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Man Seeks Treatment for Periodic “Eruptions”
The correct answer is benign familial pemphigus (choice “b”). Also known as Hailey-Hailey disease, this is an unusual autosomally inherited blistering disease.
Benign familial pemphigus (BFP) is often mistaken for bacterial infection, such as pyoderma (choice “a”) or impetigo (choice “c”). Although it can become secondarily infected, its origins are entirely different.
Contact dermatitis (choice “d”) in its more severe forms can present in a similar manner. However, it would have shown entirely different changes (acute inflammation and spongiosis) on biopsy.
See next page for the discussion...
DISCUSSION
In 1939, two dermatologist-brothers in Georgia saw a patient with this previously unreported condition. They uncovered the family history and worked out the histologic basis, which they then described in the literature. They named the condition benign familial pemphigus, but it is now more commonly known as Hailey-Hailey disease in their honor.
Pemphigus vulgaris (PV), a serious blistering disease, was more common and far more feared at the time of the Hailey brothers’ discovery. Nearly 100% of PV patients died from the condition in that pre-steroid, pre-antibiotic era (most from secondary bacterial infection).
Fortunately, BFP is more benign, though it shares some features with PV. Both are said to be Nikolsky-positive, meaning the initial blisters can be extended with digital pressure. But BFP, unlike PV, does not involve deposition of immunoglobulins (IgA in the case of PV), nor is it accompanied by circulating auto-antibodies. BFP patients typically have no systemic symptoms, whereas in those with PV, the oral mucosae are often affected.
Herpes simplex virus, which was the primary care provider’s initial suspected diagnosis, can cause somewhat similar outbreaks, even in this area. However, it was effectively ruled out by the lack of response to treatment and by the biopsy results.
Although BFP is an inherited condition, it demonstrates variable penetrance, as in our case. It is rare enough that diagnosis is almost invariably delayed while other diagnoses are considered and treated. The actual “lesion” of BFP is still debated, but appears to involve the quality and quantity of desmosomes (microscopic structures that act as connecting fibers between layers of tissue) breaking down, often because of heat and friction, eventuating in blistering. This theory is bolstered by considerable research and by the fact that most cases present in intertriginous areas, such as the neck, axillae, and groin. Appearing episodically, it typically begins in the third to fourth decade of life, tending to diminish with age.
Biopsy is often necessary to confirm the diagnosis of BFP, with the sample best taken from perilesional skin to avoid separation of friable sample fragments. Additional specimens can be taken for special handling (Michel’s media) to detect immunoglobulins that might be seen in other blistering diseases.
See next page for treatment...
TREATMENT
BFP can be treated empirically with application of a soothing solution of aluminum acetate, or more specifically with topical corticosteroids (class III to IV) and topical antibiotics (eg, clindamycin 2% solution), plus/minus oral minocycline, which has potent anti-inflammatory as well as antimicrobial effects.
Difficult cases should be referred to dermatology, which has a number of treatments at its disposal. This includes diaminodiphenyl sulfone (dapsone), systemic glucocorticoids, methotrexate, systemic retinoids, and even local injection of botulinum toxin to decrease local hidrosis.
This patient is responding well to a regimen of oral minocycline 100 mg bid, topical clindamycin 2% bid application, and topical tacrolimus.
The correct answer is benign familial pemphigus (choice “b”). Also known as Hailey-Hailey disease, this is an unusual autosomally inherited blistering disease.
Benign familial pemphigus (BFP) is often mistaken for bacterial infection, such as pyoderma (choice “a”) or impetigo (choice “c”). Although it can become secondarily infected, its origins are entirely different.
Contact dermatitis (choice “d”) in its more severe forms can present in a similar manner. However, it would have shown entirely different changes (acute inflammation and spongiosis) on biopsy.
See next page for the discussion...
DISCUSSION
In 1939, two dermatologist-brothers in Georgia saw a patient with this previously unreported condition. They uncovered the family history and worked out the histologic basis, which they then described in the literature. They named the condition benign familial pemphigus, but it is now more commonly known as Hailey-Hailey disease in their honor.
Pemphigus vulgaris (PV), a serious blistering disease, was more common and far more feared at the time of the Hailey brothers’ discovery. Nearly 100% of PV patients died from the condition in that pre-steroid, pre-antibiotic era (most from secondary bacterial infection).
Fortunately, BFP is more benign, though it shares some features with PV. Both are said to be Nikolsky-positive, meaning the initial blisters can be extended with digital pressure. But BFP, unlike PV, does not involve deposition of immunoglobulins (IgA in the case of PV), nor is it accompanied by circulating auto-antibodies. BFP patients typically have no systemic symptoms, whereas in those with PV, the oral mucosae are often affected.
Herpes simplex virus, which was the primary care provider’s initial suspected diagnosis, can cause somewhat similar outbreaks, even in this area. However, it was effectively ruled out by the lack of response to treatment and by the biopsy results.
Although BFP is an inherited condition, it demonstrates variable penetrance, as in our case. It is rare enough that diagnosis is almost invariably delayed while other diagnoses are considered and treated. The actual “lesion” of BFP is still debated, but appears to involve the quality and quantity of desmosomes (microscopic structures that act as connecting fibers between layers of tissue) breaking down, often because of heat and friction, eventuating in blistering. This theory is bolstered by considerable research and by the fact that most cases present in intertriginous areas, such as the neck, axillae, and groin. Appearing episodically, it typically begins in the third to fourth decade of life, tending to diminish with age.
Biopsy is often necessary to confirm the diagnosis of BFP, with the sample best taken from perilesional skin to avoid separation of friable sample fragments. Additional specimens can be taken for special handling (Michel’s media) to detect immunoglobulins that might be seen in other blistering diseases.
See next page for treatment...
TREATMENT
BFP can be treated empirically with application of a soothing solution of aluminum acetate, or more specifically with topical corticosteroids (class III to IV) and topical antibiotics (eg, clindamycin 2% solution), plus/minus oral minocycline, which has potent anti-inflammatory as well as antimicrobial effects.
Difficult cases should be referred to dermatology, which has a number of treatments at its disposal. This includes diaminodiphenyl sulfone (dapsone), systemic glucocorticoids, methotrexate, systemic retinoids, and even local injection of botulinum toxin to decrease local hidrosis.
This patient is responding well to a regimen of oral minocycline 100 mg bid, topical clindamycin 2% bid application, and topical tacrolimus.
The correct answer is benign familial pemphigus (choice “b”). Also known as Hailey-Hailey disease, this is an unusual autosomally inherited blistering disease.
Benign familial pemphigus (BFP) is often mistaken for bacterial infection, such as pyoderma (choice “a”) or impetigo (choice “c”). Although it can become secondarily infected, its origins are entirely different.
Contact dermatitis (choice “d”) in its more severe forms can present in a similar manner. However, it would have shown entirely different changes (acute inflammation and spongiosis) on biopsy.
See next page for the discussion...
DISCUSSION
In 1939, two dermatologist-brothers in Georgia saw a patient with this previously unreported condition. They uncovered the family history and worked out the histologic basis, which they then described in the literature. They named the condition benign familial pemphigus, but it is now more commonly known as Hailey-Hailey disease in their honor.
Pemphigus vulgaris (PV), a serious blistering disease, was more common and far more feared at the time of the Hailey brothers’ discovery. Nearly 100% of PV patients died from the condition in that pre-steroid, pre-antibiotic era (most from secondary bacterial infection).
Fortunately, BFP is more benign, though it shares some features with PV. Both are said to be Nikolsky-positive, meaning the initial blisters can be extended with digital pressure. But BFP, unlike PV, does not involve deposition of immunoglobulins (IgA in the case of PV), nor is it accompanied by circulating auto-antibodies. BFP patients typically have no systemic symptoms, whereas in those with PV, the oral mucosae are often affected.
Herpes simplex virus, which was the primary care provider’s initial suspected diagnosis, can cause somewhat similar outbreaks, even in this area. However, it was effectively ruled out by the lack of response to treatment and by the biopsy results.
Although BFP is an inherited condition, it demonstrates variable penetrance, as in our case. It is rare enough that diagnosis is almost invariably delayed while other diagnoses are considered and treated. The actual “lesion” of BFP is still debated, but appears to involve the quality and quantity of desmosomes (microscopic structures that act as connecting fibers between layers of tissue) breaking down, often because of heat and friction, eventuating in blistering. This theory is bolstered by considerable research and by the fact that most cases present in intertriginous areas, such as the neck, axillae, and groin. Appearing episodically, it typically begins in the third to fourth decade of life, tending to diminish with age.
Biopsy is often necessary to confirm the diagnosis of BFP, with the sample best taken from perilesional skin to avoid separation of friable sample fragments. Additional specimens can be taken for special handling (Michel’s media) to detect immunoglobulins that might be seen in other blistering diseases.
See next page for treatment...
TREATMENT
BFP can be treated empirically with application of a soothing solution of aluminum acetate, or more specifically with topical corticosteroids (class III to IV) and topical antibiotics (eg, clindamycin 2% solution), plus/minus oral minocycline, which has potent anti-inflammatory as well as antimicrobial effects.
Difficult cases should be referred to dermatology, which has a number of treatments at its disposal. This includes diaminodiphenyl sulfone (dapsone), systemic glucocorticoids, methotrexate, systemic retinoids, and even local injection of botulinum toxin to decrease local hidrosis.
This patient is responding well to a regimen of oral minocycline 100 mg bid, topical clindamycin 2% bid application, and topical tacrolimus.
For three months, a 38-year-old man has been trying to resolve an “eruption” on his neck. The rash burns and itches, though only mildly, and produces clear fluid. His primary care provider initially prescribed acyclovir, then valacyclovir; neither helped. Subsequent courses of oral antibiotics (cephalexin 500 mg qid for three weeks, then ciprofloxacin 500 mg bid for two weeks) also had no beneficial effect. There is no family history of similar outbreaks. The patient, however, has had several of these eruptions—on the face as well as the neck—since his 20s. They typically last two to four weeks, then disappear completely for months or years. The eruptions tend to occur in the summer. He denies any history of cold sores and does not recall any premonitory symptoms prior to this eruption. He further denies any history of atopy or immunosuppression. His health is otherwise excellent, and he is taking no prescription medications. The denuded area measures about 8 x 4 cm, from his nuchal scalp down to the C6 area of the posterior neck. Discrete ruptured vesicles are seen on the periphery of the site. A layer of peeling skin, resembling wet toilet tissue, covers the partially denuded central portion, at the base of which is distinctly erythematous underlying raw tissue. There is no erythema surrounding the lesion, and no nodes are palpable in the area. A 4-mm punch biopsy is performed, with a sample taken from the periphery of the lesion and submitted for routine handling. It shows a hyperplastic epithelium, as well as intradermal and suprabasilar acantholysis extending focally into the spinous layer.
A 10-Year Wait for Diagnosis
An 84-year-old woman has had a lesion on her face for 10 years. During that time, it has slowly changed, becoming darker and larger. For the past few months, it has developed a new, raised area in its center.
Several providers, including a dermatologist, have seen the lesion over the years; none has ever ordered a biopsy. The lesion is asymptomatic and only began to change noticeably a few years ago.
The patient mentions having multiple sunburns as a young woman. But she says she never tried to tan, because she could only do so with difficulty.
Her medical history is significant for breast cancer, for which she is receiving chemotherapy. She was told she did not need surgery and has little chance of metastasis.
EXAMINATION
The patient is alert and cooperative. A large, black, 3.5-cm lesion is obvious on the right jawline. In the center of the lesion is a 2-cm, faintly eroded nodule. No tenderness can be elicited on palpation of the lesion or the area, and there are no palable nodes in the adjacent head and neck. The patient has type 2 skin, blue eyes, and modest evidence of sun damage elsewhere on her face.
DISCUSSION
Of course, the next logical step was to biopsy the lesion. The rule of thumb for biopsy of a possible melanoma is this: Removal of the entire lesion, down to adipose tissue, is the gold standard, because it is certain to provide both an accurate diagnosis and an assessment of the lesion’s vertical thickness—a key predictor of prognosis.
The drawback to complete excision of lesions this size is that the defect can be huge (about 10 cm in this case) and almost certainly disfiguring. If the lesion turns out to be benign, this outcome might well be seen as unacceptable. Therefore, an alternative to complete excision is incisional biopsy, in which a significant but modest portion of the lesion is removed, purposely obtaining the darkest, most irregular part. In this case, such a biopsy would include either all or a significant part of the nodular portion of the lesion, along with a generous section of the discolored macular area.
