakeratotic horn with retention of keratohyalin granules (Figure 2). The subjacent epidermis showed some acanthosis and spongiosis with mild chronic inflammation of the dermal rim. Based on histopathology, granular parakeratosis was diagnosed.

At a subsequent visit 2 weeks later, we prescribed glycolic acid lotion 10% applied to the axillae twice daily, plus tretinoin gel 0.05% applied to the axillae each evening. She reported clearing after 1 week of therapy. She also had changed her deodorant from Secret Clinical Strength back to the usual Secret Original. The patient discontinued topical treatment after clearing of the lesions. Three weeks later, clinical examination revealed postinflammatory hyperpigmentation in the axillae, and the prior lesions had resolved (Figure 3).

The cause is unknown but possibly related to irritation from rubbing, occlusion, sweating, or deodorants.^5,7 Cases indicate a link to obesity. Hypotheses as to the etiology include the disruption of cornification. Normally, filaggrin maintains the keratohyaline granules in the stratum corneum during cornification. Therefore, the retention of keratohyaline granules in granular parakeratosis may be due to a defect in processing profilaggrin to filaggrin, which has been proposed based on ultrastructural and immunohistochemical studies.⁸

The differential diagnosis includes granular parakeratosis, intertrigo (caused by seborrheic dermatitis, candidiasis, inverse psoriasis, or erythrasma), Hailey-Hailey disease, Darier disease, pemphigus vegetans, confluent and reticulated papillomatosis, and irritant or allergic contact dermatitis. The papules may resemble seborrheic keratoses, while the plaques can be mistaken for acanthosis nigricans.

Therapeutic success has been reported with topical corticosteroids, vitamin D analogues, topical or oral retinoids, ammonium lactate, calcineurin inhibitors, topical or oral antifungals, cryotherapy, and botulinum toxin injections.^3,9-11 In addition, parakeratosis has decreased in biopsies from psoriatic patients after acitretin, methotrexate, and phototherapy, which may be alternative treatments for unusually difficult or recalcitrant cases of granular parakeratosis. To minimize side effects and resolve the papules quickly, we combined 2 synergistic agents—glycolic acid and tretinoin—each with different mechanisms of action, and we observed excellent clinical response.

Granular parakeratosis is possibly related to a combination of topical products that potentiate irritation, rubbing, and occlusion of sweat. Multiple treatment modalities likely contribute to clearing, the most important being removal of any triggering topical products. Our patient’s change in deodorant may have been the inciting factor for the disease. Withdrawal of the Secret Clinical Strength deodorant prompted clearing, though topical retinoid and glycolic acid acted as facilitating therapies for timely results. A thorough history, as highlighted by this case, may help pinpoint etiologic factors. By identifying a seemingly innocuous change in hygienic routine, we were able to minimize the need for ongoing therapy.

To the Editor:

Hailey-Hailey disease (HHD), also known as benign familial pemphigus, is an uncommon autosomal-dominant skin disease.¹ Defects in the ATPase type 2C member 1 gene, ATP2C1, result in abnormal intracellular epidermal adherence, and patients experience recurring blisters in skin folds. Longitudinal white streaks of the fingernails also may be present.¹ The illness does not appear until puberty and is heightened by the second or third decade of life. Family history often suggests the presence of disease.² Misdiagnosis of HHD occurs because of a wide spectrum of presentations. The presence of superimposed infections and carcinomas may both obscure and exacerbate this disease.²

Herpes simplex viruse types 1 and 2 (HSV-1 and HSV-2) are DNA viruses that cause common recurrent diseases. Usually, HSV-1 is associated with infection of the mouth and HSV-2 is associated with infection of the genitalia.³ Longitudinal cutaneous lesions manifest as grouped vesicles on an erythematous base. Tzanck smear of herpetic vesicles will reveal the presence of multinucleated giant cells. A direct fluorescent antibody technique also may be used to confirm the diagnosis.³

Erythrodermic HHD disease is a rare condition; moreover, there are only a few reported cases with coexistence of HHD and HSV in the literature.^3-6 We report a rare presentation of erythrodermic HHD and coexistent psoriasis with HSV superinfection.

A 69-year-old man presented to an outpatient dermatology clinic for evaluation and treatment of a rash on the scalp, face, back, and lower legs. The patient confirmed a dandruff diagnosis on the scalp and face as well as psoriasis on the trunk and extremities for the last 45 years. He described a history of successful treatment with topical agents and UV light therapy. A family history revealed that the patient’s father and 1 of 2 siblings had a similar rash and “skin problems.” The patient had a medical history of thyroid cancer treated with radiation treatment and a partial thyroidectomy 35 years prior to the current presentation as well as incompletely treated chronic hepatitis C.

A search of medical records revealed a punch biopsy from the posterior neck that demonstrated an acantholytic dyskeratosis with suprabasal acantholysis. Clinicians were unable to differentiate if it was Darier disease (DAR) or HHD. Treatment of the patient’s seborrheic dermatitis and acantholytic disorder was successful at that time with ketoconazole shampoo, ketoconazole cream, desonide cream, and triamcinolone cream. The patient remained stable for 5 years before presenting again to the dermatology clinic for worsening rash despite topical therapies.

At the current presentation, physical examination at the outpatient dermatology clinic revealed few scaly, erythematous, eroded papules distributed on the mid-back; erythematous greasy scaling on the scalp, face, and chest; and pink scaly plaques with white-silvery scale on the anterior lower legs. Histopathology of a specimen from the right mid-back demonstrated acantholysis with suprabasal clefting, hyperkeratosis, and parakeratosis with no dyskeratotic cells identified. The pathologic differential diagnosis included primary acantholytic processes including Grover disease, DAR, HHD, and pemphigus. Pathology from the right shin demonstrated acanthosis, confluent parakeratosis with associated decreased granular cell layer and collections of neutrophils within the stratum corneum, spongiosis, and superficial dermal perivascular chronic inflammation with focal exocytosis and dilated blood vessels in the papillary dermis. The clinical and pathological diagnosis on the lower legs was consistent with psoriasis. Diagnoses of seborrheic dermatitis, psoriasis on the lower legs, and HHD vs DAR on the back and chest were made. The patient was instructed to continue ketoconazole shampoo, ketoconazole cream, and desonide for seborrheic dermatitis; fluocinonide ointment 0.05% to the lower legs for psoriasis; and triamcinolone cream and a bland moisturizer to the back and chest for HHD.

Over the ensuing months, the rash worsened with erythema and scaling affecting more than half of the body surface area. Topical corticosteroids and bland emollients resulted in minimal success. Biologics and acitretin were considered for the psoriasiform dermatitis but avoided due to the patient’s medical history of thyroid cancer and chronic hepatitis C infection. Because the patient described prior success with UV light therapy for psoriasis, he requested light therapy. A subsequent trial of narrowband UVB light therapy initially improved some of the psoriasiform dermatitis on the trunk and extremities; however, after 4 weeks of treatment, the patient described pain in some of the skin and felt he was burned by minimal exposure to light therapy on one particular visit, which caused him to stop light therapy.

Approximately 2 weeks later, the patient presented to the emergency department stating his psoriasis was infected; he was diagnosed with psoriasis with secondary cellulitis and received intravenous vancomycin and piperacillin-tazobactam, with bacterial cultures demonstrating Corynebacterium and methicillin-resistant Staphylococcus aureus. Some improvement was noted in the patient’s skin after antibiotics were initiated, but he continued to describe worsening “burning and pain” throughout the psoriasis lesions. The patient’s care was transferred to the Veterans Affairs hospital where a dermatology inpatient consultation was placed.

Our initial dermatologic examination revealed generalized scaly erythroderma on the neck, trunk, and extremities, sparing the face, palms, and soles (Figure 1). Multiple crusted and intact vesicles also were present overlying the erythematous plaques on the chest, back, and proximal extremities, most grouped in clusters. The patient endorsed new symptoms of pain and burning. Tzanck smear from the abdomen along with shave biopsies from the left flank and right abdomen were performed, and intravenous acyclovir was initiated immediately after these procedures.

Hailey-Hailey disease is characterized by recurrent episodes of erythema, blisters, and plaques localized to intertriginous and perianal areas.^1,2 Patients display a spectrum of lesions that vary in severity.⁸ Typical histologic examination reveals a dilapidated brick wall appearance. Pathology of well-developed lesions will show suprabasal acantholysis with minimal dyskeratosis.²

The generalized form of HHD is an extremely rare variant of the disease.¹⁰ Generalized HHD may resemble acute hypersensitivity reaction, erythema multiforme, and toxic epidermal necrolysis.¹ Chronic diseases, such as psoriasis (as in this patient), also may contribute to a clinically confusing picture.⁸ Hailey-Hailey disease and psoriasis are thought to occasionally koebnerize (isomorphic response) to areas of trauma.¹⁶ Our patient experienced widespread erythematous papules and plaques not restricted to skin folds. His skin lesions continued to worsen over several months progressing to erythroderma. The presence of suprabasal acantholysis in a dilapidated brick wall pattern, along with the patient’s history, prior pathology reports, clinical picture, and negative direct immunofluorescence and indirect immunofluorescence studies helped to confirm the diagnosis of erythrodermic HHD.

Hailey-Hailey disease is caused by heterozygous mutations in the ATP2C1 gene on chromosome 3q21-24 coding for a Golgi ATPase called SPCA1 (secretory pathway calcium/manganese-ATPase).⁹ Subsequent disturbances in cytosolic-Golgi calcium concentrations interfere with epidermal keratinocyte adherence resulting in acantholytic disease. Studies of interfamilial and intrafamilial mutations fail to pinpoint a common mutation pattern among patients with generalized phenotypes,⁹ which further supports theories that intrinsic or extrinsic factors such as friction, heat, radiation, contact allergens, and infection affect the severity of HHD disease and not the type of mutation.^3,9

Generalization of HHD is likely caused by nonspecific triggers in an already genetically disturbed epidermis.¹⁰ Interrupted epithelial function exposes skin to infections that exacerbate the underlying disease. Superimposing bacterial infections are commonly reported in HHD. Staphylococcus, Streptococcus, and Candida species colonize the skin and aggravate the disease.¹¹ Much less commonly, HSV superinfection can complicate HHD.^3-7 No data are currently available about the frequency or incidence of Herpesviridae in HHD.⁷ Some studies suggest that UVB light therapy can be an exacerbating factor in DAR and some but not all HHD patients,^12,13 while other case reports^14,15 document clinically improved responses using phototherapy for patients with HHD. Clinicians should remain suspicious and evaluate for HSV infection in refractory or sudden exacerbation of HHD.⁷ Furthermore, coexistent psoriasis and HHD also is a rare entity but has been described,⁸ which illustrates the importance of not attributing all skin manifestations to a previously diagnosed disorder but instead keeping an open mind in case new dermatologic conditions present themselves at a later time.

We present a rare case of erythrodermic HHD and coexistent psoriasis with HSV superinfection. We hope to draw awareness to this association of generalized HHD with both HSV and psoriasis to help clinicians make the correct diagnosis promptly in similar cases in the future.

