Case Letter

To the Editor:
Leser-Trélat sign is a rare skin condition characterized by the sudden appearance of seborrheic keratoses that rapidly increase in number and size within weeks to months. Co-occurrence has been reported with a large number of malignancies, particularly adenocarcinoma and lymphoma. We present a case of Leser-Trélat sign that was not associated with an underlying malignancy.

A 44-year-old man was admitted to our dermatology outpatient department with a serpigo on the neck that had grown rapidly in the last month. His medical history and family history were unremarkable. Dermatologic examination revealed numerous 3- to 4-mm brown and slightly verrucous papules on the neck (Figure 1). A punch biopsy of the lesion showed acanthosis of predominantly basaloid cells, papillomatosis, and hyperkeratosis, as well as the presence of characteristic horn cysts (Figure 2). He was tested for possible underlying internal malignancy. Liver and kidney function tests, electrolyte count, protein electrophoresis, and whole blood and urine tests were within reference range. Chest radiography and abdominal ultrasonography revealed no signs of pathology. The erythrocyte sedimentation rate was 20 mm/h (reference range, 0–20 mm/h) and tests for hepatitis, human immunodeficiency virus, and syphilis were negative. Abdominal, cranial, and thorax computed tomography revealed no abnormalities. Otolaryngologic examinations also were negative. Additional endoscopic analyses, esophagogastroduodenoscopy, and colonoscopy revealed no abnormalities. At 1-year follow-up, the seborrheic keratoses remained unchanged. He has remained in good health without specific signs or symptoms suggestive of an underlying malignancy.

Paraneoplastic syndromes are associated with malignancy but progress without connection to a primary tumor or metastasis and form a group of clinical manifestations. The characteristic progress of paraneoplastic syndromes shows parallelism with the progression of the tumor. The mechanism underlying the development is not known, though the actions of bioactive substances that cause responses in the tumor, such as polypeptide hormones, hormonelike peptides, antibodies or immune complexes, and cytokines or growth factors, have been implicated.¹

Although the term paraneoplastic syndrome commonly is used for Leser-Trélat sign, we do not believe it is accurate. As Fink et al² and Schwengle et al³ indicated, the possibility of the co-occurrence being fortuitous is high. Showing a parallel progress of malignancy with paraneoplastic dermatosis requires that the paraneoplastic syndrome also diminish when the tumor is cured.⁴ It should then reappear with cancer recurrence or metastasis, which has not been exhibited in many case presentations in the literature.³ Disease regression was observed in only 1 of 3 seborrheic keratosis cases after primary cancer treatment.⁵

In patients with a malignancy, the sudden increase in seborrheic keratosis is based exclusively on the subjective evaluation of the patient, which may not be reliable. Schwengle et al³ stated that this sudden increase can be related to the awareness level of the patient who had a cancer diagnosis. Bräuer et al⁶ stated that no plausible definition distinguishes eruptive versus common seborrheic keratoses.

As a result, the results regarding the relationship between malignancy and Leser-Trélat sign are conflicting, and no strong evidence supports the presence of the sign. Only case reports have suggested that Leser-Trélat sign accompanies malignancy. Studies investigating its etiopathogenesis have not revealed a substance that has been released from or as a response to a tumor.

We believe that the presence of eruptive seborrheic keratosis does not necessitate screening for underlying internal malignancies.

To the Editor:
Leser-Trélat sign is a rare skin condition characterized by the sudden appearance of seborrheic keratoses that rapidly increase in number and size within weeks to months. Co-occurrence has been reported with a large number of malignancies, particularly adenocarcinoma and lymphoma. We present a case of Leser-Trélat sign that was not associated with an underlying malignancy.

A 44-year-old man was admitted to our dermatology outpatient department with a serpigo on the neck that had grown rapidly in the last month. His medical history and family history were unremarkable. Dermatologic examination revealed numerous 3- to 4-mm brown and slightly verrucous papules on the neck (Figure 1). A punch biopsy of the lesion showed acanthosis of predominantly basaloid cells, papillomatosis, and hyperkeratosis, as well as the presence of characteristic horn cysts (Figure 2). He was tested for possible underlying internal malignancy. Liver and kidney function tests, electrolyte count, protein electrophoresis, and whole blood and urine tests were within reference range. Chest radiography and abdominal ultrasonography revealed no signs of pathology. The erythrocyte sedimentation rate was 20 mm/h (reference range, 0–20 mm/h) and tests for hepatitis, human immunodeficiency virus, and syphilis were negative. Abdominal, cranial, and thorax computed tomography revealed no abnormalities. Otolaryngologic examinations also were negative. Additional endoscopic analyses, esophagogastroduodenoscopy, and colonoscopy revealed no abnormalities. At 1-year follow-up, the seborrheic keratoses remained unchanged. He has remained in good health without specific signs or symptoms suggestive of an underlying malignancy.

Paraneoplastic syndromes are associated with malignancy but progress without connection to a primary tumor or metastasis and form a group of clinical manifestations. The characteristic progress of paraneoplastic syndromes shows parallelism with the progression of the tumor. The mechanism underlying the development is not known, though the actions of bioactive substances that cause responses in the tumor, such as polypeptide hormones, hormonelike peptides, antibodies or immune complexes, and cytokines or growth factors, have been implicated.¹

Although the term paraneoplastic syndrome commonly is used for Leser-Trélat sign, we do not believe it is accurate. As Fink et al² and Schwengle et al³ indicated, the possibility of the co-occurrence being fortuitous is high. Showing a parallel progress of malignancy with paraneoplastic dermatosis requires that the paraneoplastic syndrome also diminish when the tumor is cured.⁴ It should then reappear with cancer recurrence or metastasis, which has not been exhibited in many case presentations in the literature.³ Disease regression was observed in only 1 of 3 seborrheic keratosis cases after primary cancer treatment.⁵

In patients with a malignancy, the sudden increase in seborrheic keratosis is based exclusively on the subjective evaluation of the patient, which may not be reliable. Schwengle et al³ stated that this sudden increase can be related to the awareness level of the patient who had a cancer diagnosis. Bräuer et al⁶ stated that no plausible definition distinguishes eruptive versus common seborrheic keratoses.