Shave biopsy of a suspected melanoma is acceptable with small, shallow lesions, as long as it effectively removes the lesion with deep margins. Shave biopsy in this case would be unacceptable for several reasons. Among them is the cosmetic outcome and the possibility of transecting the lesion, thereby making an accurate vertical assessment difficult. The latter is important, since it is the single most accurate prognostic indicator, in effect dictating the margins of re-excision as well as the extent of subsequent workup (imaging, bloodwork, lymph node dissection, and possible chemotherapeutic regimen).
Punch biopsy of suspected melanoma is generally unacceptable, unless the punch effectively removes most or all of the lesion. Referral to dermatology is almost never the wrong thing to do, but it often requires provider-to-provider communication to expedite.
Providers who don’t perform these biopsies often tend to quote the misinformed attending physicians who taught them that “cutting through a melanoma will make it spread.” This is a totally unfounded fear, long since disproven in large studies that showed no difference in mortality or morbidity between patients whose melanomas were incised versus those whose cancers were removed without incision.
DIAGNOSIS AND TREATMENT
Incisional biopsy was performed on this lesion, with about one-third removed by wedge incision. Essentially, the center of the lesion was removed. This was, of course, done under local and sterile conditions, with closure by simple interrupted sutures.
Within 48 hours, the report was issued. It showed a nodular melanoma with 3.7-mm Breslow thickness, definite signs of focal ulceration, and an increased mitotic rate of 6 to 8 per high-powered field—all of which predict a less-than-favorable prognosis.
The patient was scheduled for Mohs surgery, which will likely involve wide excision of the lesion and surrounding skin. She will also have the option of lymph node dissection, done for prognostic value only (as it will add nothing to her potential for survival).
TAKE-HOME LEARNING POINTS
• Biopsy of melanoma (or other skin cancers) cannot cause it to spread.
• The object of biopsying a potential melanoma is to obtain a specimen that, on pathologic examination, not only conveys the correct diagnosis but also allows for accurate staging.
• Successful biopsy entails going deep and wide enough, but without causing unnecessary scarring or deformation.
• The diagnosis and staging then determine the extent of any re-excision and the need for ancillary studies (eg, lymph node dissection, imaging, referral to oncology).
• This particular case also shows that melanoma growth can be unbelievably slow, allowing plenty of opportunity for discovery.
• Unfortunately, given enough time, many melanomas will reach a point at which they will begin to grow vertically, becoming more dangerous and invasive.
An 84-year-old woman has had a lesion on her face for 10 years. During that time, it has slowly changed, becoming darker and larger. For the past few months, it has developed a new, raised area in its center.
Several providers, including a dermatologist, have seen the lesion over the years; none has ever ordered a biopsy. The lesion is asymptomatic and only began to change noticeably a few years ago.
The patient mentions having multiple sunburns as a young woman. But she says she never tried to tan, because she could only do so with difficulty.
Her medical history is significant for breast cancer, for which she is receiving chemotherapy. She was told she did not need surgery and has little chance of metastasis.
EXAMINATION
The patient is alert and cooperative. A large, black, 3.5-cm lesion is obvious on the right jawline. In the center of the lesion is a 2-cm, faintly eroded nodule. No tenderness can be elicited on palpation of the lesion or the area, and there are no palable nodes in the adjacent head and neck. The patient has type 2 skin, blue eyes, and modest evidence of sun damage elsewhere on her face.
DISCUSSION
Of course, the next logical step was to biopsy the lesion. The rule of thumb for biopsy of a possible melanoma is this: Removal of the entire lesion, down to adipose tissue, is the gold standard, because it is certain to provide both an accurate diagnosis and an assessment of the lesion’s vertical thickness—a key predictor of prognosis.
The drawback to complete excision of lesions this size is that the defect can be huge (about 10 cm in this case) and almost certainly disfiguring. If the lesion turns out to be benign, this outcome might well be seen as unacceptable. Therefore, an alternative to complete excision is incisional biopsy, in which a significant but modest portion of the lesion is removed, purposely obtaining the darkest, most irregular part. In this case, such a biopsy would include either all or a significant part of the nodular portion of the lesion, along with a generous section of the discolored macular area.
Shave biopsy of a suspected melanoma is acceptable with small, shallow lesions, as long as it effectively removes the lesion with deep margins. Shave biopsy in this case would be unacceptable for several reasons. Among them is the cosmetic outcome and the possibility of transecting the lesion, thereby making an accurate vertical assessment difficult. The latter is important, since it is the single most accurate prognostic indicator, in effect dictating the margins of re-excision as well as the extent of subsequent workup (imaging, bloodwork, lymph node dissection, and possible chemotherapeutic regimen).
Punch biopsy of suspected melanoma is generally unacceptable, unless the punch effectively removes most or all of the lesion. Referral to dermatology is almost never the wrong thing to do, but it often requires provider-to-provider communication to expedite.
Providers who don’t perform these biopsies often tend to quote the misinformed attending physicians who taught them that “cutting through a melanoma will make it spread.” This is a totally unfounded fear, long since disproven in large studies that showed no difference in mortality or morbidity between patients whose melanomas were incised versus those whose cancers were removed without incision.
DIAGNOSIS AND TREATMENT
Incisional biopsy was performed on this lesion, with about one-third removed by wedge incision. Essentially, the center of the lesion was removed. This was, of course, done under local and sterile conditions, with closure by simple interrupted sutures.
Within 48 hours, the report was issued. It showed a nodular melanoma with 3.7-mm Breslow thickness, definite signs of focal ulceration, and an increased mitotic rate of 6 to 8 per high-powered field—all of which predict a less-than-favorable prognosis.
The patient was scheduled for Mohs surgery, which will likely involve wide excision of the lesion and surrounding skin. She will also have the option of lymph node dissection, done for prognostic value only (as it will add nothing to her potential for survival).
TAKE-HOME LEARNING POINTS
• Biopsy of melanoma (or other skin cancers) cannot cause it to spread.
• The object of biopsying a potential melanoma is to obtain a specimen that, on pathologic examination, not only conveys the correct diagnosis but also allows for accurate staging.
• Successful biopsy entails going deep and wide enough, but without causing unnecessary scarring or deformation.
• The diagnosis and staging then determine the extent of any re-excision and the need for ancillary studies (eg, lymph node dissection, imaging, referral to oncology).
• This particular case also shows that melanoma growth can be unbelievably slow, allowing plenty of opportunity for discovery.
• Unfortunately, given enough time, many melanomas will reach a point at which they will begin to grow vertically, becoming more dangerous and invasive.
An 84-year-old woman has had a lesion on her face for 10 years. During that time, it has slowly changed, becoming darker and larger. For the past few months, it has developed a new, raised area in its center.
Several providers, including a dermatologist, have seen the lesion over the years; none has ever ordered a biopsy. The lesion is asymptomatic and only began to change noticeably a few years ago.
The patient mentions having multiple sunburns as a young woman. But she says she never tried to tan, because she could only do so with difficulty.
Her medical history is significant for breast cancer, for which she is receiving chemotherapy. She was told she did not need surgery and has little chance of metastasis.
EXAMINATION
The patient is alert and cooperative. A large, black, 3.5-cm lesion is obvious on the right jawline. In the center of the lesion is a 2-cm, faintly eroded nodule. No tenderness can be elicited on palpation of the lesion or the area, and there are no palable nodes in the adjacent head and neck. The patient has type 2 skin, blue eyes, and modest evidence of sun damage elsewhere on her face.
DISCUSSION
Of course, the next logical step was to biopsy the lesion. The rule of thumb for biopsy of a possible melanoma is this: Removal of the entire lesion, down to adipose tissue, is the gold standard, because it is certain to provide both an accurate diagnosis and an assessment of the lesion’s vertical thickness—a key predictor of prognosis.
The drawback to complete excision of lesions this size is that the defect can be huge (about 10 cm in this case) and almost certainly disfiguring. If the lesion turns out to be benign, this outcome might well be seen as unacceptable. Therefore, an alternative to complete excision is incisional biopsy, in which a significant but modest portion of the lesion is removed, purposely obtaining the darkest, most irregular part. In this case, such a biopsy would include either all or a significant part of the nodular portion of the lesion, along with a generous section of the discolored macular area.
Shave biopsy of a suspected melanoma is acceptable with small, shallow lesions, as long as it effectively removes the lesion with deep margins. Shave biopsy in this case would be unacceptable for several reasons. Among them is the cosmetic outcome and the possibility of transecting the lesion, thereby making an accurate vertical assessment difficult. The latter is important, since it is the single most accurate prognostic indicator, in effect dictating the margins of re-excision as well as the extent of subsequent workup (imaging, bloodwork, lymph node dissection, and possible chemotherapeutic regimen).
Punch biopsy of suspected melanoma is generally unacceptable, unless the punch effectively removes most or all of the lesion. Referral to dermatology is almost never the wrong thing to do, but it often requires provider-to-provider communication to expedite.
Providers who don’t perform these biopsies often tend to quote the misinformed attending physicians who taught them that “cutting through a melanoma will make it spread.” This is a totally unfounded fear, long since disproven in large studies that showed no difference in mortality or morbidity between patients whose melanomas were incised versus those whose cancers were removed without incision.
DIAGNOSIS AND TREATMENT
Incisional biopsy was performed on this lesion, with about one-third removed by wedge incision. Essentially, the center of the lesion was removed. This was, of course, done under local and sterile conditions, with closure by simple interrupted sutures.
Within 48 hours, the report was issued. It showed a nodular melanoma with 3.7-mm Breslow thickness, definite signs of focal ulceration, and an increased mitotic rate of 6 to 8 per high-powered field—all of which predict a less-than-favorable prognosis.
The patient was scheduled for Mohs surgery, which will likely involve wide excision of the lesion and surrounding skin. She will also have the option of lymph node dissection, done for prognostic value only (as it will add nothing to her potential for survival).
TAKE-HOME LEARNING POINTS
• Biopsy of melanoma (or other skin cancers) cannot cause it to spread.
• The object of biopsying a potential melanoma is to obtain a specimen that, on pathologic examination, not only conveys the correct diagnosis but also allows for accurate staging.
• Successful biopsy entails going deep and wide enough, but without causing unnecessary scarring or deformation.
• The diagnosis and staging then determine the extent of any re-excision and the need for ancillary studies (eg, lymph node dissection, imaging, referral to oncology).
• This particular case also shows that melanoma growth can be unbelievably slow, allowing plenty of opportunity for discovery.
• Unfortunately, given enough time, many melanomas will reach a point at which they will begin to grow vertically, becoming more dangerous and invasive.
Being “Puzzled” as the First Step to Diagnosis
A 43-year-old Hispanic man has a very itchy rash on his left leg. It manifested within a few weeks of the patient starting a new job, one that involved working more hours than he had previously and on rotating shifts, which interfered with his sleep schedule.
For several years, he tried using different products—mostly OTC creams—on it, with no success. Increasingly annoyed by the rash, he finally consulted his family physician, who prescribed a combination clotrimazole/betamethasone cream. This helped a bit, but over time, the rash steadily worsened, and his primary care provider referred him to dermatology.
The patient claims to be otherwise healthy, with no history of atopy. He takes no prescription medications. He admits finding it difficult to refrain from scratching his legs.
EXAMINATION
There are large, slightly purplish, hypertrophic papulosquamous plaques of up to 8 cm on both anterior tibial areas. His arms, trunk, and wrists are rash-free, and his oral mucosal surfaces show no obvious signs of disease.
Punch biopsy shows a hypertrophic epidermis. There is marked obliteration of the dermoepidermal junction by a dense bandlike layer of lymphocytic infiltrate, associated with vacuolar change and necrotic keratinocytes in the basal layer of the epidermis.
DISCUSSION
This is a classic clinical and histologic picture of a dermatosis commonly seen in dermatology offices: lichen planus (LP). As is often the case, by the time the patient gets to dermatology, the clinical picture can be confused and atypical. But there is much to learn from such a case.
LP is the prototypical interface lichenoid dermatitis, which can be depended on to present in certain identifiable ways. In this case, what suggested this diagnosis was the word “puzzling.” It’s the perfect example of the intuitive, somewhat subjective side of dermatologic diagnosis: When I see a condition that baffles me momentarily, my puzzled reaction becomes meaningful because the word puzzling starts with a “p.” Lichen planus has long been associated with an extremely useful mnemonic device known as “The Ps.” Besides puzzling, it can stand for purple, plaquish, polygonal (ie, not round), prurtitic, papular, penile (LP has a predilection for this area), and planar (ie, flat-topped).