To the Editor:

Hailey-Hailey disease (HHD), also known as benign familial pemphigus, is an uncommon autosomal-dominant skin disease.¹ Defects in the ATPase type 2C member 1 gene, ATP2C1, result in abnormal intracellular epidermal adherence, and patients experience recurring blisters in skin folds. Longitudinal white streaks of the fingernails also may be present.¹ The illness does not appear until puberty and is heightened by the second or third decade of life. Family history often suggests the presence of disease.² Misdiagnosis of HHD occurs because of a wide spectrum of presentations. The presence of superimposed infections and carcinomas may both obscure and exacerbate this disease.²

Herpes simplex viruse types 1 and 2 (HSV-1 and HSV-2) are DNA viruses that cause common recurrent diseases. Usually, HSV-1 is associated with infection of the mouth and HSV-2 is associated with infection of the genitalia.³ Longitudinal cutaneous lesions manifest as grouped vesicles on an erythematous base. Tzanck smear of herpetic vesicles will reveal the presence of multinucleated giant cells. A direct fluorescent antibody technique also may be used to confirm the diagnosis.³

Erythrodermic HHD disease is a rare condition; moreover, there are only a few reported cases with coexistence of HHD and HSV in the literature.^3-6 We report a rare presentation of erythrodermic HHD and coexistent psoriasis with HSV superinfection.

A 69-year-old man presented to an outpatient dermatology clinic for evaluation and treatment of a rash on the scalp, face, back, and lower legs. The patient confirmed a dandruff diagnosis on the scalp and face as well as psoriasis on the trunk and extremities for the last 45 years. He described a history of successful treatment with topical agents and UV light therapy. A family history revealed that the patient’s father and 1 of 2 siblings had a similar rash and “skin problems.” The patient had a medical history of thyroid cancer treated with radiation treatment and a partial thyroidectomy 35 years prior to the current presentation as well as incompletely treated chronic hepatitis C.

A search of medical records revealed a punch biopsy from the posterior neck that demonstrated an acantholytic dyskeratosis with suprabasal acantholysis. Clinicians were unable to differentiate if it was Darier disease (DAR) or HHD. Treatment of the patient’s seborrheic dermatitis and acantholytic disorder was successful at that time with ketoconazole shampoo, ketoconazole cream, desonide cream, and triamcinolone cream. The patient remained stable for 5 years before presenting again to the dermatology clinic for worsening rash despite topical therapies.

At the current presentation, physical examination at the outpatient dermatology clinic revealed few scaly, erythematous, eroded papules distributed on the mid-back; erythematous greasy scaling on the scalp, face, and chest; and pink scaly plaques with white-silvery scale on the anterior lower legs. Histopathology of a specimen from the right mid-back demonstrated acantholysis with suprabasal clefting, hyperkeratosis, and parakeratosis with no dyskeratotic cells identified. The pathologic differential diagnosis included primary acantholytic processes including Grover disease, DAR, HHD, and pemphigus. Pathology from the right shin demonstrated acanthosis, confluent parakeratosis with associated decreased granular cell layer and collections of neutrophils within the stratum corneum, spongiosis, and superficial dermal perivascular chronic inflammation with focal exocytosis and dilated blood vessels in the papillary dermis. The clinical and pathological diagnosis on the lower legs was consistent with psoriasis. Diagnoses of seborrheic dermatitis, psoriasis on the lower legs, and HHD vs DAR on the back and chest were made. The patient was instructed to continue ketoconazole shampoo, ketoconazole cream, and desonide for seborrheic dermatitis; fluocinonide ointment 0.05% to the lower legs for psoriasis; and triamcinolone cream and a bland moisturizer to the back and chest for HHD.

Over the ensuing months, the rash worsened with erythema and scaling affecting more than half of the body surface area. Topical corticosteroids and bland emollients resulted in minimal success. Biologics and acitretin were considered for the psoriasiform dermatitis but avoided due to the patient’s medical history of thyroid cancer and chronic hepatitis C infection. Because the patient described prior success with UV light therapy for psoriasis, he requested light therapy. A subsequent trial of narrowband UVB light therapy initially improved some of the psoriasiform dermatitis on the trunk and extremities; however, after 4 weeks of treatment, the patient described pain in some of the skin and felt he was burned by minimal exposure to light therapy on one particular visit, which caused him to stop light therapy.

Approximately 2 weeks later, the patient presented to the emergency department stating his psoriasis was infected; he was diagnosed with psoriasis with secondary cellulitis and received intravenous vancomycin and piperacillin-tazobactam, with bacterial cultures demonstrating Corynebacterium and methicillin-resistant Staphylococcus aureus. Some improvement was noted in the patient’s skin after antibiotics were initiated, but he continued to describe worsening “burning and pain” throughout the psoriasis lesions. The patient’s care was transferred to the Veterans Affairs hospital where a dermatology inpatient consultation was placed.

Our initial dermatologic examination revealed generalized scaly erythroderma on the neck, trunk, and extremities, sparing the face, palms, and soles (Figure 1). Multiple crusted and intact vesicles also were present overlying the erythematous plaques on the chest, back, and proximal extremities, most grouped in clusters. The patient endorsed new symptoms of pain and burning. Tzanck smear from the abdomen along with shave biopsies from the left flank and right abdomen were performed, and intravenous acyclovir was initiated immediately after these procedures.

Hailey-Hailey disease is characterized by recurrent episodes of erythema, blisters, and plaques localized to intertriginous and perianal areas.^1,2 Patients display a spectrum of lesions that vary in severity.⁸ Typical histologic examination reveals a dilapidated brick wall appearance. Pathology of well-developed lesions will show suprabasal acantholysis with minimal dyskeratosis.²

The generalized form of HHD is an extremely rare variant of the disease.¹⁰ Generalized HHD may resemble acute hypersensitivity reaction, erythema multiforme, and toxic epidermal necrolysis.¹ Chronic diseases, such as psoriasis (as in this patient), also may contribute to a clinically confusing picture.⁸ Hailey-Hailey disease and psoriasis are thought to occasionally koebnerize (isomorphic response) to areas of trauma.¹⁶ Our patient experienced widespread erythematous papules and plaques not restricted to skin folds. His skin lesions continued to worsen over several months progressing to erythroderma. The presence of suprabasal acantholysis in a dilapidated brick wall pattern, along with the patient’s history, prior pathology reports, clinical picture, and negative direct immunofluorescence and indirect immunofluorescence studies helped to confirm the diagnosis of erythrodermic HHD.

Hailey-Hailey disease is caused by heterozygous mutations in the ATP2C1 gene on chromosome 3q21-24 coding for a Golgi ATPase called SPCA1 (secretory pathway calcium/manganese-ATPase).⁹ Subsequent disturbances in cytosolic-Golgi calcium concentrations interfere with epidermal keratinocyte adherence resulting in acantholytic disease. Studies of interfamilial and intrafamilial mutations fail to pinpoint a common mutation pattern among patients with generalized phenotypes,⁹ which further supports theories that intrinsic or extrinsic factors such as friction, heat, radiation, contact allergens, and infection affect the severity of HHD disease and not the type of mutation.^3,9

Generalization of HHD is likely caused by nonspecific triggers in an already genetically disturbed epidermis.¹⁰ Interrupted epithelial function exposes skin to infections that exacerbate the underlying disease. Superimposing bacterial infections are commonly reported in HHD. Staphylococcus, Streptococcus, and Candida species colonize the skin and aggravate the disease.¹¹ Much less commonly, HSV superinfection can complicate HHD.^3-7 No data are currently available about the frequency or incidence of Herpesviridae in HHD.⁷ Some studies suggest that UVB light therapy can be an exacerbating factor in DAR and some but not all HHD patients,^12,13 while other case reports^14,15 document clinically improved responses using phototherapy for patients with HHD. Clinicians should remain suspicious and evaluate for HSV infection in refractory or sudden exacerbation of HHD.⁷ Furthermore, coexistent psoriasis and HHD also is a rare entity but has been described,⁸ which illustrates the importance of not attributing all skin manifestations to a previously diagnosed disorder but instead keeping an open mind in case new dermatologic conditions present themselves at a later time.

We present a rare case of erythrodermic HHD and coexistent psoriasis with HSV superinfection. We hope to draw awareness to this association of generalized HHD with both HSV and psoriasis to help clinicians make the correct diagnosis promptly in similar cases in the future.

To the Editor:

Hailey-Hailey disease (HHD), also known as benign familial pemphigus, is an uncommon autosomal-dominant skin disease.¹ Defects in the ATPase type 2C member 1 gene, ATP2C1, result in abnormal intracellular epidermal adherence, and patients experience recurring blisters in skin folds. Longitudinal white streaks of the fingernails also may be present.¹ The illness does not appear until puberty and is heightened by the second or third decade of life. Family history often suggests the presence of disease.² Misdiagnosis of HHD occurs because of a wide spectrum of presentations. The presence of superimposed infections and carcinomas may both obscure and exacerbate this disease.²

Herpes simplex viruse types 1 and 2 (HSV-1 and HSV-2) are DNA viruses that cause common recurrent diseases. Usually, HSV-1 is associated with infection of the mouth and HSV-2 is associated with infection of the genitalia.³ Longitudinal cutaneous lesions manifest as grouped vesicles on an erythematous base. Tzanck smear of herpetic vesicles will reveal the presence of multinucleated giant cells. A direct fluorescent antibody technique also may be used to confirm the diagnosis.³

Erythrodermic HHD disease is a rare condition; moreover, there are only a few reported cases with coexistence of HHD and HSV in the literature.^3-6 We report a rare presentation of erythrodermic HHD and coexistent psoriasis with HSV superinfection.

A 69-year-old man presented to an outpatient dermatology clinic for evaluation and treatment of a rash on the scalp, face, back, and lower legs. The patient confirmed a dandruff diagnosis on the scalp and face as well as psoriasis on the trunk and extremities for the last 45 years. He described a history of successful treatment with topical agents and UV light therapy. A family history revealed that the patient’s father and 1 of 2 siblings had a similar rash and “skin problems.” The patient had a medical history of thyroid cancer treated with radiation treatment and a partial thyroidectomy 35 years prior to the current presentation as well as incompletely treated chronic hepatitis C.

A search of medical records revealed a punch biopsy from the posterior neck that demonstrated an acantholytic dyskeratosis with suprabasal acantholysis. Clinicians were unable to differentiate if it was Darier disease (DAR) or HHD. Treatment of the patient’s seborrheic dermatitis and acantholytic disorder was successful at that time with ketoconazole shampoo, ketoconazole cream, desonide cream, and triamcinolone cream. The patient remained stable for 5 years before presenting again to the dermatology clinic for worsening rash despite topical therapies.

At the current presentation, physical examination at the outpatient dermatology clinic revealed few scaly, erythematous, eroded papules distributed on the mid-back; erythematous greasy scaling on the scalp, face, and chest; and pink scaly plaques with white-silvery scale on the anterior lower legs. Histopathology of a specimen from the right mid-back demonstrated acantholysis with suprabasal clefting, hyperkeratosis, and parakeratosis with no dyskeratotic cells identified. The pathologic differential diagnosis included primary acantholytic processes including Grover disease, DAR, HHD, and pemphigus. Pathology from the right shin demonstrated acanthosis, confluent parakeratosis with associated decreased granular cell layer and collections of neutrophils within the stratum corneum, spongiosis, and superficial dermal perivascular chronic inflammation with focal exocytosis and dilated blood vessels in the papillary dermis. The clinical and pathological diagnosis on the lower legs was consistent with psoriasis. Diagnoses of seborrheic dermatitis, psoriasis on the lower legs, and HHD vs DAR on the back and chest were made. The patient was instructed to continue ketoconazole shampoo, ketoconazole cream, and desonide for seborrheic dermatitis; fluocinonide ointment 0.05% to the lower legs for psoriasis; and triamcinolone cream and a bland moisturizer to the back and chest for HHD.

Over the ensuing months, the rash worsened with erythema and scaling affecting more than half of the body surface area. Topical corticosteroids and bland emollients resulted in minimal success. Biologics and acitretin were considered for the psoriasiform dermatitis but avoided due to the patient’s medical history of thyroid cancer and chronic hepatitis C infection. Because the patient described prior success with UV light therapy for psoriasis, he requested light therapy. A subsequent trial of narrowband UVB light therapy initially improved some of the psoriasiform dermatitis on the trunk and extremities; however, after 4 weeks of treatment, the patient described pain in some of the skin and felt he was burned by minimal exposure to light therapy on one particular visit, which caused him to stop light therapy.