But it’s the “puzzling” aspect that unlocks the entrance to “The Ps,” and that in turn leads to consideration of this diagnosis. In this case, LP was not totally obvious, but a biopsy confirmed my clinical suspicions by demonstrating the classic findings associated with it.
An LP-like eruption can be provoked by drugs, such as the antimalarials, gold compounds, and penicillamine. It has also been associated with hepatitis C. But in my experience, the most common trigger appears to be stress, since it’s a rare case that doesn’t involve some. (I’ll happily concede that this is my opinion and not established fact.)
This patient’s LP demonstrated two common variants: For one, the darker the patient’s skin, the deeper the purple color of the LP—sometimes to the point of obscuring the diagnosis.
As if that were not enough to confuse the examiner, there was an element of lichen simplex chronicus (LSC; also known as neurodermatitis), which instigates the “itch-scratch-itch” cycle. By its nature, LSC tends to hyperpigmentation (brown). By definition, it involves hypertrophy of the epidermis secondary to scratching. But there is always a primary cause; sometimes mere dry skin will trigger it, or insect bites, eczema, or even psoriasis. This confuses the issue, but the biopsy will establish the true basis for the patient’s condition.
The more common presentation of LP is that of a collection of flat-topped (“planar”), pruritic, purplish papules on the volar wrist, ankles, and sacral area. The surfaces of these lesions often demonstrate a faintly white, shiny surface. LP can also appear on oral mucosal surfaces, where it has a totally different, lacy-white look. In these areas, it tends to burn and not itch. LP appears to have a predilection for the penile glans, where it presents as an annular purplish plaque that sits astride the proximal glans, spilling over onto the coronal area.
LP can present as a widespread generalized eruption mimicking granuloma annulare and secondary syphilis. The differential for simple, classic LP includes psoriasis, granuloma annulare and pityriasis rosea.
Treatment of LP is with topical steroid creams or ointments, tailored in terms of strength to the area of involvement. For example, a class II or III corticosteroid would be used on areas with thicker skin and a class IV or V on thinner skin. Stubborn, solitary plaques can be treated with intralesional steroid injection (2.5 to 10 mg/cc triamcinolone solution).
TAKE-HOME LEARNING POINTS
• Lichen planus (LP) is common and typically presents on the volar wrists, dorsal ankles, and sacrum.
• In a minority of cases, LP can present on the legs as a hypertrophic, plaquish condition with secondary features of lichen simplex chronicus.
• A mnemonic device (“The Ps”) describes LP: papular, pruritic, purple, planar, polygonal, plaquish, and (perhaps most importantly) “puzzling.”
• LP can present as an annular plaque on the penile glans.
• LP has a pathognomic histologic picture on biopsy.
A 43-year-old Hispanic man has a very itchy rash on his left leg. It manifested within a few weeks of the patient starting a new job, one that involved working more hours than he had previously and on rotating shifts, which interfered with his sleep schedule.
For several years, he tried using different products—mostly OTC creams—on it, with no success. Increasingly annoyed by the rash, he finally consulted his family physician, who prescribed a combination clotrimazole/betamethasone cream. This helped a bit, but over time, the rash steadily worsened, and his primary care provider referred him to dermatology.
The patient claims to be otherwise healthy, with no history of atopy. He takes no prescription medications. He admits finding it difficult to refrain from scratching his legs.
EXAMINATION
There are large, slightly purplish, hypertrophic papulosquamous plaques of up to 8 cm on both anterior tibial areas. His arms, trunk, and wrists are rash-free, and his oral mucosal surfaces show no obvious signs of disease.
Punch biopsy shows a hypertrophic epidermis. There is marked obliteration of the dermoepidermal junction by a dense bandlike layer of lymphocytic infiltrate, associated with vacuolar change and necrotic keratinocytes in the basal layer of the epidermis.
DISCUSSION
This is a classic clinical and histologic picture of a dermatosis commonly seen in dermatology offices: lichen planus (LP). As is often the case, by the time the patient gets to dermatology, the clinical picture can be confused and atypical. But there is much to learn from such a case.
LP is the prototypical interface lichenoid dermatitis, which can be depended on to present in certain identifiable ways. In this case, what suggested this diagnosis was the word “puzzling.” It’s the perfect example of the intuitive, somewhat subjective side of dermatologic diagnosis: When I see a condition that baffles me momentarily, my puzzled reaction becomes meaningful because the word puzzling starts with a “p.” Lichen planus has long been associated with an extremely useful mnemonic device known as “The Ps.” Besides puzzling, it can stand for purple, plaquish, polygonal (ie, not round), prurtitic, papular, penile (LP has a predilection for this area), and planar (ie, flat-topped).
But it’s the “puzzling” aspect that unlocks the entrance to “The Ps,” and that in turn leads to consideration of this diagnosis. In this case, LP was not totally obvious, but a biopsy confirmed my clinical suspicions by demonstrating the classic findings associated with it.
An LP-like eruption can be provoked by drugs, such as the antimalarials, gold compounds, and penicillamine. It has also been associated with hepatitis C. But in my experience, the most common trigger appears to be stress, since it’s a rare case that doesn’t involve some. (I’ll happily concede that this is my opinion and not established fact.)
This patient’s LP demonstrated two common variants: For one, the darker the patient’s skin, the deeper the purple color of the LP—sometimes to the point of obscuring the diagnosis.
As if that were not enough to confuse the examiner, there was an element of lichen simplex chronicus (LSC; also known as neurodermatitis), which instigates the “itch-scratch-itch” cycle. By its nature, LSC tends to hyperpigmentation (brown). By definition, it involves hypertrophy of the epidermis secondary to scratching. But there is always a primary cause; sometimes mere dry skin will trigger it, or insect bites, eczema, or even psoriasis. This confuses the issue, but the biopsy will establish the true basis for the patient’s condition.
The more common presentation of LP is that of a collection of flat-topped (“planar”), pruritic, purplish papules on the volar wrist, ankles, and sacral area. The surfaces of these lesions often demonstrate a faintly white, shiny surface. LP can also appear on oral mucosal surfaces, where it has a totally different, lacy-white look. In these areas, it tends to burn and not itch. LP appears to have a predilection for the penile glans, where it presents as an annular purplish plaque that sits astride the proximal glans, spilling over onto the coronal area.
LP can present as a widespread generalized eruption mimicking granuloma annulare and secondary syphilis. The differential for simple, classic LP includes psoriasis, granuloma annulare and pityriasis rosea.
Treatment of LP is with topical steroid creams or ointments, tailored in terms of strength to the area of involvement. For example, a class II or III corticosteroid would be used on areas with thicker skin and a class IV or V on thinner skin. Stubborn, solitary plaques can be treated with intralesional steroid injection (2.5 to 10 mg/cc triamcinolone solution).
TAKE-HOME LEARNING POINTS
• Lichen planus (LP) is common and typically presents on the volar wrists, dorsal ankles, and sacrum.
• In a minority of cases, LP can present on the legs as a hypertrophic, plaquish condition with secondary features of lichen simplex chronicus.
• A mnemonic device (“The Ps”) describes LP: papular, pruritic, purple, planar, polygonal, plaquish, and (perhaps most importantly) “puzzling.”
• LP can present as an annular plaque on the penile glans.
• LP has a pathognomic histologic picture on biopsy.
A 43-year-old Hispanic man has a very itchy rash on his left leg. It manifested within a few weeks of the patient starting a new job, one that involved working more hours than he had previously and on rotating shifts, which interfered with his sleep schedule.
For several years, he tried using different products—mostly OTC creams—on it, with no success. Increasingly annoyed by the rash, he finally consulted his family physician, who prescribed a combination clotrimazole/betamethasone cream. This helped a bit, but over time, the rash steadily worsened, and his primary care provider referred him to dermatology.
The patient claims to be otherwise healthy, with no history of atopy. He takes no prescription medications. He admits finding it difficult to refrain from scratching his legs.
EXAMINATION
There are large, slightly purplish, hypertrophic papulosquamous plaques of up to 8 cm on both anterior tibial areas. His arms, trunk, and wrists are rash-free, and his oral mucosal surfaces show no obvious signs of disease.
Punch biopsy shows a hypertrophic epidermis. There is marked obliteration of the dermoepidermal junction by a dense bandlike layer of lymphocytic infiltrate, associated with vacuolar change and necrotic keratinocytes in the basal layer of the epidermis.
DISCUSSION
This is a classic clinical and histologic picture of a dermatosis commonly seen in dermatology offices: lichen planus (LP). As is often the case, by the time the patient gets to dermatology, the clinical picture can be confused and atypical. But there is much to learn from such a case.
LP is the prototypical interface lichenoid dermatitis, which can be depended on to present in certain identifiable ways. In this case, what suggested this diagnosis was the word “puzzling.” It’s the perfect example of the intuitive, somewhat subjective side of dermatologic diagnosis: When I see a condition that baffles me momentarily, my puzzled reaction becomes meaningful because the word puzzling starts with a “p.” Lichen planus has long been associated with an extremely useful mnemonic device known as “The Ps.” Besides puzzling, it can stand for purple, plaquish, polygonal (ie, not round), prurtitic, papular, penile (LP has a predilection for this area), and planar (ie, flat-topped).
But it’s the “puzzling” aspect that unlocks the entrance to “The Ps,” and that in turn leads to consideration of this diagnosis. In this case, LP was not totally obvious, but a biopsy confirmed my clinical suspicions by demonstrating the classic findings associated with it.
An LP-like eruption can be provoked by drugs, such as the antimalarials, gold compounds, and penicillamine. It has also been associated with hepatitis C. But in my experience, the most common trigger appears to be stress, since it’s a rare case that doesn’t involve some. (I’ll happily concede that this is my opinion and not established fact.)
This patient’s LP demonstrated two common variants: For one, the darker the patient’s skin, the deeper the purple color of the LP—sometimes to the point of obscuring the diagnosis.
As if that were not enough to confuse the examiner, there was an element of lichen simplex chronicus (LSC; also known as neurodermatitis), which instigates the “itch-scratch-itch” cycle. By its nature, LSC tends to hyperpigmentation (brown). By definition, it involves hypertrophy of the epidermis secondary to scratching. But there is always a primary cause; sometimes mere dry skin will trigger it, or insect bites, eczema, or even psoriasis. This confuses the issue, but the biopsy will establish the true basis for the patient’s condition.
The more common presentation of LP is that of a collection of flat-topped (“planar”), pruritic, purplish papules on the volar wrist, ankles, and sacral area. The surfaces of these lesions often demonstrate a faintly white, shiny surface. LP can also appear on oral mucosal surfaces, where it has a totally different, lacy-white look. In these areas, it tends to burn and not itch. LP appears to have a predilection for the penile glans, where it presents as an annular purplish plaque that sits astride the proximal glans, spilling over onto the coronal area.
LP can present as a widespread generalized eruption mimicking granuloma annulare and secondary syphilis. The differential for simple, classic LP includes psoriasis, granuloma annulare and pityriasis rosea.
Treatment of LP is with topical steroid creams or ointments, tailored in terms of strength to the area of involvement. For example, a class II or III corticosteroid would be used on areas with thicker skin and a class IV or V on thinner skin. Stubborn, solitary plaques can be treated with intralesional steroid injection (2.5 to 10 mg/cc triamcinolone solution).
TAKE-HOME LEARNING POINTS
• Lichen planus (LP) is common and typically presents on the volar wrists, dorsal ankles, and sacrum.
• In a minority of cases, LP can present on the legs as a hypertrophic, plaquish condition with secondary features of lichen simplex chronicus.
• A mnemonic device (“The Ps”) describes LP: papular, pruritic, purple, planar, polygonal, plaquish, and (perhaps most importantly) “puzzling.”
• LP can present as an annular plaque on the penile glans.
• LP has a pathognomic histologic picture on biopsy.
Hair Loss at a Very Young Age
ANSWER
The correct answer is trichotillomania (choice “c”). See Discussion for more information.
Alopecia mucinosa (choice “a”) is a rare cause of focal hair loss that can occur in children. However, it usually presents with papules or plaques, unlike the smooth skin surface seen here.
Alopecia areata (choice “b”), common in children, typically entails complete hair loss in a given area—or, as hair regrows, with hairs of equal length. The uneven hairs seen in trichotillomania help a great deal in distinguishing it from alopecia areata.
Traction alopecia (choice “d”) is focal hair loss caused by chronic tension related to hairstyling. Most common in African-American women, and typically affecting the frontal periphery of the scalp, it is an unlikely explanation for hair loss in a 10-year-old boy.