Approximately 2 weeks later, the patient presented to the emergency department stating his psoriasis was infected; he was diagnosed with psoriasis with secondary cellulitis and received intravenous vancomycin and piperacillin-tazobactam, with bacterial cultures demonstrating Corynebacterium and methicillin-resistant Staphylococcus aureus. Some improvement was noted in the patient’s skin after antibiotics were initiated, but he continued to describe worsening “burning and pain” throughout the psoriasis lesions. The patient’s care was transferred to the Veterans Affairs hospital where a dermatology inpatient consultation was placed.

Our initial dermatologic examination revealed generalized scaly erythroderma on the neck, trunk, and extremities, sparing the face, palms, and soles (Figure 1). Multiple crusted and intact vesicles also were present overlying the erythematous plaques on the chest, back, and proximal extremities, most grouped in clusters. The patient endorsed new symptoms of pain and burning. Tzanck smear from the abdomen along with shave biopsies from the left flank and right abdomen were performed, and intravenous acyclovir was initiated immediately after these procedures.

Hailey-Hailey disease is characterized by recurrent episodes of erythema, blisters, and plaques localized to intertriginous and perianal areas.^1,2 Patients display a spectrum of lesions that vary in severity.⁸ Typical histologic examination reveals a dilapidated brick wall appearance. Pathology of well-developed lesions will show suprabasal acantholysis with minimal dyskeratosis.²

The generalized form of HHD is an extremely rare variant of the disease.¹⁰ Generalized HHD may resemble acute hypersensitivity reaction, erythema multiforme, and toxic epidermal necrolysis.¹ Chronic diseases, such as psoriasis (as in this patient), also may contribute to a clinically confusing picture.⁸ Hailey-Hailey disease and psoriasis are thought to occasionally koebnerize (isomorphic response) to areas of trauma.¹⁶ Our patient experienced widespread erythematous papules and plaques not restricted to skin folds. His skin lesions continued to worsen over several months progressing to erythroderma. The presence of suprabasal acantholysis in a dilapidated brick wall pattern, along with the patient’s history, prior pathology reports, clinical picture, and negative direct immunofluorescence and indirect immunofluorescence studies helped to confirm the diagnosis of erythrodermic HHD.

Hailey-Hailey disease is caused by heterozygous mutations in the ATP2C1 gene on chromosome 3q21-24 coding for a Golgi ATPase called SPCA1 (secretory pathway calcium/manganese-ATPase).⁹ Subsequent disturbances in cytosolic-Golgi calcium concentrations interfere with epidermal keratinocyte adherence resulting in acantholytic disease. Studies of interfamilial and intrafamilial mutations fail to pinpoint a common mutation pattern among patients with generalized phenotypes,⁹ which further supports theories that intrinsic or extrinsic factors such as friction, heat, radiation, contact allergens, and infection affect the severity of HHD disease and not the type of mutation.^3,9

Generalization of HHD is likely caused by nonspecific triggers in an already genetically disturbed epidermis.¹⁰ Interrupted epithelial function exposes skin to infections that exacerbate the underlying disease. Superimposing bacterial infections are commonly reported in HHD. Staphylococcus, Streptococcus, and Candida species colonize the skin and aggravate the disease.¹¹ Much less commonly, HSV superinfection can complicate HHD.^3-7 No data are currently available about the frequency or incidence of Herpesviridae in HHD.⁷ Some studies suggest that UVB light therapy can be an exacerbating factor in DAR and some but not all HHD patients,^12,13 while other case reports^14,15 document clinically improved responses using phototherapy for patients with HHD. Clinicians should remain suspicious and evaluate for HSV infection in refractory or sudden exacerbation of HHD.⁷ Furthermore, coexistent psoriasis and HHD also is a rare entity but has been described,⁸ which illustrates the importance of not attributing all skin manifestations to a previously diagnosed disorder but instead keeping an open mind in case new dermatologic conditions present themselves at a later time.

We present a rare case of erythrodermic HHD and coexistent psoriasis with HSV superinfection. We hope to draw awareness to this association of generalized HHD with both HSV and psoriasis to help clinicians make the correct diagnosis promptly in similar cases in the future.

To the Editor: 

A 61-year-old man presented to the emergency department with a rash on the right leg, generalized pruritus, and chest pain. The patient described intermittent exertional pressure-like chest pain over the last few days but had no known prior cardiac history. He also noted worsening edema of the right leg with erythema. Three months prior he had been hospitalized for a similar presentation and was diagnosed with cellulitis of the right leg. The patient was treated with a course of trimethoprim-sulfamethoxazole and permethrin cream for presumed scabies and followed up with dermatology for the persistent generalized pruritic rash and cellulitis. At that time, he was diagnosed with stasis dermatitis with dermatitis neglecta and excoriations. He was educated on general hygiene and treated with triamcinolone, hydrophilic ointment, and pramoxine lotion for pruritus. He also was empirically treated again for scabies.  

At the current presentation, preliminary investigation showed profound anemia with a hemoglobin level of 6.2 g/dL (baseline hemoglobin level 3 months prior, 13.1 g/dL). He was subsequently admitted to the general medicine ward for further investigation of severe symptomatic anemia. A medical history revealed moderate chronic obstructive pulmonary disease, hypertension, gastroesophageal reflux disease, xerosis, and fracture of the right ankle following open reduction internal fixation 6 years prior to admission. There was no history of blood loss, antiplatelet agents, or anticoagulants. He was on disability and lived in a single-room occupancy hotel. He did not report any high-risk sexual behaviors or abuse of alcohol or drugs. He actively smoked 1.5 packs of cigarettes per day for the last 30 years. He denied any allergies. 

Physical examination revealed the patient was afebrile, nontoxic, disheveled, and in no acute distress. He had anicteric sclera and pale conjunctiva. The right leg appeared more erythematous and edematous compared to the left leg but without warmth or tenderness to palpation. He had innumerable 4- to 5-mm, erythematous, excoriated papules on the skin (Figure). His bed sheets were noted to have multiple rusty-black specks thought to be related to the crusted lesions. Physical examination was otherwise unremarkable.  

Laboratory workup revealed severe iron-deficiency anemia without any evidence of hemolysis, marrow suppression, infection, or immune compromise (Table). He had a vitamin B12 deficiency (197 pg/mL [reference range, 239-931 pg/mL]), but we felt it was very unlikely to be responsible for his profound, sudden-onset microcytic anemia. Further evaluation for occult bleeding revealed an unremarkable upper endoscopy with push enteroscopy and colonoscopy. An alternate etiology of the anemia could not be identified.     

Subsequently, he reported multiple pruritic bug bites sustained at the hotel room where he resided and continued to note pruritus while hospitalized. Pest control inspected the hospital room and identified bedbugs, Cimex lectularius, among his belongings. Upon further review, his clothes and walker were found to be completely infested with these organisms in different stages of development. Treatment included blood transfusions, iron supplementation, and environmental control of the infested living space both in the hospital and at his residence, with subsequent resolution of symptoms and anemia. Two weeks following discharge, the patient no longer reported pruritus, and his hemoglobin level had returned to baseline.  

Over the last decade there has been an exponential resurgence in C lectularius infestations in developed countries attributed to increasing global travel, growing pesticide resistance, lack of public awareness, and inadequate pest control programs. This re-emergence has resulted in a public health problem. Although bedbugs are not known to transmit infectious diseases, severe infestation can result in notable dermatitis, iron-deficiency anemia from chronic blood loss, superinfection, allergic reactions including anaphylaxis in rare cases, and psychologic distress. 

Iron-deficiency anemia caused by excessive bedbug biting in infants and children has been documented as early as the 1960s.¹ Our knowledge of severe anemia due to bedbug infestation is limited to only 4 cases in the literature, according to a PubMed search of articles indexed for MEDLINE using the terms bedbugs anemia and cimex anemia.^1-4 All cases reported bedbug infestations involving personal clothing, belongings, and/or living spaces. Patient concerns at presentation ranged from lethargy and fatigue with pruritic rash to chest pain and syncope with findings of severe microcytic or normocytic anemia (hemoglobin level, 5-8 g/dL). All cases were treated supportively with blood transfusion and iron supplementation, with hemoglobin recovery after several weeks. Environmental extermination also was required to prevent recurrence.^1-4 Given that each bedbug blood meal is on average 7 mm3, one would have to incur a minimum of 143,000 bites to experience a blood loss of 1 L.³  

The differential diagnosis for a patient with generalized pruritus should be broad and includes dermatologic conditions (eg, xerosis, atopic dermatitis, contact dermatitis, urticaria, dermatophytosis, lichen simplex chronicus, psoriasis, scabies, pediculosis corporis and pubis, other arthropod bites, bullous pemphigoid), systemic disorders (eg, renal disease, diabetes mellitus, thyroid disease, cholestasis, human immunodeficiency virus), malignancy, connective tissue disease, medication side effects, and psychogenic and neuropathic itch.     

The diagnosis of C lectularius infestation is confirmed by finding the wingless, reddish brown, flat and ovular arthropod, with adult lengths of 4 to 7 mm, approximately the size of an apple seed.^5-11 Bedbugs typically are active at night and feed for 3 to 10 minutes. After their feed or during the day, bedbugs will return to their nest in furniture, mattresses, beds, walls, and floors. Bedbug bites appear as small clusters or lines of pruritic erythematous papules with a central hemorrhagic puncta. Other cutaneous symptoms include isolated pruritus, papules, nodules, and bullous eruptions.⁷ Additional signs of bedbug infestation include black fecal stains in areas of inhabitation as well as actual bedbugs feeding during the day due to overcrowding.  

Treatment of pruritic localized cutaneous reactions is supportive and includes antipruritic agents, topical steroids, topical anesthetics, antihistamines, or topical or systemic antibiotics for secondary infections.^5-11 Systemic reactions, including anaphylaxis, are treated with epinephrine, antihistamines, and/or corticosteroids, while severe anemia is treated supportively with blood transfusions and iron supplementation.^5-11 To prevent reoccurrence, environmental control in the form of nonchemical and chemical treatments is crucial in controlling bedbug infestations.^5-11  

This case highlights the relevance of a rare but notable morbidity associated with bedbug infestation and the adverse effects of bedbugs on public health. This patient's living situation in a single-room occupancy hotel, poor hygiene, and possible cognitive impairment from his multiple medical conditions may have increased his risk for extreme bedbug infestation. With a good history, physical examination, proper inspection of the patient's belongings, and provider awareness of this epidemic, the severity of this patient's anemia may have been circumvented on the prior hospital admission and follow-up office visit. Once such an infestation is confirmed, a multidisciplinary approach including social work assistance, health services, and pest control is needed to appropriately treat the patient and the environment. Methods in preventing and managing this growing public health problem include improving hygiene, avoiding secondhand goods, and increasing awareness in the identification and proper elimination of bedbugs.^5-7  

To the Editor: 

A 61-year-old man presented to the emergency department with a rash on the right leg, generalized pruritus, and chest pain. The patient described intermittent exertional pressure-like chest pain over the last few days but had no known prior cardiac history. He also noted worsening edema of the right leg with erythema. Three months prior he had been hospitalized for a similar presentation and was diagnosed with cellulitis of the right leg. The patient was treated with a course of trimethoprim-sulfamethoxazole and permethrin cream for presumed scabies and followed up with dermatology for the persistent generalized pruritic rash and cellulitis. At that time, he was diagnosed with stasis dermatitis with dermatitis neglecta and excoriations. He was educated on general hygiene and treated with triamcinolone, hydrophilic ointment, and pramoxine lotion for pruritus. He also was empirically treated again for scabies.  