DISCUSSION
Trichotillomania (TT) means, literally, “hair-pulling madness.” But in reality, there’s little actual plucking of hairs in this common condition. Instead, patients habitually manipulate hair by twirling and tugging, which weakens the shafts and follicles and renders them more susceptible to everyday wear and tear. In some cases, individual hairs speed through their growth phases and others break off in mid-shaft. All of this contributes to the classic “uneven” look of TT.
Patients with TT tend to be in the 4-to-17 age range, and most have issues with unresolved anxiety that manifest in part with manipulation of the hair. Officially considered an impulse control disorder, TT in most cases belongs to the psychiatrist’s domain.
In this case, it was enormously helpful to have corroboration from the patient and his mother regarding his role in creating and perpetuating the problem. Had that not been the case—or in the event of other doubts as to the correct diagnosis—biopsy could have been performed to rule out most of the other items in the differential, particularly alopecia areata.
Interestingly enough, studies have shown that the more sharply defined the area of hair loss, the more likely the patient is to admit his/her role in its creation. However, as is often the case with scientific research, contradictory findings have also been made.
TREATMENT
Treatment of TT is problematic, since no medications have proven to be completely helpful. Psychiatrists use a combination of medication, cognitive behavioral therapy, and other behavior modifications that are designed to overcome the habitual component of the problem. Most cases of TT resolve on their own, but in severe cases that persist for years, permanent hair loss can result.
In this case, there was enough insight and motivation on the part of the patient and his family to stop the offending behavior and allow the hair to regrow.
ANSWER
The correct answer is trichotillomania (choice “c”). See Discussion for more information.
Alopecia mucinosa (choice “a”) is a rare cause of focal hair loss that can occur in children. However, it usually presents with papules or plaques, unlike the smooth skin surface seen here.
Alopecia areata (choice “b”), common in children, typically entails complete hair loss in a given area—or, as hair regrows, with hairs of equal length. The uneven hairs seen in trichotillomania help a great deal in distinguishing it from alopecia areata.
Traction alopecia (choice “d”) is focal hair loss caused by chronic tension related to hairstyling. Most common in African-American women, and typically affecting the frontal periphery of the scalp, it is an unlikely explanation for hair loss in a 10-year-old boy.
DISCUSSION
Trichotillomania (TT) means, literally, “hair-pulling madness.” But in reality, there’s little actual plucking of hairs in this common condition. Instead, patients habitually manipulate hair by twirling and tugging, which weakens the shafts and follicles and renders them more susceptible to everyday wear and tear. In some cases, individual hairs speed through their growth phases and others break off in mid-shaft. All of this contributes to the classic “uneven” look of TT.
Patients with TT tend to be in the 4-to-17 age range, and most have issues with unresolved anxiety that manifest in part with manipulation of the hair. Officially considered an impulse control disorder, TT in most cases belongs to the psychiatrist’s domain.
In this case, it was enormously helpful to have corroboration from the patient and his mother regarding his role in creating and perpetuating the problem. Had that not been the case—or in the event of other doubts as to the correct diagnosis—biopsy could have been performed to rule out most of the other items in the differential, particularly alopecia areata.
Interestingly enough, studies have shown that the more sharply defined the area of hair loss, the more likely the patient is to admit his/her role in its creation. However, as is often the case with scientific research, contradictory findings have also been made.
TREATMENT
Treatment of TT is problematic, since no medications have proven to be completely helpful. Psychiatrists use a combination of medication, cognitive behavioral therapy, and other behavior modifications that are designed to overcome the habitual component of the problem. Most cases of TT resolve on their own, but in severe cases that persist for years, permanent hair loss can result.
In this case, there was enough insight and motivation on the part of the patient and his family to stop the offending behavior and allow the hair to regrow.
ANSWER
The correct answer is trichotillomania (choice “c”). See Discussion for more information.
Alopecia mucinosa (choice “a”) is a rare cause of focal hair loss that can occur in children. However, it usually presents with papules or plaques, unlike the smooth skin surface seen here.
Alopecia areata (choice “b”), common in children, typically entails complete hair loss in a given area—or, as hair regrows, with hairs of equal length. The uneven hairs seen in trichotillomania help a great deal in distinguishing it from alopecia areata.
Traction alopecia (choice “d”) is focal hair loss caused by chronic tension related to hairstyling. Most common in African-American women, and typically affecting the frontal periphery of the scalp, it is an unlikely explanation for hair loss in a 10-year-old boy.
DISCUSSION
Trichotillomania (TT) means, literally, “hair-pulling madness.” But in reality, there’s little actual plucking of hairs in this common condition. Instead, patients habitually manipulate hair by twirling and tugging, which weakens the shafts and follicles and renders them more susceptible to everyday wear and tear. In some cases, individual hairs speed through their growth phases and others break off in mid-shaft. All of this contributes to the classic “uneven” look of TT.
Patients with TT tend to be in the 4-to-17 age range, and most have issues with unresolved anxiety that manifest in part with manipulation of the hair. Officially considered an impulse control disorder, TT in most cases belongs to the psychiatrist’s domain.
In this case, it was enormously helpful to have corroboration from the patient and his mother regarding his role in creating and perpetuating the problem. Had that not been the case—or in the event of other doubts as to the correct diagnosis—biopsy could have been performed to rule out most of the other items in the differential, particularly alopecia areata.
Interestingly enough, studies have shown that the more sharply defined the area of hair loss, the more likely the patient is to admit his/her role in its creation. However, as is often the case with scientific research, contradictory findings have also been made.
TREATMENT
Treatment of TT is problematic, since no medications have proven to be completely helpful. Psychiatrists use a combination of medication, cognitive behavioral therapy, and other behavior modifications that are designed to overcome the habitual component of the problem. Most cases of TT resolve on their own, but in severe cases that persist for years, permanent hair loss can result.
In this case, there was enough insight and motivation on the part of the patient and his family to stop the offending behavior and allow the hair to regrow.
A 10-year-old boy is referred to dermatology with a four-month history of hair loss. The affected area of the vertex is now large enough to alarm his mother, who accompanies him to his appointment. The child’s primary care provider had diagnosed alopecia areata and prescribed triamcinolone 0.1% solution. But after a month of twice-daily application, even more hair has been lost. There is no family history of alopecia areata or other autoimmune disease. The child is otherwise healthy, although he is being treated by a psychiatrist for attention deficit disorder and chronic anxiety (with two medications whose names are unknown). The patient denies any symptoms associated with his hair loss, and his mother denies any skin changes in the affected area. However, she emphasizes that she has seen her son manipulating the area with his hand on several occasions, despite her attempts to make him stop. When pressed, the patient finally admits that throughout the day he twirls and tugs on his hair—although he denies actually pulling out any. On inspection, an 11 x 8–cm oval area of distinct and sharply demarcated hair loss is noted in the vertex scalp. Hairs of different lengths are noted in the central portion of the site; some have obviously been broken off, while others are longer, with thin, tapering ends. There is no disruption (eg, scaling, redness, edema) in the surface of the scalp, but the whole area is darker (brown) than the surrounding, uninvolved scalp. No other areas of hair loss are noted in the scalp or face. No nodes are palpable in the neck.
When There’s More to the Story …
Three or four weeks ago, this 56-year-old man noticed asymptomatic lesions on his hands and feet. He says he’s never had anything like them before. He denies taking any new medications and claims to “feel fine,” with no fever, joint pain, or malaise.
EXAMINATION
Numerous round macules and papules are seen on the patient’s palms and soles, many of which have scaly peripheral margins. Most are brownish-red, and they are especially dense on the periphery of the feet.
Looking elsewhere, similar lesions are noted on the penile corona and glans. There is a faint but definite morbiliform, blanchable pink rash covering most of the patient’s trunk, taking on a “shawl” distribution across the shoulders.
These findings prompt a more directed and thorough history, which reveals that the patient is exclusively homosexual and recently engaged in high-risk sexual activity. He denies being HIV-positive, though he admits he hasn’t been tested for several years.
At this point, the patient admits to consulting a urologist two weeks ago. However, he came away from that visit with an assurance that serious disease was “unlikely.”
Accordingly, laboratory tests, including a rapid plasma reagin (RPR), are obtained. The RPR results are positive (1:64). The case is reported to the county health department.
DISCUSSION
It’s been said (by me and others) that half the job of any clinician is staying awake, in advance of the inevitable appearance of serious disease. These diseases are out there but don’t always appear the way they do in the textbooks. “Monroe’s rule #4” says the more serious the skin disease, the less likely it is to be diagnosed in a timely fashion.
It would be hard to imagine a more classic example of secondary syphilis than was seen in this case, occurring in a patient so obviously at risk. But it’s only “obvious” if you’re ready and aware of how syphilis manifests. It also helps if you understand how common it is and who’s likely to get it.
This patient’s primary care provider didn’t recognize the condition and referred the patient to a provider whose specialty is surgical diseases of the urogenital system. I mean no disrespect to urologists (or to urology PAs/NPs), but there’s no particular reason they would know what this was. It’s analogous to the referral of patients to podiatry for a rash or other lesion on the foot. Being a surgical expert on the structural maladies of the foot and ankle in no way imparts expertise in skin diseases of the same areas.
The point is: Skin diseases belong with the experts in skin disease (ie, dermatology providers). They are uniquely qualified, not only in terms of recognizing what is being seen, but in being able to see those findings in the context of other physical and historical data. This case is the perfect example.
Here’s the thought process that occurred in this case: Rashes of the feet and palms are unusual, though far from unknown. But when the rash is composed of round, slightly scaly lesions concentrated on the peripheral feet and hands, the differential narrows significantly. Pointed questions regarding sexual history become necessary. Homosexuality, by itself, is not a risk factor, but engaging in high-risk behaviors performed with exclusively homosexual partners is. These facts, combined with the discovery of the widespread truncal rash, mandated specific blood tests; once those tests confirmed the suspected diagnosis, the law mandated reporting of the case to the health department.
Representatives of said entity will likely confirm the diagnosis with more specific testing, treat the patient (probably with penicillin injection), then take a detailed history of sexual exposure in order to stop the spread of the disease in the community. Physically, this patient will be fine. But, as one might imagine, more fallout can be expected in terms of accusations and denials.
Other items in the differential for such rashes include: lichen planus, psoriasis, and erythema multiforme. A biopsy would have been necessary had the tests for syphilis been negative.
TAKE-HOME LEARNING POINTS
• Palmar and plantar rashes are unusual and should prompt the examiner to expand the history and physical.
• Secondary syphilis, though uncommon, is far from rare, especially among gay men engaging in high-risk sexual behavior.
• It’s common for the patient to deny the appearance of the chancre of primary syphilis, and such a lesion would be long gone by the time those of secondary syphilis manifest.
• Conditions involving the skin should be seen by a dermatology provider, regardless of location. This includes diseases of the skin, hair, nails, oral mucosa, genitals, feet, or palms. One potential exception is the eye itself, though most diseases “of the eye” are, in reality, diseases of the periocular skin—and belong with a dermatology provider.
Three or four weeks ago, this 56-year-old man noticed asymptomatic lesions on his hands and feet. He says he’s never had anything like them before. He denies taking any new medications and claims to “feel fine,” with no fever, joint pain, or malaise.
EXAMINATION
Numerous round macules and papules are seen on the patient’s palms and soles, many of which have scaly peripheral margins. Most are brownish-red, and they are especially dense on the periphery of the feet.
Looking elsewhere, similar lesions are noted on the penile corona and glans. There is a faint but definite morbiliform, blanchable pink rash covering most of the patient’s trunk, taking on a “shawl” distribution across the shoulders.
These findings prompt a more directed and thorough history, which reveals that the patient is exclusively homosexual and recently engaged in high-risk sexual activity. He denies being HIV-positive, though he admits he hasn’t been tested for several years.
At this point, the patient admits to consulting a urologist two weeks ago. However, he came away from that visit with an assurance that serious disease was “unlikely.”
Accordingly, laboratory tests, including a rapid plasma reagin (RPR), are obtained. The RPR results are positive (1:64). The case is reported to the county health department.
DISCUSSION
It’s been said (by me and others) that half the job of any clinician is staying awake, in advance of the inevitable appearance of serious disease. These diseases are out there but don’t always appear the way they do in the textbooks. “Monroe’s rule #4” says the more serious the skin disease, the less likely it is to be diagnosed in a timely fashion.
It would be hard to imagine a more classic example of secondary syphilis than was seen in this case, occurring in a patient so obviously at risk. But it’s only “obvious” if you’re ready and aware of how syphilis manifests. It also helps if you understand how common it is and who’s likely to get it.
This patient’s primary care provider didn’t recognize the condition and referred the patient to a provider whose specialty is surgical diseases of the urogenital system. I mean no disrespect to urologists (or to urology PAs/NPs), but there’s no particular reason they would know what this was. It’s analogous to the referral of patients to podiatry for a rash or other lesion on the foot. Being a surgical expert on the structural maladies of the foot and ankle in no way imparts expertise in skin diseases of the same areas.
The point is: Skin diseases belong with the experts in skin disease (ie, dermatology providers). They are uniquely qualified, not only in terms of recognizing what is being seen, but in being able to see those findings in the context of other physical and historical data. This case is the perfect example.
Here’s the thought process that occurred in this case: Rashes of the feet and palms are unusual, though far from unknown. But when the rash is composed of round, slightly scaly lesions concentrated on the peripheral feet and hands, the differential narrows significantly. Pointed questions regarding sexual history become necessary. Homosexuality, by itself, is not a risk factor, but engaging in high-risk behaviors performed with exclusively homosexual partners is. These facts, combined with the discovery of the widespread truncal rash, mandated specific blood tests; once those tests confirmed the suspected diagnosis, the law mandated reporting of the case to the health department.
Representatives of said entity will likely confirm the diagnosis with more specific testing, treat the patient (probably with penicillin injection), then take a detailed history of sexual exposure in order to stop the spread of the disease in the community. Physically, this patient will be fine. But, as one might imagine, more fallout can be expected in terms of accusations and denials.
Other items in the differential for such rashes include: lichen planus, psoriasis, and erythema multiforme. A biopsy would have been necessary had the tests for syphilis been negative.
TAKE-HOME LEARNING POINTS
• Palmar and plantar rashes are unusual and should prompt the examiner to expand the history and physical.
• Secondary syphilis, though uncommon, is far from rare, especially among gay men engaging in high-risk sexual behavior.
• It’s common for the patient to deny the appearance of the chancre of primary syphilis, and such a lesion would be long gone by the time those of secondary syphilis manifest.
• Conditions involving the skin should be seen by a dermatology provider, regardless of location. This includes diseases of the skin, hair, nails, oral mucosa, genitals, feet, or palms. One potential exception is the eye itself, though most diseases “of the eye” are, in reality, diseases of the periocular skin—and belong with a dermatology provider.
Three or four weeks ago, this 56-year-old man noticed asymptomatic lesions on his hands and feet. He says he’s never had anything like them before. He denies taking any new medications and claims to “feel fine,” with no fever, joint pain, or malaise.
EXAMINATION
Numerous round macules and papules are seen on the patient’s palms and soles, many of which have scaly peripheral margins. Most are brownish-red, and they are especially dense on the periphery of the feet.
Looking elsewhere, similar lesions are noted on the penile corona and glans. There is a faint but definite morbiliform, blanchable pink rash covering most of the patient’s trunk, taking on a “shawl” distribution across the shoulders.
These findings prompt a more directed and thorough history, which reveals that the patient is exclusively homosexual and recently engaged in high-risk sexual activity. He denies being HIV-positive, though he admits he hasn’t been tested for several years.
At this point, the patient admits to consulting a urologist two weeks ago. However, he came away from that visit with an assurance that serious disease was “unlikely.”
Accordingly, laboratory tests, including a rapid plasma reagin (RPR), are obtained. The RPR results are positive (1:64). The case is reported to the county health department.
DISCUSSION
It’s been said (by me and others) that half the job of any clinician is staying awake, in advance of the inevitable appearance of serious disease. These diseases are out there but don’t always appear the way they do in the textbooks. “Monroe’s rule #4” says the more serious the skin disease, the less likely it is to be diagnosed in a timely fashion.
It would be hard to imagine a more classic example of secondary syphilis than was seen in this case, occurring in a patient so obviously at risk. But it’s only “obvious” if you’re ready and aware of how syphilis manifests. It also helps if you understand how common it is and who’s likely to get it.
This patient’s primary care provider didn’t recognize the condition and referred the patient to a provider whose specialty is surgical diseases of the urogenital system. I mean no disrespect to urologists (or to urology PAs/NPs), but there’s no particular reason they would know what this was. It’s analogous to the referral of patients to podiatry for a rash or other lesion on the foot. Being a surgical expert on the structural maladies of the foot and ankle in no way imparts expertise in skin diseases of the same areas.
The point is: Skin diseases belong with the experts in skin disease (ie, dermatology providers). They are uniquely qualified, not only in terms of recognizing what is being seen, but in being able to see those findings in the context of other physical and historical data. This case is the perfect example.
Here’s the thought process that occurred in this case: Rashes of the feet and palms are unusual, though far from unknown. But when the rash is composed of round, slightly scaly lesions concentrated on the peripheral feet and hands, the differential narrows significantly. Pointed questions regarding sexual history become necessary. Homosexuality, by itself, is not a risk factor, but engaging in high-risk behaviors performed with exclusively homosexual partners is. These facts, combined with the discovery of the widespread truncal rash, mandated specific blood tests; once those tests confirmed the suspected diagnosis, the law mandated reporting of the case to the health department.
Representatives of said entity will likely confirm the diagnosis with more specific testing, treat the patient (probably with penicillin injection), then take a detailed history of sexual exposure in order to stop the spread of the disease in the community. Physically, this patient will be fine. But, as one might imagine, more fallout can be expected in terms of accusations and denials.
Other items in the differential for such rashes include: lichen planus, psoriasis, and erythema multiforme. A biopsy would have been necessary had the tests for syphilis been negative.
TAKE-HOME LEARNING POINTS
• Palmar and plantar rashes are unusual and should prompt the examiner to expand the history and physical.
• Secondary syphilis, though uncommon, is far from rare, especially among gay men engaging in high-risk sexual behavior.
• It’s common for the patient to deny the appearance of the chancre of primary syphilis, and such a lesion would be long gone by the time those of secondary syphilis manifest.
• Conditions involving the skin should be seen by a dermatology provider, regardless of location. This includes diseases of the skin, hair, nails, oral mucosa, genitals, feet, or palms. One potential exception is the eye itself, though most diseases “of the eye” are, in reality, diseases of the periocular skin—and belong with a dermatology provider.
Painless Lesion Interferes With Man’s Vision
For years, this 80-year-old man has had a lesion on his right upper eyelid. It has slowly grown, though it causes no pain or other symptoms, and is now interfering with his lateral vision. This is what prompts him to seek evaluation.
His primary care providers over the years have seen the lesion. All have assured him of its benignancy.
In the distant past, the patient had a great deal of overexposure to the sun. Several skin cancers have been removed from his face.
At this time, he is residing in a rehab center, where he is recovering from a stroke. With his daughter’s assistance, the patient, who is not ambulatory and is a bit confused, is able to understand what is happening.
EXAMINATION
The lesion is a well-defined, 5 mm x 1.1–cm pearly plaque located on the lateral portion of his left upper eyelid, within 2 to 3 mm of the lateral palpebral margin. It is seen in the context of heavily sun-damaged type II facial skin.
A shave biopsy indicates the lesion is a basal cell carcinoma (BCC).
DISCUSSION
BCCs, though rarely fatal, can be associated with a great deal of morbidity. This case highlights several issues regarding the diagnosis and treatment of nonmelanoma skin cancers (eg, basal or squamous cell carcinoma)—not the least of which is the delayed diagnosis. In this instance, the delay is unlikely to harm the patient; however, that is not always the case. If this BCC had been a bit more aggressive, it could have invaded the periocular structures, which might have necessitated extensive surgery and possibly postoperative radiation.
Fortunately, this particular BCC was exceptionally slow to grow, to the extent that doing nothing was a serious consideration. If the patient had been older and/or less capable of cooperating with the surgical process, taking no action might have been the best choice.
But his BCC had grown, and he was able to state his preference (as did the family) to have it surgically removed. As of this writing, he has been scheduled for an appointment with a Mohs surgeon, who is likely to remove the lesion with margins. If the sample tests negative for residual cancer, the patient may not require further treatment. (Given the lesion’s location, the surgical wound does not even require closure, since they usually heal nicely by secondary intention.)
If microscopic examination reveals that the cancer extends into the eyelid itself, the patient will probably be referred to an oculoplastic surgeon for definitive excision and repair, which can be complex and difficult.
While the initial biopsy identified the lesion as a BCC, other items in the differential include seborrheic keratosis and even sebaceous carcinoma (an unusual diagnosis, but one common in patients this age). Squamous cell carcinoma and wart were also possibilities.
TAKE-HOME LEARNING POINTS
• Changing lesions require investigation, usually in the form of a simple shave biopsy.
• Patients with a history of skin cancer tend to develop additional skin cancers.
• Not all basal cell carcinomas are aggressive. Some are remarkably slow-growing.
• For nonaggressive basal cell carcinomas in patients who are unable to cooperate with treatment decisions, consider doing nothing.
• The eyelid is a favorite location for an unusual type of skin cancer: sebaceous carcinoma.
For years, this 80-year-old man has had a lesion on his right upper eyelid. It has slowly grown, though it causes no pain or other symptoms, and is now interfering with his lateral vision. This is what prompts him to seek evaluation.
His primary care providers over the years have seen the lesion. All have assured him of its benignancy.
In the distant past, the patient had a great deal of overexposure to the sun. Several skin cancers have been removed from his face.
At this time, he is residing in a rehab center, where he is recovering from a stroke. With his daughter’s assistance, the patient, who is not ambulatory and is a bit confused, is able to understand what is happening.
EXAMINATION
The lesion is a well-defined, 5 mm x 1.1–cm pearly plaque located on the lateral portion of his left upper eyelid, within 2 to 3 mm of the lateral palpebral margin. It is seen in the context of heavily sun-damaged type II facial skin.
A shave biopsy indicates the lesion is a basal cell carcinoma (BCC).
DISCUSSION
BCCs, though rarely fatal, can be associated with a great deal of morbidity. This case highlights several issues regarding the diagnosis and treatment of nonmelanoma skin cancers (eg, basal or squamous cell carcinoma)—not the least of which is the delayed diagnosis. In this instance, the delay is unlikely to harm the patient; however, that is not always the case. If this BCC had been a bit more aggressive, it could have invaded the periocular structures, which might have necessitated extensive surgery and possibly postoperative radiation.
Fortunately, this particular BCC was exceptionally slow to grow, to the extent that doing nothing was a serious consideration. If the patient had been older and/or less capable of cooperating with the surgical process, taking no action might have been the best choice.
But his BCC had grown, and he was able to state his preference (as did the family) to have it surgically removed. As of this writing, he has been scheduled for an appointment with a Mohs surgeon, who is likely to remove the lesion with margins. If the sample tests negative for residual cancer, the patient may not require further treatment. (Given the lesion’s location, the surgical wound does not even require closure, since they usually heal nicely by secondary intention.)
If microscopic examination reveals that the cancer extends into the eyelid itself, the patient will probably be referred to an oculoplastic surgeon for definitive excision and repair, which can be complex and difficult.
While the initial biopsy identified the lesion as a BCC, other items in the differential include seborrheic keratosis and even sebaceous carcinoma (an unusual diagnosis, but one common in patients this age). Squamous cell carcinoma and wart were also possibilities.
TAKE-HOME LEARNING POINTS
• Changing lesions require investigation, usually in the form of a simple shave biopsy.
• Patients with a history of skin cancer tend to develop additional skin cancers.
• Not all basal cell carcinomas are aggressive. Some are remarkably slow-growing.
• For nonaggressive basal cell carcinomas in patients who are unable to cooperate with treatment decisions, consider doing nothing.
• The eyelid is a favorite location for an unusual type of skin cancer: sebaceous carcinoma.
For years, this 80-year-old man has had a lesion on his right upper eyelid. It has slowly grown, though it causes no pain or other symptoms, and is now interfering with his lateral vision. This is what prompts him to seek evaluation.
His primary care providers over the years have seen the lesion. All have assured him of its benignancy.
In the distant past, the patient had a great deal of overexposure to the sun. Several skin cancers have been removed from his face.
At this time, he is residing in a rehab center, where he is recovering from a stroke. With his daughter’s assistance, the patient, who is not ambulatory and is a bit confused, is able to understand what is happening.
EXAMINATION
The lesion is a well-defined, 5 mm x 1.1–cm pearly plaque located on the lateral portion of his left upper eyelid, within 2 to 3 mm of the lateral palpebral margin. It is seen in the context of heavily sun-damaged type II facial skin.
A shave biopsy indicates the lesion is a basal cell carcinoma (BCC).