At the current presentation, preliminary investigation showed profound anemia with a hemoglobin level of 6.2 g/dL (baseline hemoglobin level 3 months prior, 13.1 g/dL). He was subsequently admitted to the general medicine ward for further investigation of severe symptomatic anemia. A medical history revealed moderate chronic obstructive pulmonary disease, hypertension, gastroesophageal reflux disease, xerosis, and fracture of the right ankle following open reduction internal fixation 6 years prior to admission. There was no history of blood loss, antiplatelet agents, or anticoagulants. He was on disability and lived in a single-room occupancy hotel. He did not report any high-risk sexual behaviors or abuse of alcohol or drugs. He actively smoked 1.5 packs of cigarettes per day for the last 30 years. He denied any allergies. 

Physical examination revealed the patient was afebrile, nontoxic, disheveled, and in no acute distress. He had anicteric sclera and pale conjunctiva. The right leg appeared more erythematous and edematous compared to the left leg but without warmth or tenderness to palpation. He had innumerable 4- to 5-mm, erythematous, excoriated papules on the skin (Figure). His bed sheets were noted to have multiple rusty-black specks thought to be related to the crusted lesions. Physical examination was otherwise unremarkable.  

Laboratory workup revealed severe iron-deficiency anemia without any evidence of hemolysis, marrow suppression, infection, or immune compromise (Table). He had a vitamin B12 deficiency (197 pg/mL [reference range, 239-931 pg/mL]), but we felt it was very unlikely to be responsible for his profound, sudden-onset microcytic anemia. Further evaluation for occult bleeding revealed an unremarkable upper endoscopy with push enteroscopy and colonoscopy. An alternate etiology of the anemia could not be identified.     

Subsequently, he reported multiple pruritic bug bites sustained at the hotel room where he resided and continued to note pruritus while hospitalized. Pest control inspected the hospital room and identified bedbugs, Cimex lectularius, among his belongings. Upon further review, his clothes and walker were found to be completely infested with these organisms in different stages of development. Treatment included blood transfusions, iron supplementation, and environmental control of the infested living space both in the hospital and at his residence, with subsequent resolution of symptoms and anemia. Two weeks following discharge, the patient no longer reported pruritus, and his hemoglobin level had returned to baseline.  

Over the last decade there has been an exponential resurgence in C lectularius infestations in developed countries attributed to increasing global travel, growing pesticide resistance, lack of public awareness, and inadequate pest control programs. This re-emergence has resulted in a public health problem. Although bedbugs are not known to transmit infectious diseases, severe infestation can result in notable dermatitis, iron-deficiency anemia from chronic blood loss, superinfection, allergic reactions including anaphylaxis in rare cases, and psychologic distress. 

Iron-deficiency anemia caused by excessive bedbug biting in infants and children has been documented as early as the 1960s.¹ Our knowledge of severe anemia due to bedbug infestation is limited to only 4 cases in the literature, according to a PubMed search of articles indexed for MEDLINE using the terms bedbugs anemia and cimex anemia.^1-4 All cases reported bedbug infestations involving personal clothing, belongings, and/or living spaces. Patient concerns at presentation ranged from lethargy and fatigue with pruritic rash to chest pain and syncope with findings of severe microcytic or normocytic anemia (hemoglobin level, 5-8 g/dL). All cases were treated supportively with blood transfusion and iron supplementation, with hemoglobin recovery after several weeks. Environmental extermination also was required to prevent recurrence.^1-4 Given that each bedbug blood meal is on average 7 mm3, one would have to incur a minimum of 143,000 bites to experience a blood loss of 1 L.³  

The differential diagnosis for a patient with generalized pruritus should be broad and includes dermatologic conditions (eg, xerosis, atopic dermatitis, contact dermatitis, urticaria, dermatophytosis, lichen simplex chronicus, psoriasis, scabies, pediculosis corporis and pubis, other arthropod bites, bullous pemphigoid), systemic disorders (eg, renal disease, diabetes mellitus, thyroid disease, cholestasis, human immunodeficiency virus), malignancy, connective tissue disease, medication side effects, and psychogenic and neuropathic itch.     

The diagnosis of C lectularius infestation is confirmed by finding the wingless, reddish brown, flat and ovular arthropod, with adult lengths of 4 to 7 mm, approximately the size of an apple seed.^5-11 Bedbugs typically are active at night and feed for 3 to 10 minutes. After their feed or during the day, bedbugs will return to their nest in furniture, mattresses, beds, walls, and floors. Bedbug bites appear as small clusters or lines of pruritic erythematous papules with a central hemorrhagic puncta. Other cutaneous symptoms include isolated pruritus, papules, nodules, and bullous eruptions.⁷ Additional signs of bedbug infestation include black fecal stains in areas of inhabitation as well as actual bedbugs feeding during the day due to overcrowding.  

Treatment of pruritic localized cutaneous reactions is supportive and includes antipruritic agents, topical steroids, topical anesthetics, antihistamines, or topical or systemic antibiotics for secondary infections.^5-11 Systemic reactions, including anaphylaxis, are treated with epinephrine, antihistamines, and/or corticosteroids, while severe anemia is treated supportively with blood transfusions and iron supplementation.^5-11 To prevent reoccurrence, environmental control in the form of nonchemical and chemical treatments is crucial in controlling bedbug infestations.^5-11  

This case highlights the relevance of a rare but notable morbidity associated with bedbug infestation and the adverse effects of bedbugs on public health. This patient's living situation in a single-room occupancy hotel, poor hygiene, and possible cognitive impairment from his multiple medical conditions may have increased his risk for extreme bedbug infestation. With a good history, physical examination, proper inspection of the patient's belongings, and provider awareness of this epidemic, the severity of this patient's anemia may have been circumvented on the prior hospital admission and follow-up office visit. Once such an infestation is confirmed, a multidisciplinary approach including social work assistance, health services, and pest control is needed to appropriately treat the patient and the environment. Methods in preventing and managing this growing public health problem include improving hygiene, avoiding secondhand goods, and increasing awareness in the identification and proper elimination of bedbugs.^5-7  

To the Editor: 

A 61-year-old man presented to the emergency department with a rash on the right leg, generalized pruritus, and chest pain. The patient described intermittent exertional pressure-like chest pain over the last few days but had no known prior cardiac history. He also noted worsening edema of the right leg with erythema. Three months prior he had been hospitalized for a similar presentation and was diagnosed with cellulitis of the right leg. The patient was treated with a course of trimethoprim-sulfamethoxazole and permethrin cream for presumed scabies and followed up with dermatology for the persistent generalized pruritic rash and cellulitis. At that time, he was diagnosed with stasis dermatitis with dermatitis neglecta and excoriations. He was educated on general hygiene and treated with triamcinolone, hydrophilic ointment, and pramoxine lotion for pruritus. He also was empirically treated again for scabies.  

At the current presentation, preliminary investigation showed profound anemia with a hemoglobin level of 6.2 g/dL (baseline hemoglobin level 3 months prior, 13.1 g/dL). He was subsequently admitted to the general medicine ward for further investigation of severe symptomatic anemia. A medical history revealed moderate chronic obstructive pulmonary disease, hypertension, gastroesophageal reflux disease, xerosis, and fracture of the right ankle following open reduction internal fixation 6 years prior to admission. There was no history of blood loss, antiplatelet agents, or anticoagulants. He was on disability and lived in a single-room occupancy hotel. He did not report any high-risk sexual behaviors or abuse of alcohol or drugs. He actively smoked 1.5 packs of cigarettes per day for the last 30 years. He denied any allergies. 

Physical examination revealed the patient was afebrile, nontoxic, disheveled, and in no acute distress. He had anicteric sclera and pale conjunctiva. The right leg appeared more erythematous and edematous compared to the left leg but without warmth or tenderness to palpation. He had innumerable 4- to 5-mm, erythematous, excoriated papules on the skin (Figure). His bed sheets were noted to have multiple rusty-black specks thought to be related to the crusted lesions. Physical examination was otherwise unremarkable.  

Laboratory workup revealed severe iron-deficiency anemia without any evidence of hemolysis, marrow suppression, infection, or immune compromise (Table). He had a vitamin B12 deficiency (197 pg/mL [reference range, 239-931 pg/mL]), but we felt it was very unlikely to be responsible for his profound, sudden-onset microcytic anemia. Further evaluation for occult bleeding revealed an unremarkable upper endoscopy with push enteroscopy and colonoscopy. An alternate etiology of the anemia could not be identified.     

Subsequently, he reported multiple pruritic bug bites sustained at the hotel room where he resided and continued to note pruritus while hospitalized. Pest control inspected the hospital room and identified bedbugs, Cimex lectularius, among his belongings. Upon further review, his clothes and walker were found to be completely infested with these organisms in different stages of development. Treatment included blood transfusions, iron supplementation, and environmental control of the infested living space both in the hospital and at his residence, with subsequent resolution of symptoms and anemia. Two weeks following discharge, the patient no longer reported pruritus, and his hemoglobin level had returned to baseline.  

Over the last decade there has been an exponential resurgence in C lectularius infestations in developed countries attributed to increasing global travel, growing pesticide resistance, lack of public awareness, and inadequate pest control programs. This re-emergence has resulted in a public health problem. Although bedbugs are not known to transmit infectious diseases, severe infestation can result in notable dermatitis, iron-deficiency anemia from chronic blood loss, superinfection, allergic reactions including anaphylaxis in rare cases, and psychologic distress. 

Iron-deficiency anemia caused by excessive bedbug biting in infants and children has been documented as early as the 1960s.¹ Our knowledge of severe anemia due to bedbug infestation is limited to only 4 cases in the literature, according to a PubMed search of articles indexed for MEDLINE using the terms bedbugs anemia and cimex anemia.^1-4 All cases reported bedbug infestations involving personal clothing, belongings, and/or living spaces. Patient concerns at presentation ranged from lethargy and fatigue with pruritic rash to chest pain and syncope with findings of severe microcytic or normocytic anemia (hemoglobin level, 5-8 g/dL). All cases were treated supportively with blood transfusion and iron supplementation, with hemoglobin recovery after several weeks. Environmental extermination also was required to prevent recurrence.^1-4 Given that each bedbug blood meal is on average 7 mm3, one would have to incur a minimum of 143,000 bites to experience a blood loss of 1 L.³  

The differential diagnosis for a patient with generalized pruritus should be broad and includes dermatologic conditions (eg, xerosis, atopic dermatitis, contact dermatitis, urticaria, dermatophytosis, lichen simplex chronicus, psoriasis, scabies, pediculosis corporis and pubis, other arthropod bites, bullous pemphigoid), systemic disorders (eg, renal disease, diabetes mellitus, thyroid disease, cholestasis, human immunodeficiency virus), malignancy, connective tissue disease, medication side effects, and psychogenic and neuropathic itch.     

The diagnosis of C lectularius infestation is confirmed by finding the wingless, reddish brown, flat and ovular arthropod, with adult lengths of 4 to 7 mm, approximately the size of an apple seed.^5-11 Bedbugs typically are active at night and feed for 3 to 10 minutes. After their feed or during the day, bedbugs will return to their nest in furniture, mattresses, beds, walls, and floors. Bedbug bites appear as small clusters or lines of pruritic erythematous papules with a central hemorrhagic puncta. Other cutaneous symptoms include isolated pruritus, papules, nodules, and bullous eruptions.⁷ Additional signs of bedbug infestation include black fecal stains in areas of inhabitation as well as actual bedbugs feeding during the day due to overcrowding.  

Treatment of pruritic localized cutaneous reactions is supportive and includes antipruritic agents, topical steroids, topical anesthetics, antihistamines, or topical or systemic antibiotics for secondary infections.^5-11 Systemic reactions, including anaphylaxis, are treated with epinephrine, antihistamines, and/or corticosteroids, while severe anemia is treated supportively with blood transfusions and iron supplementation.^5-11 To prevent reoccurrence, environmental control in the form of nonchemical and chemical treatments is crucial in controlling bedbug infestations.^5-11  

This case highlights the relevance of a rare but notable morbidity associated with bedbug infestation and the adverse effects of bedbugs on public health. This patient's living situation in a single-room occupancy hotel, poor hygiene, and possible cognitive impairment from his multiple medical conditions may have increased his risk for extreme bedbug infestation. With a good history, physical examination, proper inspection of the patient's belongings, and provider awareness of this epidemic, the severity of this patient's anemia may have been circumvented on the prior hospital admission and follow-up office visit. Once such an infestation is confirmed, a multidisciplinary approach including social work assistance, health services, and pest control is needed to appropriately treat the patient and the environment. Methods in preventing and managing this growing public health problem include improving hygiene, avoiding secondhand goods, and increasing awareness in the identification and proper elimination of bedbugs.^5-7  

To the Editor:

Raynaud phenomenon is characterized by vasospasm of arterioles causing intermittent ischemia of the digits. The characteristic triphasic color change presents first as a dramatic change in skin color from normal to white, as the vasoconstriction causes pallor secondary to ischemia. This change is followed by a blue appearance, as cyanosis results from the deoxygenated venous blood. Finally, reflex vasodilation and reperfusion manifest as a red color from erythema. Several cases have been reported describing Raynaud phenomenon affecting the nipples of breastfeeding women.^1-5 This vasospasm results in episodic nipple pain manifesting from breastfeeding and exposure to cold. If it is not appropriately treated, the pain’s severity causes affected women to stop breastfeeding. We report a case of vasospasm of the nipple in which the patient experienced nipple pain and a separate lancinating pain that radiated through the breasts.

A 36-year-old woman presented with excruciating nipple and breast pain 3 weeks after delivering her first child. She had no history of smoking or Raynaud phenomenon. The nipple pain was triggered upon breastfeeding and exposure to cold. During these episodes, the nipples would initially blanch white, then turn purple and finally a deep red. The patient also experienced an episodic excruciating lancinating pain of the breast that would randomly and spontaneously radiate through either breast several times per day for 15 to 30 seconds. A workup including an antinuclear antibody test, complete blood cell count with differential, and comprehensive metabolic panel all were within reference range.

The patient was diagnosed with nipple vasospasm. Partial relief of nipple pain occurred after treatment with 30 mg daily of nifedipine; 60 mg daily resulted in complete control, allowing the patient to breastfeed without discomfort, but the lancinating pain continued unabated. The patient could not discontinue breastfeeding because her child was intolerant to formula. She became despondent, as she could find no relief from the pain that she found to be intolerable. Because the patient’s description was reminiscent of the lancinating pain seen in postherpetic neuralgia, a trial of pregabalin was prescribed. A dosage of 75 mg twice daily resulted in near-complete resolution of the pain. After 3 months, the patient successfully weaned her child from breast milk to formula, and the nipple and breast pain promptly resolved. The baby experienced no adverse effects from the patient’s use of pregabalin.

This condition was first described by Gunther¹ in 1970 as initial blanching of the nipple followed by a mulberry color. It was termed psychosomatic sore nipples.¹ Lawlor-Smith and Lawlor-Smith² described the condition in 1997 and termed it vasospasm of the nipple. They reported 5 patients who experienced debilitating nipple pain as well as the triphasic color change of Raynaud phenomenon or a biphasic color change (white and blue). Two patients had a history of Raynaud phenomenon affecting the digits before their first pregnancy.² Anderson et al³ presented 12 breastfeeding women with Raynaud phenomenon of the nipple; only 1 patient had a history of Raynaud phenomenon. In this series, all 6 women who chose to try nifedipine responded well to the drug.³ 

Raynaud phenomenon of the nipple also has been reported to be associated with the use of labetalol.⁴ In this case, the patient had a history of Raynaud phenomenon affecting the toes and nipples on cold days. In 2 subsequent pregnancies she was treated with labetalol for pregnancy-induced hypertension, which resulted in severe nipple pain with each pregnancy unrelated to cold weather. Unlike other cases, this patient experienced antenatal symptoms in addition to the typical postnatal symptoms. The nipple pain resolved with discontinuation of the labetalol.⁴

Barrett et al⁵ conducted a retrospective review of medical records of 88 breastfeeding mothers who presented with nipple pain and dermatitis. They defined the criteria for Raynaud phenomenon of the nipple as chronic deep breast pain (in general lasting >4 weeks) that responded to therapy for the condition and had at least 2 of the following characteristics: (1) observed or self-reported color changes of the nipple, especially with cold exposure (white, blue, or red); (2) cold sensitivity or color changes of the hands or feet with cold exposure; or (3) failed therapy with oral antifungals. Using these criteria, they diagnosed 22 women (25%) with Raynaud phenomenon of the nipple; 20 (91%) reported a history of cold sensitivity or color change of acral surfaces. Of 12 patients who received and tolerated nifedipine use, 10 (83%) reported decreased pain or complete resolution. This series described breast or nipple pain, whereas other reported cases only described nipple pain. The authors described a sharp, shooting, or stabbing pain—qualifications not previously noted.⁵ Our patient experienced both nipple pain and a lancinating breast pain consistent with the cases reported by Barrett et al.⁵

The nipple pain and treatment response in our patient was typical of previously reported cases of vasospasm of the nipple in breastfeeding women; however, Barrett et al⁵ did not describe individual patients who exhibited the dual nature of the pain described in our patient. The nipple pain experienced during breastfeeding in our patient was successfully treated with nifedipine. We report the successful treatment of the separate lancinating pain with pregabalin.

To the Editor:

Raynaud phenomenon is characterized by vasospasm of arterioles causing intermittent ischemia of the digits. The characteristic triphasic color change presents first as a dramatic change in skin color from normal to white, as the vasoconstriction causes pallor secondary to ischemia. This change is followed by a blue appearance, as cyanosis results from the deoxygenated venous blood. Finally, reflex vasodilation and reperfusion manifest as a red color from erythema. Several cases have been reported describing Raynaud phenomenon affecting the nipples of breastfeeding women.^1-5 This vasospasm results in episodic nipple pain manifesting from breastfeeding and exposure to cold. If it is not appropriately treated, the pain’s severity causes affected women to stop breastfeeding. We report a case of vasospasm of the nipple in which the patient experienced nipple pain and a separate lancinating pain that radiated through the breasts.

A 36-year-old woman presented with excruciating nipple and breast pain 3 weeks after delivering her first child. She had no history of smoking or Raynaud phenomenon. The nipple pain was triggered upon breastfeeding and exposure to cold. During these episodes, the nipples would initially blanch white, then turn purple and finally a deep red. The patient also experienced an episodic excruciating lancinating pain of the breast that would randomly and spontaneously radiate through either breast several times per day for 15 to 30 seconds. A workup including an antinuclear antibody test, complete blood cell count with differential, and comprehensive metabolic panel all were within reference range.

The patient was diagnosed with nipple vasospasm. Partial relief of nipple pain occurred after treatment with 30 mg daily of nifedipine; 60 mg daily resulted in complete control, allowing the patient to breastfeed without discomfort, but the lancinating pain continued unabated. The patient could not discontinue breastfeeding because her child was intolerant to formula. She became despondent, as she could find no relief from the pain that she found to be intolerable. Because the patient’s description was reminiscent of the lancinating pain seen in postherpetic neuralgia, a trial of pregabalin was prescribed. A dosage of 75 mg twice daily resulted in near-complete resolution of the pain. After 3 months, the patient successfully weaned her child from breast milk to formula, and the nipple and breast pain promptly resolved. The baby experienced no adverse effects from the patient’s use of pregabalin.

This condition was first described by Gunther¹ in 1970 as initial blanching of the nipple followed by a mulberry color. It was termed psychosomatic sore nipples.¹ Lawlor-Smith and Lawlor-Smith² described the condition in 1997 and termed it vasospasm of the nipple. They reported 5 patients who experienced debilitating nipple pain as well as the triphasic color change of Raynaud phenomenon or a biphasic color change (white and blue). Two patients had a history of Raynaud phenomenon affecting the digits before their first pregnancy.² Anderson et al³ presented 12 breastfeeding women with Raynaud phenomenon of the nipple; only 1 patient had a history of Raynaud phenomenon. In this series, all 6 women who chose to try nifedipine responded well to the drug.³ 

Raynaud phenomenon of the nipple also has been reported to be associated with the use of labetalol.⁴ In this case, the patient had a history of Raynaud phenomenon affecting the toes and nipples on cold days. In 2 subsequent pregnancies she was treated with labetalol for pregnancy-induced hypertension, which resulted in severe nipple pain with each pregnancy unrelated to cold weather. Unlike other cases, this patient experienced antenatal symptoms in addition to the typical postnatal symptoms. The nipple pain resolved with discontinuation of the labetalol.⁴

Barrett et al⁵ conducted a retrospective review of medical records of 88 breastfeeding mothers who presented with nipple pain and dermatitis. They defined the criteria for Raynaud phenomenon of the nipple as chronic deep breast pain (in general lasting >4 weeks) that responded to therapy for the condition and had at least 2 of the following characteristics: (1) observed or self-reported color changes of the nipple, especially with cold exposure (white, blue, or red); (2) cold sensitivity or color changes of the hands or feet with cold exposure; or (3) failed therapy with oral antifungals. Using these criteria, they diagnosed 22 women (25%) with Raynaud phenomenon of the nipple; 20 (91%) reported a history of cold sensitivity or color change of acral surfaces. Of 12 patients who received and tolerated nifedipine use, 10 (83%) reported decreased pain or complete resolution. This series described breast or nipple pain, whereas other reported cases only described nipple pain. The authors described a sharp, shooting, or stabbing pain—qualifications not previously noted.⁵ Our patient experienced both nipple pain and a lancinating breast pain consistent with the cases reported by Barrett et al.⁵

The nipple pain and treatment response in our patient was typical of previously reported cases of vasospasm of the nipple in breastfeeding women; however, Barrett et al⁵ did not describe individual patients who exhibited the dual nature of the pain described in our patient. The nipple pain experienced during breastfeeding in our patient was successfully treated with nifedipine. We report the successful treatment of the separate lancinating pain with pregabalin.

To the Editor:

Raynaud phenomenon is characterized by vasospasm of arterioles causing intermittent ischemia of the digits. The characteristic triphasic color change presents first as a dramatic change in skin color from normal to white, as the vasoconstriction causes pallor secondary to ischemia. This change is followed by a blue appearance, as cyanosis results from the deoxygenated venous blood. Finally, reflex vasodilation and reperfusion manifest as a red color from erythema. Several cases have been reported describing Raynaud phenomenon affecting the nipples of breastfeeding women.^1-5 This vasospasm results in episodic nipple pain manifesting from breastfeeding and exposure to cold. If it is not appropriately treated, the pain’s severity causes affected women to stop breastfeeding. We report a case of vasospasm of the nipple in which the patient experienced nipple pain and a separate lancinating pain that radiated through the breasts.

A 36-year-old woman presented with excruciating nipple and breast pain 3 weeks after delivering her first child. She had no history of smoking or Raynaud phenomenon. The nipple pain was triggered upon breastfeeding and exposure to cold. During these episodes, the nipples would initially blanch white, then turn purple and finally a deep red. The patient also experienced an episodic excruciating lancinating pain of the breast that would randomly and spontaneously radiate through either breast several times per day for 15 to 30 seconds. A workup including an antinuclear antibody test, complete blood cell count with differential, and comprehensive metabolic panel all were within reference range.