DISCUSSION
BCCs, though rarely fatal, can be associated with a great deal of morbidity. This case highlights several issues regarding the diagnosis and treatment of nonmelanoma skin cancers (eg, basal or squamous cell carcinoma)—not the least of which is the delayed diagnosis. In this instance, the delay is unlikely to harm the patient; however, that is not always the case. If this BCC had been a bit more aggressive, it could have invaded the periocular structures, which might have necessitated extensive surgery and possibly postoperative radiation.
Fortunately, this particular BCC was exceptionally slow to grow, to the extent that doing nothing was a serious consideration. If the patient had been older and/or less capable of cooperating with the surgical process, taking no action might have been the best choice.
But his BCC had grown, and he was able to state his preference (as did the family) to have it surgically removed. As of this writing, he has been scheduled for an appointment with a Mohs surgeon, who is likely to remove the lesion with margins. If the sample tests negative for residual cancer, the patient may not require further treatment. (Given the lesion’s location, the surgical wound does not even require closure, since they usually heal nicely by secondary intention.)
If microscopic examination reveals that the cancer extends into the eyelid itself, the patient will probably be referred to an oculoplastic surgeon for definitive excision and repair, which can be complex and difficult.
While the initial biopsy identified the lesion as a BCC, other items in the differential include seborrheic keratosis and even sebaceous carcinoma (an unusual diagnosis, but one common in patients this age). Squamous cell carcinoma and wart were also possibilities.
TAKE-HOME LEARNING POINTS
• Changing lesions require investigation, usually in the form of a simple shave biopsy.
• Patients with a history of skin cancer tend to develop additional skin cancers.
• Not all basal cell carcinomas are aggressive. Some are remarkably slow-growing.
• For nonaggressive basal cell carcinomas in patients who are unable to cooperate with treatment decisions, consider doing nothing.
• The eyelid is a favorite location for an unusual type of skin cancer: sebaceous carcinoma.
Growing Lesion Impedes Finger Flexion
ANSWER
The correct answer is implantation cyst (choice “b”). This type of cyst is typically caused by trauma (eg, a puncture wound), and its contents are in stark contrast to those of ganglion cysts (choice “a”), which are thick and clear.
Warts (choice “c”) are essentially an epidermal process, not subcutaneous. They almost always disrupt normal skin lines, which often curve around the wart—a finding that was missing in this case.
Acquired digital fibrokeratomas (choice “d”) are benign solid tumors frequently seen on fingers. However, they are more epidermal than intradermal and demonstrate a diagnostic feature called an epidermal collarette (missing in this case).
DISCUSSION
Sometimes called implantation dermoid cysts, these sacs have a well-defined white cyst wall and cheesy, often odoriferous contents. Although common on hands and fingers, they can occur in almost any location and as a result of many types of trauma.
This includes surgical trauma, which effectively implants surface adnexal tissue (eg, the sebaceous apparatus) where it can continue to produce and accumulate its cheesy contents over time. Patients often forget the trauma that caused the cyst, but it is still worth inquiring into.
Merely emptying the sac can confirm the diagnosis; however, this almost always results in recurrence. Fortunately, implantation cysts are usually easily removed with minimal risk to hand function.
As with almost any tissue removed from the body, the specimen needs to be sent for pathologic examination. In addition to the differential items already noted, a number of rare or unusual conditions can present in a similar fashion, including eccrine carcinoma and a variety of sarcomas.
This patient recovered from his surgery without complication. Pathologic examination confirmed the benign nature of the lesion.
ANSWER
The correct answer is implantation cyst (choice “b”). This type of cyst is typically caused by trauma (eg, a puncture wound), and its contents are in stark contrast to those of ganglion cysts (choice “a”), which are thick and clear.
Warts (choice “c”) are essentially an epidermal process, not subcutaneous. They almost always disrupt normal skin lines, which often curve around the wart—a finding that was missing in this case.
Acquired digital fibrokeratomas (choice “d”) are benign solid tumors frequently seen on fingers. However, they are more epidermal than intradermal and demonstrate a diagnostic feature called an epidermal collarette (missing in this case).
DISCUSSION
Sometimes called implantation dermoid cysts, these sacs have a well-defined white cyst wall and cheesy, often odoriferous contents. Although common on hands and fingers, they can occur in almost any location and as a result of many types of trauma.
This includes surgical trauma, which effectively implants surface adnexal tissue (eg, the sebaceous apparatus) where it can continue to produce and accumulate its cheesy contents over time. Patients often forget the trauma that caused the cyst, but it is still worth inquiring into.
Merely emptying the sac can confirm the diagnosis; however, this almost always results in recurrence. Fortunately, implantation cysts are usually easily removed with minimal risk to hand function.
As with almost any tissue removed from the body, the specimen needs to be sent for pathologic examination. In addition to the differential items already noted, a number of rare or unusual conditions can present in a similar fashion, including eccrine carcinoma and a variety of sarcomas.
This patient recovered from his surgery without complication. Pathologic examination confirmed the benign nature of the lesion.
ANSWER
The correct answer is implantation cyst (choice “b”). This type of cyst is typically caused by trauma (eg, a puncture wound), and its contents are in stark contrast to those of ganglion cysts (choice “a”), which are thick and clear.
Warts (choice “c”) are essentially an epidermal process, not subcutaneous. They almost always disrupt normal skin lines, which often curve around the wart—a finding that was missing in this case.
Acquired digital fibrokeratomas (choice “d”) are benign solid tumors frequently seen on fingers. However, they are more epidermal than intradermal and demonstrate a diagnostic feature called an epidermal collarette (missing in this case).
DISCUSSION
Sometimes called implantation dermoid cysts, these sacs have a well-defined white cyst wall and cheesy, often odoriferous contents. Although common on hands and fingers, they can occur in almost any location and as a result of many types of trauma.
This includes surgical trauma, which effectively implants surface adnexal tissue (eg, the sebaceous apparatus) where it can continue to produce and accumulate its cheesy contents over time. Patients often forget the trauma that caused the cyst, but it is still worth inquiring into.
Merely emptying the sac can confirm the diagnosis; however, this almost always results in recurrence. Fortunately, implantation cysts are usually easily removed with minimal risk to hand function.
As with almost any tissue removed from the body, the specimen needs to be sent for pathologic examination. In addition to the differential items already noted, a number of rare or unusual conditions can present in a similar fashion, including eccrine carcinoma and a variety of sarcomas.
This patient recovered from his surgery without complication. Pathologic examination confirmed the benign nature of the lesion.
For several years, a 45-year-old man has had an asymptomatic lesion on the volar aspect of his fourth finger. The lesion is growing and increasingly “in the way.” That, coupled with the patient’s concern about cancer or other serious disease, leads him to request referral to dermatology. There is no history of similar lesions anywhere on his body. Additional questioning reveals that several months prior to the lesion’s manifestation, the patient sustained a puncture wound to the same finger. Initially, the affected area was only a millimeter or two in size. X-rays ordered by his primary care provider did not indicate any bony abnormality, nor did they shed any light on the lesion itself. An impressive 2.6 cm in diameter, the lesion is prominent in vertical elevation as well. Motor and sensory function are found to be intact, although the bulk of the lesion prohibits full flexion of the finger. The lesion is opaque to attempted transillumination. No surface changes are apparent in the overlying skin. Skin lines are intact and parallel. The lesion is quite firm but compressible. The decision is made to excise the lesion, employing a digital block and tourniquet. A football-shaped ellipse of skin with 50° angles on the ends is removed from the surface, revealing a glistening white, smooth mass that comes out intact, with very little blunt dissection. Motor function is again assessed and found to be intact. The large angles in the ends of the ellipse allow the wound edges to be pulled together with interrupted vertical mattress sutures and no leftover redundant skin. The lesion is submitted intact for pathologic examination.
Cryotherapy: A Beginner’s Guide
Without a doubt, the most useful treatment modality I’ve acquired in my career is the proper use of liquid nitrogen. Cryotherapy is the standard treatment for small epidermal lesions, because it allows for removal without the need for anesthesia, with no broken skin or bleeding, and with a tolerable amount of pain.
Over the years, I’ve learned—often the hard way!—how to work with liquid nitrogen. For example, for the past 25 years, I’ve used a cryogun (or “unit,” as it’s sometimes called), having discovered that applying LN2 with anything else (eg, a cotton-tipped applicator) is a huge exercise in futility.
As with any tool in medicine, there are uses and misuses of cryotherapy. It is not intuitive—but it does yield to common sense. So allow me to give you the benefit of my experience.
APPROPRIATE AND INAPPROPRIATE TARGETS
In general, only epidermal lesions of obvious origin are treated with liquid nitrogen. This includes ordinary skin tags, small warts, and seborrheic and actinic keratosis. Utterly common and easy to treat, these lesions have no potential for malignant transformation and have relatively poor vasculature, which makes them ideal candidates for controlled, localized frostbite. This process (which can take as long as two weeks) kills the cells, disrupts the blood supply, and causes the lesions to die and fall away.
Other uses for cryotherapy include softening keloids or hypertrophic scars sufficiently to facilitate intralesional injection; treating condyloma, molluscum contagiosum, and small chondromdermatitis nodules; and even to destroy skin cancers (basal cell and squamous cell carcinomas)—the latter, however, only by clinicians with specialized training.
Inappropriate targets for cryotherapy include “moles” (nevi), vascular lesions such as hemangiomas, “birthmarks,” or any lesion of unknown nature. There are two reasons not to use liquid nitrogen in these cases: First, relatively well–vascularized lesions will be superficially blistered but will survive the treatment. Second, these lesions have at least a theoretical chance of having undergone malignant transformation. (As such, these lesions may need to be sampled to determine whether they are malignant; but that is a discussion for another column.)
THE NEED FOR CAUTION
For all its positive features, there are drawbacks to using cryotherapy. Here are six to consider:
• Blistering, which can be severe in sensitive patients
• Dyschromia (color changes in treated skin), especially in darker-skinned patients
• Pain, particularly in children
• Loss of function (eg, nerve or cartilage damage)
• Scarring, usually from overtreatment
• Disability—walking (let alone running or working) may be painful for a day or two after treatment, especially following brisk cryotherapy of a larger plantar wart
TREATING WARTS WITH CRYOTHERAPY
First, you must confirm the diagnosis: “Seeds” (black dots on the surface, which really represent vertically aligned thrombosed capillaries) are pathognomic. Warts lack surface skin lines (dermatoglyphics) and do not umbilicate on paring (unlike clavi or corns).
Always discuss indications, procedure, alternatives, and risks prior to performing cryotherapy. Patients (and parents) need to understand that the “perfect” treatment for warts has yet to be devised. All currently available methods have shortcomings.
Consider using an ear speculum (3 to 5 mm) to concentrate the spray of liquid nitrogen, reducing pain and shortening the length of treatment. Particularly with thicker plantar warts, you might want to pare away surface keratotic material first, then use the “freeze-thaw-freeze” technique. The average length of a single treatment seldom exceeds five seconds, particularly if a speculum or other dam is used.
Arrange for follow-up, typically one month later, since it’s a rare wart that clears with a single treatment.
Without a doubt, the most useful treatment modality I’ve acquired in my career is the proper use of liquid nitrogen. Cryotherapy is the standard treatment for small epidermal lesions, because it allows for removal without the need for anesthesia, with no broken skin or bleeding, and with a tolerable amount of pain.
Over the years, I’ve learned—often the hard way!—how to work with liquid nitrogen. For example, for the past 25 years, I’ve used a cryogun (or “unit,” as it’s sometimes called), having discovered that applying LN2 with anything else (eg, a cotton-tipped applicator) is a huge exercise in futility.
As with any tool in medicine, there are uses and misuses of cryotherapy. It is not intuitive—but it does yield to common sense. So allow me to give you the benefit of my experience.
APPROPRIATE AND INAPPROPRIATE TARGETS
In general, only epidermal lesions of obvious origin are treated with liquid nitrogen. This includes ordinary skin tags, small warts, and seborrheic and actinic keratosis. Utterly common and easy to treat, these lesions have no potential for malignant transformation and have relatively poor vasculature, which makes them ideal candidates for controlled, localized frostbite. This process (which can take as long as two weeks) kills the cells, disrupts the blood supply, and causes the lesions to die and fall away.
Other uses for cryotherapy include softening keloids or hypertrophic scars sufficiently to facilitate intralesional injection; treating condyloma, molluscum contagiosum, and small chondromdermatitis nodules; and even to destroy skin cancers (basal cell and squamous cell carcinomas)—the latter, however, only by clinicians with specialized training.
Inappropriate targets for cryotherapy include “moles” (nevi), vascular lesions such as hemangiomas, “birthmarks,” or any lesion of unknown nature. There are two reasons not to use liquid nitrogen in these cases: First, relatively well–vascularized lesions will be superficially blistered but will survive the treatment. Second, these lesions have at least a theoretical chance of having undergone malignant transformation. (As such, these lesions may need to be sampled to determine whether they are malignant; but that is a discussion for another column.)
THE NEED FOR CAUTION
For all its positive features, there are drawbacks to using cryotherapy. Here are six to consider:
• Blistering, which can be severe in sensitive patients
• Dyschromia (color changes in treated skin), especially in darker-skinned patients
• Pain, particularly in children
• Loss of function (eg, nerve or cartilage damage)
• Scarring, usually from overtreatment
• Disability—walking (let alone running or working) may be painful for a day or two after treatment, especially following brisk cryotherapy of a larger plantar wart
TREATING WARTS WITH CRYOTHERAPY
First, you must confirm the diagnosis: “Seeds” (black dots on the surface, which really represent vertically aligned thrombosed capillaries) are pathognomic. Warts lack surface skin lines (dermatoglyphics) and do not umbilicate on paring (unlike clavi or corns).
Always discuss indications, procedure, alternatives, and risks prior to performing cryotherapy. Patients (and parents) need to understand that the “perfect” treatment for warts has yet to be devised. All currently available methods have shortcomings.
Consider using an ear speculum (3 to 5 mm) to concentrate the spray of liquid nitrogen, reducing pain and shortening the length of treatment. Particularly with thicker plantar warts, you might want to pare away surface keratotic material first, then use the “freeze-thaw-freeze” technique. The average length of a single treatment seldom exceeds five seconds, particularly if a speculum or other dam is used.
Arrange for follow-up, typically one month later, since it’s a rare wart that clears with a single treatment.
Without a doubt, the most useful treatment modality I’ve acquired in my career is the proper use of liquid nitrogen. Cryotherapy is the standard treatment for small epidermal lesions, because it allows for removal without the need for anesthesia, with no broken skin or bleeding, and with a tolerable amount of pain.
Over the years, I’ve learned—often the hard way!—how to work with liquid nitrogen. For example, for the past 25 years, I’ve used a cryogun (or “unit,” as it’s sometimes called), having discovered that applying LN2 with anything else (eg, a cotton-tipped applicator) is a huge exercise in futility.
As with any tool in medicine, there are uses and misuses of cryotherapy. It is not intuitive—but it does yield to common sense. So allow me to give you the benefit of my experience.
APPROPRIATE AND INAPPROPRIATE TARGETS
In general, only epidermal lesions of obvious origin are treated with liquid nitrogen. This includes ordinary skin tags, small warts, and seborrheic and actinic keratosis. Utterly common and easy to treat, these lesions have no potential for malignant transformation and have relatively poor vasculature, which makes them ideal candidates for controlled, localized frostbite. This process (which can take as long as two weeks) kills the cells, disrupts the blood supply, and causes the lesions to die and fall away.
Other uses for cryotherapy include softening keloids or hypertrophic scars sufficiently to facilitate intralesional injection; treating condyloma, molluscum contagiosum, and small chondromdermatitis nodules; and even to destroy skin cancers (basal cell and squamous cell carcinomas)—the latter, however, only by clinicians with specialized training.
Inappropriate targets for cryotherapy include “moles” (nevi), vascular lesions such as hemangiomas, “birthmarks,” or any lesion of unknown nature. There are two reasons not to use liquid nitrogen in these cases: First, relatively well–vascularized lesions will be superficially blistered but will survive the treatment. Second, these lesions have at least a theoretical chance of having undergone malignant transformation. (As such, these lesions may need to be sampled to determine whether they are malignant; but that is a discussion for another column.)
THE NEED FOR CAUTION
For all its positive features, there are drawbacks to using cryotherapy. Here are six to consider:
• Blistering, which can be severe in sensitive patients
• Dyschromia (color changes in treated skin), especially in darker-skinned patients
• Pain, particularly in children
• Loss of function (eg, nerve or cartilage damage)
• Scarring, usually from overtreatment
• Disability—walking (let alone running or working) may be painful for a day or two after treatment, especially following brisk cryotherapy of a larger plantar wart
TREATING WARTS WITH CRYOTHERAPY
First, you must confirm the diagnosis: “Seeds” (black dots on the surface, which really represent vertically aligned thrombosed capillaries) are pathognomic. Warts lack surface skin lines (dermatoglyphics) and do not umbilicate on paring (unlike clavi or corns).
Always discuss indications, procedure, alternatives, and risks prior to performing cryotherapy. Patients (and parents) need to understand that the “perfect” treatment for warts has yet to be devised. All currently available methods have shortcomings.
Consider using an ear speculum (3 to 5 mm) to concentrate the spray of liquid nitrogen, reducing pain and shortening the length of treatment. Particularly with thicker plantar warts, you might want to pare away surface keratotic material first, then use the “freeze-thaw-freeze” technique. The average length of a single treatment seldom exceeds five seconds, particularly if a speculum or other dam is used.
Arrange for follow-up, typically one month later, since it’s a rare wart that clears with a single treatment.
A Little Pink in the Cheeks Signals Serious Problem
A 37-year-old man presents with a complaint of a scalp rash that first appeared several months ago. On his friends’ advice, he tried changing shampoos and applying tea tree oil, with no discernible improvement. He has never had problems with his scalp before, but there is a family history of “unknown skin disease” (mentioned by his father in past conversations). The patient denies any other skin problems.
Recently, he’s experienced a lot of stress: job loss, marital strife, and a subsequent increase in alcohol intake. He has also gained weight, adding that he isn’t getting any exercise. He denies joint pain or swelling.
EXAMINATION
Faint scaling is seen in the patient’s scalp, most of it over and behind the ears. These areas are modestly excoriated as well. There is similar faint scale in both external auditory meati. Examination of the fingernails reveals modest, scattered pits in four nail plates, which the patient says have been there “on and off for years.”
Looking elsewhere, focal heavy white scaling is noted on one knee and both elbows. These findings prompt examination of the patient’s upper intergluteal area, where definite pinkish erythema is observed. It covers an area of 7 x 4 cm, with no significant scaling.
DISCUSSION
You might expect a skin disease that “runs in the family” to be well known to all of them, but in truth, it’s quite common for family members to suffer for years without seeking evaluation by dermatology. Even worse, they might be misdiagnosed by someone else and spend a lifetime thinking they have “eczema” or “ringworm,” and passing this misinformation on to their kin.
Psoriasis is like that; it can present in so many ways, and it can also vary extensively in severity and morphology from one generation to another. As such, while most cases of psoriasis are obvious and therefore easy to diagnose, some are more obscure.
It helps to know how common psoriasis is: It affects 3% of the population, which equates to about 9 million people in the United States. Many, like this patient, have relatively mild cases that flare with stress. Known stressors include infection (especially strep) or introduction of certain medications (eg, β-blockers and lithium). A history of genetic predisposition can be obtained in about 30% of such cases.
This case illustrates a major point about the diagnosis of psoriasis: Often, it must be cobbled together from a collection of findings that appear disconnected on first glance. The scalp findings, by themselves, could have simply represented dry skin or seborrhea. However, taken in context with the nail changes, the family history, the extensor involvement, and intergluteal “pinking,” an almost certain diagnosis emerges.
The intergluteal pinking, by the way, can also be seen with seborrhea—so it’s not pathognomic for psoriasis but is highly suggestive of that diagnosis. The salmon-pink color and lack of scaling (because of the friction and moisture in the affected area) are especially typical.
Those tempted to dismiss all of this as mere sophistry have likely never experienced the effects of this disease, which is notorious for its association with serious negative psychologic repercussions, such as depression, isolation, and suicide. Imagine, for example, having to vacuum out your bed and surrounding carpet every morning just to remove the skin that was shed overnight, then having to be seen and judged by the public.
And that’s not even the worst of it. Up to 30% of psoriatic patients go on to develop psoriatic arthropathy, a destructive form of inflammatory arthritis that is relentless in its course without correct diagnosis and treatment. We know a great deal more about this autoimmune disease now than when I started out in dermatology, and we have marvelous treatment for it, more of which are in the research pipeline.
But these are of little use without the one essential ingredient the clinician must supply: a correct diagnosis.
TAKE-HOME LEARNING POINTS
• Weight gain, smoking, and stress are all documented triggers for psoriasis.
• A positive family history of psoriasis can be obtained in about 30% of suspected cases, but a negative history does not rule out the diagnosis.
• Certain medications, such as β-blockers and lithium, can trigger exacerbations of psoriasis.
• Psoriasis often presents with what appear to be unrelated findings, such as scalp rash, nail pits, genital involvement, and intergluteal pinking.
• Psoriasis is associated with significant psychologic pathology, including poor body image, depression, isolation, increased incidence of drug and alcohol abuse, and suicide.
• Almost 30% of psoriasis patients will develop a related form of arthritis called psoriatic arthropathy.
• Effective treatment is available for psoriasis and psoriatic arthropathy, but these conditions must first be suspected and diagnosed.
A 37-year-old man presents with a complaint of a scalp rash that first appeared several months ago. On his friends’ advice, he tried changing shampoos and applying tea tree oil, with no discernible improvement. He has never had problems with his scalp before, but there is a family history of “unknown skin disease” (mentioned by his father in past conversations). The patient denies any other skin problems.
Recently, he’s experienced a lot of stress: job loss, marital strife, and a subsequent increase in alcohol intake. He has also gained weight, adding that he isn’t getting any exercise. He denies joint pain or swelling.
EXAMINATION
Faint scaling is seen in the patient’s scalp, most of it over and behind the ears. These areas are modestly excoriated as well. There is similar faint scale in both external auditory meati. Examination of the fingernails reveals modest, scattered pits in four nail plates, which the patient says have been there “on and off for years.”
Looking elsewhere, focal heavy white scaling is noted on one knee and both elbows. These findings prompt examination of the patient’s upper intergluteal area, where definite pinkish erythema is observed. It covers an area of 7 x 4 cm, with no significant scaling.
DISCUSSION
You might expect a skin disease that “runs in the family” to be well known to all of them, but in truth, it’s quite common for family members to suffer for years without seeking evaluation by dermatology. Even worse, they might be misdiagnosed by someone else and spend a lifetime thinking they have “eczema” or “ringworm,” and passing this misinformation on to their kin.
Psoriasis is like that; it can present in so many ways, and it can also vary extensively in severity and morphology from one generation to another. As such, while most cases of psoriasis are obvious and therefore easy to diagnose, some are more obscure.
It helps to know how common psoriasis is: It affects 3% of the population, which equates to about 9 million people in the United States. Many, like this patient, have relatively mild cases that flare with stress. Known stressors include infection (especially strep) or introduction of certain medications (eg, β-blockers and lithium). A history of genetic predisposition can be obtained in about 30% of such cases.
This case illustrates a major point about the diagnosis of psoriasis: Often, it must be cobbled together from a collection of findings that appear disconnected on first glance. The scalp findings, by themselves, could have simply represented dry skin or seborrhea. However, taken in context with the nail changes, the family history, the extensor involvement, and intergluteal “pinking,” an almost certain diagnosis emerges.
The intergluteal pinking, by the way, can also be seen with seborrhea—so it’s not pathognomic for psoriasis but is highly suggestive of that diagnosis. The salmon-pink color and lack of scaling (because of the friction and moisture in the affected area) are especially typical.
Those tempted to dismiss all of this as mere sophistry have likely never experienced the effects of this disease, which is notorious for its association with serious negative psychologic repercussions, such as depression, isolation, and suicide. Imagine, for example, having to vacuum out your bed and surrounding carpet every morning just to remove the skin that was shed overnight, then having to be seen and judged by the public.
And that’s not even the worst of it. Up to 30% of psoriatic patients go on to develop psoriatic arthropathy, a destructive form of inflammatory arthritis that is relentless in its course without correct diagnosis and treatment. We know a great deal more about this autoimmune disease now than when I started out in dermatology, and we have marvelous treatment for it, more of which are in the research pipeline.
But these are of little use without the one essential ingredient the clinician must supply: a correct diagnosis.
TAKE-HOME LEARNING POINTS
• Weight gain, smoking, and stress are all documented triggers for psoriasis.
• A positive family history of psoriasis can be obtained in about 30% of suspected cases, but a negative history does not rule out the diagnosis.
• Certain medications, such as β-blockers and lithium, can trigger exacerbations of psoriasis.
• Psoriasis often presents with what appear to be unrelated findings, such as scalp rash, nail pits, genital involvement, and intergluteal pinking.
• Psoriasis is associated with significant psychologic pathology, including poor body image, depression, isolation, increased incidence of drug and alcohol abuse, and suicide.