The patient was diagnosed with nipple vasospasm. Partial relief of nipple pain occurred after treatment with 30 mg daily of nifedipine; 60 mg daily resulted in complete control, allowing the patient to breastfeed without discomfort, but the lancinating pain continued unabated. The patient could not discontinue breastfeeding because her child was intolerant to formula. She became despondent, as she could find no relief from the pain that she found to be intolerable. Because the patient’s description was reminiscent of the lancinating pain seen in postherpetic neuralgia, a trial of pregabalin was prescribed. A dosage of 75 mg twice daily resulted in near-complete resolution of the pain. After 3 months, the patient successfully weaned her child from breast milk to formula, and the nipple and breast pain promptly resolved. The baby experienced no adverse effects from the patient’s use of pregabalin.

This condition was first described by Gunther¹ in 1970 as initial blanching of the nipple followed by a mulberry color. It was termed psychosomatic sore nipples.¹ Lawlor-Smith and Lawlor-Smith² described the condition in 1997 and termed it vasospasm of the nipple. They reported 5 patients who experienced debilitating nipple pain as well as the triphasic color change of Raynaud phenomenon or a biphasic color change (white and blue). Two patients had a history of Raynaud phenomenon affecting the digits before their first pregnancy.² Anderson et al³ presented 12 breastfeeding women with Raynaud phenomenon of the nipple; only 1 patient had a history of Raynaud phenomenon. In this series, all 6 women who chose to try nifedipine responded well to the drug.³ 

Raynaud phenomenon of the nipple also has been reported to be associated with the use of labetalol.⁴ In this case, the patient had a history of Raynaud phenomenon affecting the toes and nipples on cold days. In 2 subsequent pregnancies she was treated with labetalol for pregnancy-induced hypertension, which resulted in severe nipple pain with each pregnancy unrelated to cold weather. Unlike other cases, this patient experienced antenatal symptoms in addition to the typical postnatal symptoms. The nipple pain resolved with discontinuation of the labetalol.⁴

Barrett et al⁵ conducted a retrospective review of medical records of 88 breastfeeding mothers who presented with nipple pain and dermatitis. They defined the criteria for Raynaud phenomenon of the nipple as chronic deep breast pain (in general lasting >4 weeks) that responded to therapy for the condition and had at least 2 of the following characteristics: (1) observed or self-reported color changes of the nipple, especially with cold exposure (white, blue, or red); (2) cold sensitivity or color changes of the hands or feet with cold exposure; or (3) failed therapy with oral antifungals. Using these criteria, they diagnosed 22 women (25%) with Raynaud phenomenon of the nipple; 20 (91%) reported a history of cold sensitivity or color change of acral surfaces. Of 12 patients who received and tolerated nifedipine use, 10 (83%) reported decreased pain or complete resolution. This series described breast or nipple pain, whereas other reported cases only described nipple pain. The authors described a sharp, shooting, or stabbing pain—qualifications not previously noted.⁵ Our patient experienced both nipple pain and a lancinating breast pain consistent with the cases reported by Barrett et al.⁵

The nipple pain and treatment response in our patient was typical of previously reported cases of vasospasm of the nipple in breastfeeding women; however, Barrett et al⁵ did not describe individual patients who exhibited the dual nature of the pain described in our patient. The nipple pain experienced during breastfeeding in our patient was successfully treated with nifedipine. We report the successful treatment of the separate lancinating pain with pregabalin.

To the Editor:

A 69-year-old woman presented to the allergy clinic for evaluation of a rash on the left breast. The patient had a history of breast cancer that was treated with a lumpectomy followed by external beam radiation therapy (total dose, 6000 cGy) to the lateral aspect of the left breast approximately 4 years prior. She developed acute breast dermatitis from the radiation, which was self-treated with over-the-counter hydrocortisone cream. The patient subsequently developed a blistering skin eruption over the area where she applied the cream. She did not recall the subtype of hydrocortisone she used (butyrate and acetate are available over-the-counter). She discontinued the hydrocortisone and was started on triamcinolone cream 0.1%, which was well tolerated, and the rash resolved.

The patient had a history of a similar reaction to hydrocortisone butyrate after blepharoplasty approximately 10 years prior to the current presentation, characterized by facial erythema, pruritus, and blistering. A patch test confirmed reactivity to hydrocortisone-17-butyrate and tixocortol pivalate. However, a skin-prick test for hydrocortisone acetate cream 1% was negative.

Subsequently, the patient developed acute-onset dyspepsia, gnawing epigastric pain, regurgitation, and bloating. A diagnosis of eosinophilic gastritis was established via biopsy, which found increased eosinophils in the lamina propria (>50 eosinophils per high-power field). Helicobacter pylori was not identified. She was started on the proton-pump inhibitor dexlansoprazole but symptoms did not improve. Her other medications included benazepril, alprazolam as needed, vitamin D, and magnesium. The patient subsequently was started on a trial of oral prednisone 40 mg/d. Three days after initiation, she developed an erythematous macular rash over the left breast.

The next day she presented to the allergy clinic. Physical examination of the left breast revealed a 20×10-cm, nipple-sparing patch of well-demarcated erythema without fluctuance or overlying lesions. The area of erythema overlapped with the prior radiation field based on radiation marker tattoos and the lumpectomy scar (Figure). There was no evidence to suggest inflammation of deeper tissue or the pectoral muscles. Vital signs were normal, and the remainder of the examination was unremarkable, including breast, lymph node, and complete skin examinations.

In contrast, triamcinolone is a class B steroid, which has a C16,17-cis-diol or -ketal. Other than budesonide, which can cross-react with D2 steroids, class B steroids do not cross-react with hydrocortisone or prednisone. Triamcinolone does not usually cross-react with D2 corticosteroids, which likely explains why our patient was later able to tolerate triamcinolone to treat eosinophilic gastrointestinal tract disease.

In summary, we present a case of radiation recall dermatitis triggered by prednisone. Radiation can prime an area for a future inflammatory response by upregulating proinflammatory cytokines or triggering stem cell mutation. In our case, clinical reactivity to hydrocortisone-17-butyrate and sensitization to tixocortol pivalate via patch testing could have increased the likelihood of a reaction with prednisone use due to cross-reactivity. This case instructs dermatologists, allergists, and oncologists to be aware of prednisone as a potential trigger of radiation recall dermatitis.

To the Editor:

A 69-year-old woman presented to the allergy clinic for evaluation of a rash on the left breast. The patient had a history of breast cancer that was treated with a lumpectomy followed by external beam radiation therapy (total dose, 6000 cGy) to the lateral aspect of the left breast approximately 4 years prior. She developed acute breast dermatitis from the radiation, which was self-treated with over-the-counter hydrocortisone cream. The patient subsequently developed a blistering skin eruption over the area where she applied the cream. She did not recall the subtype of hydrocortisone she used (butyrate and acetate are available over-the-counter). She discontinued the hydrocortisone and was started on triamcinolone cream 0.1%, which was well tolerated, and the rash resolved.

The patient had a history of a similar reaction to hydrocortisone butyrate after blepharoplasty approximately 10 years prior to the current presentation, characterized by facial erythema, pruritus, and blistering. A patch test confirmed reactivity to hydrocortisone-17-butyrate and tixocortol pivalate. However, a skin-prick test for hydrocortisone acetate cream 1% was negative.

Subsequently, the patient developed acute-onset dyspepsia, gnawing epigastric pain, regurgitation, and bloating. A diagnosis of eosinophilic gastritis was established via biopsy, which found increased eosinophils in the lamina propria (>50 eosinophils per high-power field). Helicobacter pylori was not identified. She was started on the proton-pump inhibitor dexlansoprazole but symptoms did not improve. Her other medications included benazepril, alprazolam as needed, vitamin D, and magnesium. The patient subsequently was started on a trial of oral prednisone 40 mg/d. Three days after initiation, she developed an erythematous macular rash over the left breast.

The next day she presented to the allergy clinic. Physical examination of the left breast revealed a 20×10-cm, nipple-sparing patch of well-demarcated erythema without fluctuance or overlying lesions. The area of erythema overlapped with the prior radiation field based on radiation marker tattoos and the lumpectomy scar (Figure). There was no evidence to suggest inflammation of deeper tissue or the pectoral muscles. Vital signs were normal, and the remainder of the examination was unremarkable, including breast, lymph node, and complete skin examinations.

In contrast, triamcinolone is a class B steroid, which has a C16,17-cis-diol or -ketal. Other than budesonide, which can cross-react with D2 steroids, class B steroids do not cross-react with hydrocortisone or prednisone. Triamcinolone does not usually cross-react with D2 corticosteroids, which likely explains why our patient was later able to tolerate triamcinolone to treat eosinophilic gastrointestinal tract disease.

In summary, we present a case of radiation recall dermatitis triggered by prednisone. Radiation can prime an area for a future inflammatory response by upregulating proinflammatory cytokines or triggering stem cell mutation. In our case, clinical reactivity to hydrocortisone-17-butyrate and sensitization to tixocortol pivalate via patch testing could have increased the likelihood of a reaction with prednisone use due to cross-reactivity. This case instructs dermatologists, allergists, and oncologists to be aware of prednisone as a potential trigger of radiation recall dermatitis.

To the Editor:

A 69-year-old woman presented to the allergy clinic for evaluation of a rash on the left breast. The patient had a history of breast cancer that was treated with a lumpectomy followed by external beam radiation therapy (total dose, 6000 cGy) to the lateral aspect of the left breast approximately 4 years prior. She developed acute breast dermatitis from the radiation, which was self-treated with over-the-counter hydrocortisone cream. The patient subsequently developed a blistering skin eruption over the area where she applied the cream. She did not recall the subtype of hydrocortisone she used (butyrate and acetate are available over-the-counter). She discontinued the hydrocortisone and was started on triamcinolone cream 0.1%, which was well tolerated, and the rash resolved.

The patient had a history of a similar reaction to hydrocortisone butyrate after blepharoplasty approximately 10 years prior to the current presentation, characterized by facial erythema, pruritus, and blistering. A patch test confirmed reactivity to hydrocortisone-17-butyrate and tixocortol pivalate. However, a skin-prick test for hydrocortisone acetate cream 1% was negative.

Subsequently, the patient developed acute-onset dyspepsia, gnawing epigastric pain, regurgitation, and bloating. A diagnosis of eosinophilic gastritis was established via biopsy, which found increased eosinophils in the lamina propria (>50 eosinophils per high-power field). Helicobacter pylori was not identified. She was started on the proton-pump inhibitor dexlansoprazole but symptoms did not improve. Her other medications included benazepril, alprazolam as needed, vitamin D, and magnesium. The patient subsequently was started on a trial of oral prednisone 40 mg/d. Three days after initiation, she developed an erythematous macular rash over the left breast.

The next day she presented to the allergy clinic. Physical examination of the left breast revealed a 20×10-cm, nipple-sparing patch of well-demarcated erythema without fluctuance or overlying lesions. The area of erythema overlapped with the prior radiation field based on radiation marker tattoos and the lumpectomy scar (Figure). There was no evidence to suggest inflammation of deeper tissue or the pectoral muscles. Vital signs were normal, and the remainder of the examination was unremarkable, including breast, lymph node, and complete skin examinations.

In contrast, triamcinolone is a class B steroid, which has a C16,17-cis-diol or -ketal. Other than budesonide, which can cross-react with D2 steroids, class B steroids do not cross-react with hydrocortisone or prednisone. Triamcinolone does not usually cross-react with D2 corticosteroids, which likely explains why our patient was later able to tolerate triamcinolone to treat eosinophilic gastrointestinal tract disease.