• Almost 30% of psoriasis patients will develop a related form of arthritis called psoriatic arthropathy.
• Effective treatment is available for psoriasis and psoriatic arthropathy, but these conditions must first be suspected and diagnosed.
A 37-year-old man presents with a complaint of a scalp rash that first appeared several months ago. On his friends’ advice, he tried changing shampoos and applying tea tree oil, with no discernible improvement. He has never had problems with his scalp before, but there is a family history of “unknown skin disease” (mentioned by his father in past conversations). The patient denies any other skin problems.
Recently, he’s experienced a lot of stress: job loss, marital strife, and a subsequent increase in alcohol intake. He has also gained weight, adding that he isn’t getting any exercise. He denies joint pain or swelling.
EXAMINATION
Faint scaling is seen in the patient’s scalp, most of it over and behind the ears. These areas are modestly excoriated as well. There is similar faint scale in both external auditory meati. Examination of the fingernails reveals modest, scattered pits in four nail plates, which the patient says have been there “on and off for years.”
Looking elsewhere, focal heavy white scaling is noted on one knee and both elbows. These findings prompt examination of the patient’s upper intergluteal area, where definite pinkish erythema is observed. It covers an area of 7 x 4 cm, with no significant scaling.
DISCUSSION
You might expect a skin disease that “runs in the family” to be well known to all of them, but in truth, it’s quite common for family members to suffer for years without seeking evaluation by dermatology. Even worse, they might be misdiagnosed by someone else and spend a lifetime thinking they have “eczema” or “ringworm,” and passing this misinformation on to their kin.
Psoriasis is like that; it can present in so many ways, and it can also vary extensively in severity and morphology from one generation to another. As such, while most cases of psoriasis are obvious and therefore easy to diagnose, some are more obscure.
It helps to know how common psoriasis is: It affects 3% of the population, which equates to about 9 million people in the United States. Many, like this patient, have relatively mild cases that flare with stress. Known stressors include infection (especially strep) or introduction of certain medications (eg, β-blockers and lithium). A history of genetic predisposition can be obtained in about 30% of such cases.
This case illustrates a major point about the diagnosis of psoriasis: Often, it must be cobbled together from a collection of findings that appear disconnected on first glance. The scalp findings, by themselves, could have simply represented dry skin or seborrhea. However, taken in context with the nail changes, the family history, the extensor involvement, and intergluteal “pinking,” an almost certain diagnosis emerges.
The intergluteal pinking, by the way, can also be seen with seborrhea—so it’s not pathognomic for psoriasis but is highly suggestive of that diagnosis. The salmon-pink color and lack of scaling (because of the friction and moisture in the affected area) are especially typical.
Those tempted to dismiss all of this as mere sophistry have likely never experienced the effects of this disease, which is notorious for its association with serious negative psychologic repercussions, such as depression, isolation, and suicide. Imagine, for example, having to vacuum out your bed and surrounding carpet every morning just to remove the skin that was shed overnight, then having to be seen and judged by the public.
And that’s not even the worst of it. Up to 30% of psoriatic patients go on to develop psoriatic arthropathy, a destructive form of inflammatory arthritis that is relentless in its course without correct diagnosis and treatment. We know a great deal more about this autoimmune disease now than when I started out in dermatology, and we have marvelous treatment for it, more of which are in the research pipeline.
But these are of little use without the one essential ingredient the clinician must supply: a correct diagnosis.
TAKE-HOME LEARNING POINTS
• Weight gain, smoking, and stress are all documented triggers for psoriasis.
• A positive family history of psoriasis can be obtained in about 30% of suspected cases, but a negative history does not rule out the diagnosis.
• Certain medications, such as β-blockers and lithium, can trigger exacerbations of psoriasis.
• Psoriasis often presents with what appear to be unrelated findings, such as scalp rash, nail pits, genital involvement, and intergluteal pinking.
• Psoriasis is associated with significant psychologic pathology, including poor body image, depression, isolation, increased incidence of drug and alcohol abuse, and suicide.
• Almost 30% of psoriasis patients will develop a related form of arthritis called psoriatic arthropathy.
• Effective treatment is available for psoriasis and psoriatic arthropathy, but these conditions must first be suspected and diagnosed.
A Purplish Rash on the Instep
ANSWER
The correct answer is lichen planus (choice “d”), an inflammatory condition marked by pathognomic histologic changes such as those described. Hardly on the tip of most primary care providers’ tongues, lichen planus is nonetheless quite commonly occuring.
Tinea pedis (choice “a”) certainly belongs in the differential, but it would be unusual in a woman of this age. The biopsy effectively ruled it out, as it did psoriasis (choice “b”) and contact dermatitis (choice “c”).
DISCUSSION
Lichen planus (LP) is the prototypical lichenoid interface dermatitis; an inflammatory infiltrate erases the normally well-defined dermoepidermal junction, replacing it with a jagged “sawtooth” band of lymphocytes. In the process, the cells it kills off (keratinocytes) collect at this interface and are incorporated as necrotic keratinocytes into the papillary dermis.
Classically, the recognition of LP is taught with “The Ps.” LP is said to be:
• Papular
• Planar
• Purple
• Polygonal
• Pruritic
• Plaquish
• Penile
• Puzzling
The word “puzzling” may sound nebulous, but it is actually quite useful for dermatology providers. It comes into play whenever a condition stumps us—as in, “What in the world is this?” This puzzlement causes us to at least consider LP.
LP is often papular, and these papules often have flat (planar) tops. The purple color is more striking in lighter-skinned patients. (Almost all inflammatory conditions are more difficult to diagnose in those with darker skin.) More typically, LP lesions are multi-angular or polygonal, often plaquish, and almost invariably pruritic.
LP is common on the legs and/or trunk, favoring the sacral area. It can affect the scalp (being in the differential for hair loss), can cause nail dystrophy, and is relatively common in the mouth, where it presents as a lacy, reticular, slightly erosive process, usually affecting the buccal mucosae. It may be found on the penis, where it is usually confined to the distal shaft and proximal coronal areas.
Like many otherwise benign conditions, LP can present with bullae. In anterior tibial areas, especially on darker-skinned persons, it can be remarkably hypertrophic.
The cause is usually unknown, although certain drugs—especially the antimalarials, gold salts, and penicillamine—have been known to cause LP-like eruptions. It’s been my observation that flares of LP are prompted by stress (certainly present in this patient’s case). LP may have a connection to hepatitis, although no convincing connection has been established.
TREATMENT
This patient’s condition was treated with topical class I corticosteroid ointment (halobetasol). For her, having a certain diagnosis was almost as important as successful treatment.
ANSWER
The correct answer is lichen planus (choice “d”), an inflammatory condition marked by pathognomic histologic changes such as those described. Hardly on the tip of most primary care providers’ tongues, lichen planus is nonetheless quite commonly occuring.
Tinea pedis (choice “a”) certainly belongs in the differential, but it would be unusual in a woman of this age. The biopsy effectively ruled it out, as it did psoriasis (choice “b”) and contact dermatitis (choice “c”).
DISCUSSION
Lichen planus (LP) is the prototypical lichenoid interface dermatitis; an inflammatory infiltrate erases the normally well-defined dermoepidermal junction, replacing it with a jagged “sawtooth” band of lymphocytes. In the process, the cells it kills off (keratinocytes) collect at this interface and are incorporated as necrotic keratinocytes into the papillary dermis.
Classically, the recognition of LP is taught with “The Ps.” LP is said to be:
• Papular
• Planar
• Purple
• Polygonal
• Pruritic
• Plaquish
• Penile
• Puzzling
The word “puzzling” may sound nebulous, but it is actually quite useful for dermatology providers. It comes into play whenever a condition stumps us—as in, “What in the world is this?” This puzzlement causes us to at least consider LP.
LP is often papular, and these papules often have flat (planar) tops. The purple color is more striking in lighter-skinned patients. (Almost all inflammatory conditions are more difficult to diagnose in those with darker skin.) More typically, LP lesions are multi-angular or polygonal, often plaquish, and almost invariably pruritic.
LP is common on the legs and/or trunk, favoring the sacral area. It can affect the scalp (being in the differential for hair loss), can cause nail dystrophy, and is relatively common in the mouth, where it presents as a lacy, reticular, slightly erosive process, usually affecting the buccal mucosae. It may be found on the penis, where it is usually confined to the distal shaft and proximal coronal areas.
Like many otherwise benign conditions, LP can present with bullae. In anterior tibial areas, especially on darker-skinned persons, it can be remarkably hypertrophic.
The cause is usually unknown, although certain drugs—especially the antimalarials, gold salts, and penicillamine—have been known to cause LP-like eruptions. It’s been my observation that flares of LP are prompted by stress (certainly present in this patient’s case). LP may have a connection to hepatitis, although no convincing connection has been established.
TREATMENT
This patient’s condition was treated with topical class I corticosteroid ointment (halobetasol). For her, having a certain diagnosis was almost as important as successful treatment.
ANSWER
The correct answer is lichen planus (choice “d”), an inflammatory condition marked by pathognomic histologic changes such as those described. Hardly on the tip of most primary care providers’ tongues, lichen planus is nonetheless quite commonly occuring.
Tinea pedis (choice “a”) certainly belongs in the differential, but it would be unusual in a woman of this age. The biopsy effectively ruled it out, as it did psoriasis (choice “b”) and contact dermatitis (choice “c”).
DISCUSSION
Lichen planus (LP) is the prototypical lichenoid interface dermatitis; an inflammatory infiltrate erases the normally well-defined dermoepidermal junction, replacing it with a jagged “sawtooth” band of lymphocytes. In the process, the cells it kills off (keratinocytes) collect at this interface and are incorporated as necrotic keratinocytes into the papillary dermis.
Classically, the recognition of LP is taught with “The Ps.” LP is said to be:
• Papular
• Planar
• Purple
• Polygonal
• Pruritic
• Plaquish
• Penile
• Puzzling
The word “puzzling” may sound nebulous, but it is actually quite useful for dermatology providers. It comes into play whenever a condition stumps us—as in, “What in the world is this?” This puzzlement causes us to at least consider LP.
LP is often papular, and these papules often have flat (planar) tops. The purple color is more striking in lighter-skinned patients. (Almost all inflammatory conditions are more difficult to diagnose in those with darker skin.) More typically, LP lesions are multi-angular or polygonal, often plaquish, and almost invariably pruritic.
LP is common on the legs and/or trunk, favoring the sacral area. It can affect the scalp (being in the differential for hair loss), can cause nail dystrophy, and is relatively common in the mouth, where it presents as a lacy, reticular, slightly erosive process, usually affecting the buccal mucosae. It may be found on the penis, where it is usually confined to the distal shaft and proximal coronal areas.
Like many otherwise benign conditions, LP can present with bullae. In anterior tibial areas, especially on darker-skinned persons, it can be remarkably hypertrophic.
The cause is usually unknown, although certain drugs—especially the antimalarials, gold salts, and penicillamine—have been known to cause LP-like eruptions. It’s been my observation that flares of LP are prompted by stress (certainly present in this patient’s case). LP may have a connection to hepatitis, although no convincing connection has been established.
TREATMENT
This patient’s condition was treated with topical class I corticosteroid ointment (halobetasol). For her, having a certain diagnosis was almost as important as successful treatment.
Several months ago, an itchy rash appeared on the foot of this 61-year-old African-American woman. She tried using a variety of OTC and prescription products, including tolnaftate cream, hydrocortisone 1% cream, and triamcinolone cream, but the rash persisted. She reports that the triamcinolone cream did relieve the itching for a few minutes after application, but the symptoms always returned. The rash has gradually grown. The patient, a retired schoolteacher, denies any other skin problems. She does have several relatively minor health problems, including hypertension (well controlled with metoprolol) and mild reactive airway disease (related to a 20-year history of smoking). The rash manifested around the time she was forced to retire in an unforeseen downsizing effort by her school district. As a result, she lost her health care coverage and could not afford private insurance for herself and her husband (neither of whom are old enough to qualify for Medicare). The rash, which covers a roughly 12 × 8–cm area, begins on her left instep and spills onto the lower ankle. It is quite dark, as would be expected in a person with type V skin, but there is a slightly purplish tinge to it. The surface of the affected area is a bit shiny and atrophic, and the margins are well defined and annular. There is almost no scaling, and the rest of the skin on her feet and legs is well within normal limits. A 4-mm punch biopsy is performed on the lesion. The pathology report shows obliteration of the dermoepidermal junction by a bandlike lymphocytic infiltrate in the papillary dermis, associated with vacuolar changes and the accumulation of necrotic keratinocytes in the basal layer.