In summary, we present a case of radiation recall dermatitis triggered by prednisone. Radiation can prime an area for a future inflammatory response by upregulating proinflammatory cytokines or triggering stem cell mutation. In our case, clinical reactivity to hydrocortisone-17-butyrate and sensitization to tixocortol pivalate via patch testing could have increased the likelihood of a reaction with prednisone use due to cross-reactivity. This case instructs dermatologists, allergists, and oncologists to be aware of prednisone as a potential trigger of radiation recall dermatitis.

To the Editor:

Mycosis fungoides (MF) is the most common type of primary cutaneous lymphoma, occurring in approximately 4 of 1 million individuals per year in the United States.¹ It classically occurs in patch, plaque, and tumor stages with lesions preferentially occurring on regions of the body spared from sun exposure²; however, MF is known to have variable presentations and has been reported to imitate at least 25 other dermatoses.³ This case describes MF as a morbilliform eruption mimicking a viral exanthem.

A 30-year-old man with a 12-year history of nodular sclerosing Hodgkin lymphoma (HL) presented with a widespread rash of 2 weeks’ duration. At the time of diagnosis of HL, the patient had several slightly enlarged, hyperdense, bilateral inguinal lymph nodes seen on positron emission tomography–computed tomography. He achieved complete remission 11 years prior after 6 cycles of ABVD (doxorubicin-bleomycin-vinblastine-dacarbazine) chemotherapy. He initially presented to us prior to starting chemotherapy for evaluation of what he described as eczema on the bilateral arms and legs that had been present for 10 years. Findings from a skin biopsy of an erythematous scaling patch on the left lateral thigh were consistent with MF. One year later, new lesions on the left lateral thigh were clinically and histologically consistent with lymphomatoid papulosis (LyP).

At the current presentation, the patient denied any changes in medications, which consisted of topical clobetasol, triamcinolone, and mupirocin; however, he reported that his young child had recently been diagnosed with bronchitis and impetigo. Physical examination revealed pink-orange macules and papules on the anterior and posterior trunk, medial upper arms, and bilateral legs involving 18% of the body surface area. A complete blood cell count showed no leukocytosis or left shift. A respiratory viral panel was positive for human metapneumovirus. Two weeks later, the patient noted improvement of the rash with use of topical triamcinolone.

Four months later, the rash still had not completely resolved and now involved 50% of the body surface area. A punch biopsy of the left lower abdomen demonstrated an atypical lymphoid infiltrate with focal epidermotropism and predominance of CD4 over CD8 cells (approximately 4:1 ratio), and CD30 labeled rare cells. Polymerase chain reaction analysis of the biopsy revealed monoclonal T-cell receptor gamma chain gene rearrangement. Taken together, the findings were consistent with MF. The patient started narrowband UVB phototherapy and completed a total of 25 treatments, reaching a maximum 4-minute dose, with minimal improvement.

Three months later, the patient had 90% body surface area involvement and started treatment with intramuscular interferon alfa-2b at 1 million units 3 times weekly. He noticed improvement within the first week of treatment and reported that his skin was clear until 5 months later when he woke up one morning with a morbilliform eruption on the anterior trunk, thighs, and upper arms (Figure 1). Biopsy from the right thigh showed an infiltrate of CD3⁺ lymphocytes with a predominance of CD4 over CD8 cells (approximately 6:1 ratio), both in the dermis and epidermis (Figure 2). CD30 highlighted approximately 10% of cells (Figure 3). Findings again were consistent with MF. Flow cytometry was negative for peripheral blood involvement.

Three months later, the patient reported enlargement of several left inguinal nodes. Fine needle aspiration of 1 node demonstrated an atypical lymphoid proliferation consistent with MF. Positron emission tomography–computed tomography showed several mildly enlarged inguinal lymph nodes, which were unchanged from the initial diagnosis of HL. There were no hypermetabolic lesions. One month later, the patient started extracorporeal electrophoresis in addition to interferon alfa-2b with notable improvement of the rash. The rash later recurred after completion of these treatments and continues to have a waxing and waning course. It is currently managed with triamcinolone cream only.

At the time of the initial diagnosis of MF, the patient’s lesions appeared as eczematous patches on the face, abdomen, buttocks, and legs. Based on the history of a sick child at home, viral panel positive for human metapneumovirus, and clinical appearance, a viral exanthem was considered to be a likely explanation for the patient’s new-onset morbilliform eruption rash occurring 12 years later. A drug reaction also was considered in the differential based on the appearance of the rash; however, it was deemed less likely because the patient reported no changes in his medications at the time of rash onset. Persistence of the eruption for many months was less consistent with a reactive condition. A biopsy demonstrated the rash to be histologically consistent with MF. This patient was a rare case of MF manifesting as a morbilliform eruption mimicking a viral exanthem.

Various inflammatory conditions, including drug eruptions and lichen sclerosus et atrophicus, may mimic MF, not only based on their histophenotypic findings but also occasionally clonal proliferation by molecular study.^4,5 In our patient, one consideration was the possibility of a viral infection mimicking MF; however, biopsies showed both definite histophenotypic features of MF and clonality. More importantly, subsequent biopsy also revealed similar findings by morphology, immunohistochemical study, and T-cell gene rearrangement study, confirming the diagnosis of MF.

Another interesting feature of our case was the occurrence of HL, LyP, and MF in the same patient. Lymphomatoid papulosis is a chronic condition characterized by self-healing lesions and histologic features suggestive of malignancy that lies within a spectrum of primary cutaneous CD30⁺ lymphoproliferative disorders. There is a known association between LyP and an increased incidence of lymphomas, including MF and HL.¹ In a 2016 study, lymphomas occurred in 52% of patients with LyP (N=180), with MF being the most frequently associated lymphoma.⁶ Notably, biopsies consistent with both HL and MF, respectively, in our patient were positive for the CD30 marker. Patients with HL also are at increased risk for developing other malignancies, with the risk of leukemias and non-HLs greater than that of solid tumors.⁵ There have been multiple reported cases of HL and MF occurring in the same patient and at least one prior reported case of LyP, HL, and MF occurring in the same patient.^6,7

This case highlights the myriad presentations of MF and describes an unusual case of MF manifesting as a morbilliform eruption mimicking a viral exanthem.

To the Editor:

Mycosis fungoides (MF) is the most common type of primary cutaneous lymphoma, occurring in approximately 4 of 1 million individuals per year in the United States.¹ It classically occurs in patch, plaque, and tumor stages with lesions preferentially occurring on regions of the body spared from sun exposure²; however, MF is known to have variable presentations and has been reported to imitate at least 25 other dermatoses.³ This case describes MF as a morbilliform eruption mimicking a viral exanthem.

A 30-year-old man with a 12-year history of nodular sclerosing Hodgkin lymphoma (HL) presented with a widespread rash of 2 weeks’ duration. At the time of diagnosis of HL, the patient had several slightly enlarged, hyperdense, bilateral inguinal lymph nodes seen on positron emission tomography–computed tomography. He achieved complete remission 11 years prior after 6 cycles of ABVD (doxorubicin-bleomycin-vinblastine-dacarbazine) chemotherapy. He initially presented to us prior to starting chemotherapy for evaluation of what he described as eczema on the bilateral arms and legs that had been present for 10 years. Findings from a skin biopsy of an erythematous scaling patch on the left lateral thigh were consistent with MF. One year later, new lesions on the left lateral thigh were clinically and histologically consistent with lymphomatoid papulosis (LyP).

At the current presentation, the patient denied any changes in medications, which consisted of topical clobetasol, triamcinolone, and mupirocin; however, he reported that his young child had recently been diagnosed with bronchitis and impetigo. Physical examination revealed pink-orange macules and papules on the anterior and posterior trunk, medial upper arms, and bilateral legs involving 18% of the body surface area. A complete blood cell count showed no leukocytosis or left shift. A respiratory viral panel was positive for human metapneumovirus. Two weeks later, the patient noted improvement of the rash with use of topical triamcinolone.

Four months later, the rash still had not completely resolved and now involved 50% of the body surface area. A punch biopsy of the left lower abdomen demonstrated an atypical lymphoid infiltrate with focal epidermotropism and predominance of CD4 over CD8 cells (approximately 4:1 ratio), and CD30 labeled rare cells. Polymerase chain reaction analysis of the biopsy revealed monoclonal T-cell receptor gamma chain gene rearrangement. Taken together, the findings were consistent with MF. The patient started narrowband UVB phototherapy and completed a total of 25 treatments, reaching a maximum 4-minute dose, with minimal improvement.

Three months later, the patient had 90% body surface area involvement and started treatment with intramuscular interferon alfa-2b at 1 million units 3 times weekly. He noticed improvement within the first week of treatment and reported that his skin was clear until 5 months later when he woke up one morning with a morbilliform eruption on the anterior trunk, thighs, and upper arms (Figure 1). Biopsy from the right thigh showed an infiltrate of CD3⁺ lymphocytes with a predominance of CD4 over CD8 cells (approximately 6:1 ratio), both in the dermis and epidermis (Figure 2). CD30 highlighted approximately 10% of cells (Figure 3). Findings again were consistent with MF. Flow cytometry was negative for peripheral blood involvement.

Three months later, the patient reported enlargement of several left inguinal nodes. Fine needle aspiration of 1 node demonstrated an atypical lymphoid proliferation consistent with MF. Positron emission tomography–computed tomography showed several mildly enlarged inguinal lymph nodes, which were unchanged from the initial diagnosis of HL. There were no hypermetabolic lesions. One month later, the patient started extracorporeal electrophoresis in addition to interferon alfa-2b with notable improvement of the rash. The rash later recurred after completion of these treatments and continues to have a waxing and waning course. It is currently managed with triamcinolone cream only.

At the time of the initial diagnosis of MF, the patient’s lesions appeared as eczematous patches on the face, abdomen, buttocks, and legs. Based on the history of a sick child at home, viral panel positive for human metapneumovirus, and clinical appearance, a viral exanthem was considered to be a likely explanation for the patient’s new-onset morbilliform eruption rash occurring 12 years later. A drug reaction also was considered in the differential based on the appearance of the rash; however, it was deemed less likely because the patient reported no changes in his medications at the time of rash onset. Persistence of the eruption for many months was less consistent with a reactive condition. A biopsy demonstrated the rash to be histologically consistent with MF. This patient was a rare case of MF manifesting as a morbilliform eruption mimicking a viral exanthem.

Various inflammatory conditions, including drug eruptions and lichen sclerosus et atrophicus, may mimic MF, not only based on their histophenotypic findings but also occasionally clonal proliferation by molecular study.^4,5 In our patient, one consideration was the possibility of a viral infection mimicking MF; however, biopsies showed both definite histophenotypic features of MF and clonality. More importantly, subsequent biopsy also revealed similar findings by morphology, immunohistochemical study, and T-cell gene rearrangement study, confirming the diagnosis of MF.

Another interesting feature of our case was the occurrence of HL, LyP, and MF in the same patient. Lymphomatoid papulosis is a chronic condition characterized by self-healing lesions and histologic features suggestive of malignancy that lies within a spectrum of primary cutaneous CD30⁺ lymphoproliferative disorders. There is a known association between LyP and an increased incidence of lymphomas, including MF and HL.¹ In a 2016 study, lymphomas occurred in 52% of patients with LyP (N=180), with MF being the most frequently associated lymphoma.⁶ Notably, biopsies consistent with both HL and MF, respectively, in our patient were positive for the CD30 marker. Patients with HL also are at increased risk for developing other malignancies, with the risk of leukemias and non-HLs greater than that of solid tumors.⁵ There have been multiple reported cases of HL and MF occurring in the same patient and at least one prior reported case of LyP, HL, and MF occurring in the same patient.^6,7

This case highlights the myriad presentations of MF and describes an unusual case of MF manifesting as a morbilliform eruption mimicking a viral exanthem.

To the Editor:

Mycosis fungoides (MF) is the most common type of primary cutaneous lymphoma, occurring in approximately 4 of 1 million individuals per year in the United States.¹ It classically occurs in patch, plaque, and tumor stages with lesions preferentially occurring on regions of the body spared from sun exposure²; however, MF is known to have variable presentations and has been reported to imitate at least 25 other dermatoses.³ This case describes MF as a morbilliform eruption mimicking a viral exanthem.

A 30-year-old man with a 12-year history of nodular sclerosing Hodgkin lymphoma (HL) presented with a widespread rash of 2 weeks’ duration. At the time of diagnosis of HL, the patient had several slightly enlarged, hyperdense, bilateral inguinal lymph nodes seen on positron emission tomography–computed tomography. He achieved complete remission 11 years prior after 6 cycles of ABVD (doxorubicin-bleomycin-vinblastine-dacarbazine) chemotherapy. He initially presented to us prior to starting chemotherapy for evaluation of what he described as eczema on the bilateral arms and legs that had been present for 10 years. Findings from a skin biopsy of an erythematous scaling patch on the left lateral thigh were consistent with MF. One year later, new lesions on the left lateral thigh were clinically and histologically consistent with lymphomatoid papulosis (LyP).

At the current presentation, the patient denied any changes in medications, which consisted of topical clobetasol, triamcinolone, and mupirocin; however, he reported that his young child had recently been diagnosed with bronchitis and impetigo. Physical examination revealed pink-orange macules and papules on the anterior and posterior trunk, medial upper arms, and bilateral legs involving 18% of the body surface area. A complete blood cell count showed no leukocytosis or left shift. A respiratory viral panel was positive for human metapneumovirus. Two weeks later, the patient noted improvement of the rash with use of topical triamcinolone.

Four months later, the rash still had not completely resolved and now involved 50% of the body surface area. A punch biopsy of the left lower abdomen demonstrated an atypical lymphoid infiltrate with focal epidermotropism and predominance of CD4 over CD8 cells (approximately 4:1 ratio), and CD30 labeled rare cells. Polymerase chain reaction analysis of the biopsy revealed monoclonal T-cell receptor gamma chain gene rearrangement. Taken together, the findings were consistent with MF. The patient started narrowband UVB phototherapy and completed a total of 25 treatments, reaching a maximum 4-minute dose, with minimal improvement.

Three months later, the patient had 90% body surface area involvement and started treatment with intramuscular interferon alfa-2b at 1 million units 3 times weekly. He noticed improvement within the first week of treatment and reported that his skin was clear until 5 months later when he woke up one morning with a morbilliform eruption on the anterior trunk, thighs, and upper arms (Figure 1). Biopsy from the right thigh showed an infiltrate of CD3⁺ lymphocytes with a predominance of CD4 over CD8 cells (approximately 6:1 ratio), both in the dermis and epidermis (Figure 2). CD30 highlighted approximately 10% of cells (Figure 3). Findings again were consistent with MF. Flow cytometry was negative for peripheral blood involvement.

Three months later, the patient reported enlargement of several left inguinal nodes. Fine needle aspiration of 1 node demonstrated an atypical lymphoid proliferation consistent with MF. Positron emission tomography–computed tomography showed several mildly enlarged inguinal lymph nodes, which were unchanged from the initial diagnosis of HL. There were no hypermetabolic lesions. One month later, the patient started extracorporeal electrophoresis in addition to interferon alfa-2b with notable improvement of the rash. The rash later recurred after completion of these treatments and continues to have a waxing and waning course. It is currently managed with triamcinolone cream only.

At the time of the initial diagnosis of MF, the patient’s lesions appeared as eczematous patches on the face, abdomen, buttocks, and legs. Based on the history of a sick child at home, viral panel positive for human metapneumovirus, and clinical appearance, a viral exanthem was considered to be a likely explanation for the patient’s new-onset morbilliform eruption rash occurring 12 years later. A drug reaction also was considered in the differential based on the appearance of the rash; however, it was deemed less likely because the patient reported no changes in his medications at the time of rash onset. Persistence of the eruption for many months was less consistent with a reactive condition. A biopsy demonstrated the rash to be histologically consistent with MF. This patient was a rare case of MF manifesting as a morbilliform eruption mimicking a viral exanthem.

Various inflammatory conditions, including drug eruptions and lichen sclerosus et atrophicus, may mimic MF, not only based on their histophenotypic findings but also occasionally clonal proliferation by molecular study.^4,5 In our patient, one consideration was the possibility of a viral infection mimicking MF; however, biopsies showed both definite histophenotypic features of MF and clonality. More importantly, subsequent biopsy also revealed similar findings by morphology, immunohistochemical study, and T-cell gene rearrangement study, confirming the diagnosis of MF.

Another interesting feature of our case was the occurrence of HL, LyP, and MF in the same patient. Lymphomatoid papulosis is a chronic condition characterized by self-healing lesions and histologic features suggestive of malignancy that lies within a spectrum of primary cutaneous CD30⁺ lymphoproliferative disorders. There is a known association between LyP and an increased incidence of lymphomas, including MF and HL.¹ In a 2016 study, lymphomas occurred in 52% of patients with LyP (N=180), with MF being the most frequently associated lymphoma.⁶ Notably, biopsies consistent with both HL and MF, respectively, in our patient were positive for the CD30 marker. Patients with HL also are at increased risk for developing other malignancies, with the risk of leukemias and non-HLs greater than that of solid tumors.⁵ There have been multiple reported cases of HL and MF occurring in the same patient and at least one prior reported case of LyP, HL, and MF occurring in the same patient.^6,7

This case highlights the myriad presentations of MF and describes an unusual case of MF manifesting as a morbilliform eruption mimicking a viral exanthem.

To the Editor:

Erythema induratum, also known as nodular vasculitis, is a panniculitis that usually affects the lower extremities in middle-aged women. Classically, it has been described as a delayed-type hypersensitivity reaction to Mycobacterium tuberculosis, also known as a tuberculid.^1,2 Other infections, however, also have been implicated as causes of erythema induratum, including bacillus Calmette-Guérin (BCG), the attenuated form of Mycobacterium bovis, which commonly is used for tuberculosis vaccination. Medications also may cause erythema induratum. The characteristic distribution of the nodules on the posterior calves helps to distinguish erythema induratum from other panniculitides. A PubMed search of articles indexed for MEDLINE using the term disseminated erythema induratum revealed few case reports documenting nodules on the arms, thighs, or chest, and only 1 case report of disseminated erythema induratum.^3-8 We describe a rare combination of disseminated erythema induratum in a patient with remote exposure to tuberculosis and recent BCG exposure.

An 88-year-old woman presented for evaluation of violaceous, minimally tender, nonulcerated, subcutaneous nodules on the legs, arms, and trunk of several weeks’ duration (Figure 1). She had a remote history of tuberculosis as a child, prior to the advent of modern antituberculosis regimens. Her medical history also included hypertension, breast cancer treated with lymph node dissection, gastroesophageal reflux disease, and bladder cancer treated with intravesical BCG 10 years prior to the onset of the nodules. She reported minimal coughing and a 25-lb weight loss over the last year, but she denied night sweats, fever, or chills.

Disseminated erythema induratum, an exceptionally rare variant of panniculitis, may be seen in patients with a remote history of M tuberculosis exposure and/or recent therapeutic BCG exposure. It is imperative to rule out active tuberculosis, especially in elderly patients whose disease predated the advent of modern antituberculosis therapy. Using an IFN-γ release assay in addition to chest radiographs and other clinical stigmata allows differentiation of the etiology of erythema induratum in those patients with tuberculosis who also were treated with BCG.

To the Editor:

Erythema induratum, also known as nodular vasculitis, is a panniculitis that usually affects the lower extremities in middle-aged women. Classically, it has been described as a delayed-type hypersensitivity reaction to Mycobacterium tuberculosis, also known as a tuberculid.^1,2 Other infections, however, also have been implicated as causes of erythema induratum, including bacillus Calmette-Guérin (BCG), the attenuated form of Mycobacterium bovis, which commonly is used for tuberculosis vaccination. Medications also may cause erythema induratum. The characteristic distribution of the nodules on the posterior calves helps to distinguish erythema induratum from other panniculitides. A PubMed search of articles indexed for MEDLINE using the term disseminated erythema induratum revealed few case reports documenting nodules on the arms, thighs, or chest, and only 1 case report of disseminated erythema induratum.^3-8 We describe a rare combination of disseminated erythema induratum in a patient with remote exposure to tuberculosis and recent BCG exposure.

An 88-year-old woman presented for evaluation of violaceous, minimally tender, nonulcerated, subcutaneous nodules on the legs, arms, and trunk of several weeks’ duration (Figure 1). She had a remote history of tuberculosis as a child, prior to the advent of modern antituberculosis regimens. Her medical history also included hypertension, breast cancer treated with lymph node dissection, gastroesophageal reflux disease, and bladder cancer treated with intravesical BCG 10 years prior to the onset of the nodules. She reported minimal coughing and a 25-lb weight loss over the last year, but she denied night sweats, fever, or chills.

Disseminated erythema induratum, an exceptionally rare variant of panniculitis, may be seen in patients with a remote history of M tuberculosis exposure and/or recent therapeutic BCG exposure. It is imperative to rule out active tuberculosis, especially in elderly patients whose disease predated the advent of modern antituberculosis therapy. Using an IFN-γ release assay in addition to chest radiographs and other clinical stigmata allows differentiation of the etiology of erythema induratum in those patients with tuberculosis who also were treated with BCG.

To the Editor:

Erythema induratum, also known as nodular vasculitis, is a panniculitis that usually affects the lower extremities in middle-aged women. Classically, it has been described as a delayed-type hypersensitivity reaction to Mycobacterium tuberculosis, also known as a tuberculid.^1,2 Other infections, however, also have been implicated as causes of erythema induratum, including bacillus Calmette-Guérin (BCG), the attenuated form of Mycobacterium bovis, which commonly is used for tuberculosis vaccination. Medications also may cause erythema induratum. The characteristic distribution of the nodules on the posterior calves helps to distinguish erythema induratum from other panniculitides. A PubMed search of articles indexed for MEDLINE using the term disseminated erythema induratum revealed few case reports documenting nodules on the arms, thighs, or chest, and only 1 case report of disseminated erythema induratum.^3-8 We describe a rare combination of disseminated erythema induratum in a patient with remote exposure to tuberculosis and recent BCG exposure.

An 88-year-old woman presented for evaluation of violaceous, minimally tender, nonulcerated, subcutaneous nodules on the legs, arms, and trunk of several weeks’ duration (Figure 1). She had a remote history of tuberculosis as a child, prior to the advent of modern antituberculosis regimens. Her medical history also included hypertension, breast cancer treated with lymph node dissection, gastroesophageal reflux disease, and bladder cancer treated with intravesical BCG 10 years prior to the onset of the nodules. She reported minimal coughing and a 25-lb weight loss over the last year, but she denied night sweats, fever, or chills.

Disseminated erythema induratum, an exceptionally rare variant of panniculitis, may be seen in patients with a remote history of M tuberculosis exposure and/or recent therapeutic BCG exposure. It is imperative to rule out active tuberculosis, especially in elderly patients whose disease predated the advent of modern antituberculosis therapy. Using an IFN-γ release assay in addition to chest radiographs and other clinical stigmata allows differentiation of the etiology of erythema induratum in those patients with tuberculosis who also were treated with BCG.