As a result, the results regarding the relationship between malignancy and Leser-Trélat sign are conflicting, and no strong evidence supports the presence of the sign. Only case reports have suggested that Leser-Trélat sign accompanies malignancy. Studies investigating its etiopathogenesis have not revealed a substance that has been released from or as a response to a tumor.

We believe that the presence of eruptive seborrheic keratosis does not necessitate screening for underlying internal malignancies.

To the Editor:
Leser-Trélat sign is a rare skin condition characterized by the sudden appearance of seborrheic keratoses that rapidly increase in number and size within weeks to months. Co-occurrence has been reported with a large number of malignancies, particularly adenocarcinoma and lymphoma. We present a case of Leser-Trélat sign that was not associated with an underlying malignancy.

A 44-year-old man was admitted to our dermatology outpatient department with a serpigo on the neck that had grown rapidly in the last month. His medical history and family history were unremarkable. Dermatologic examination revealed numerous 3- to 4-mm brown and slightly verrucous papules on the neck (Figure 1). A punch biopsy of the lesion showed acanthosis of predominantly basaloid cells, papillomatosis, and hyperkeratosis, as well as the presence of characteristic horn cysts (Figure 2). He was tested for possible underlying internal malignancy. Liver and kidney function tests, electrolyte count, protein electrophoresis, and whole blood and urine tests were within reference range. Chest radiography and abdominal ultrasonography revealed no signs of pathology. The erythrocyte sedimentation rate was 20 mm/h (reference range, 0–20 mm/h) and tests for hepatitis, human immunodeficiency virus, and syphilis were negative. Abdominal, cranial, and thorax computed tomography revealed no abnormalities. Otolaryngologic examinations also were negative. Additional endoscopic analyses, esophagogastroduodenoscopy, and colonoscopy revealed no abnormalities. At 1-year follow-up, the seborrheic keratoses remained unchanged. He has remained in good health without specific signs or symptoms suggestive of an underlying malignancy.

Paraneoplastic syndromes are associated with malignancy but progress without connection to a primary tumor or metastasis and form a group of clinical manifestations. The characteristic progress of paraneoplastic syndromes shows parallelism with the progression of the tumor. The mechanism underlying the development is not known, though the actions of bioactive substances that cause responses in the tumor, such as polypeptide hormones, hormonelike peptides, antibodies or immune complexes, and cytokines or growth factors, have been implicated.¹

Although the term paraneoplastic syndrome commonly is used for Leser-Trélat sign, we do not believe it is accurate. As Fink et al² and Schwengle et al³ indicated, the possibility of the co-occurrence being fortuitous is high. Showing a parallel progress of malignancy with paraneoplastic dermatosis requires that the paraneoplastic syndrome also diminish when the tumor is cured.⁴ It should then reappear with cancer recurrence or metastasis, which has not been exhibited in many case presentations in the literature.³ Disease regression was observed in only 1 of 3 seborrheic keratosis cases after primary cancer treatment.⁵

In patients with a malignancy, the sudden increase in seborrheic keratosis is based exclusively on the subjective evaluation of the patient, which may not be reliable. Schwengle et al³ stated that this sudden increase can be related to the awareness level of the patient who had a cancer diagnosis. Bräuer et al⁶ stated that no plausible definition distinguishes eruptive versus common seborrheic keratoses.

As a result, the results regarding the relationship between malignancy and Leser-Trélat sign are conflicting, and no strong evidence supports the presence of the sign. Only case reports have suggested that Leser-Trélat sign accompanies malignancy. Studies investigating its etiopathogenesis have not revealed a substance that has been released from or as a response to a tumor.

We believe that the presence of eruptive seborrheic keratosis does not necessitate screening for underlying internal malignancies.

To the Editor:
Intralesional corticosteroids and pulsed dye laser (PDL)(585–595 nm), either as monotherapy or combination therapy, are commonly used to treat hypertrophic scars.¹ We describe an unusual adverse effect of protracted necrosis following combination therapy with intralesional triamcinolone acetonide and PDL for hypertrophic scar revision.

A 29-year-old healthy man presented after sustaining a contaminated crush trauma to the medial aspect of the left foot (Figure, A), requiring staged reconstruction with split-thickness skin grafting. The site healed with a hypertrophic scar (Figure, B), and treatment was pursued 18 months later with a community dermatologist with laser experience. Intralesional triamcinolone acetonide from a new vial diluted with 2% lidocaine was injected into the hypertrophic portions of the scar (20 mg/mL; 1.5 cc injected). The scar was then treated with a PDL at settings of 595 nm, 7-mm spot size, 10 J/cm², pulse duration of 1.5 milliseconds, 30-millisecond spray with a 20-millisecond delay of cryogen spray, and double-stacked pulses with a 2- to 3-second delay between pulses. The patient reported that purpura was present in treated areas immediately after treatment.

At 6 days posttreatment, the first evidence of possible necrosis with tissue depression in mid posterior portions of the scar appeared (Figure, C). At day 14, deep ulcerations became evident (Figure, D), and by day 21, an exudative, yellow, fibrinopurulent membrane appeared (Figure, E). By 7 weeks posttreatment, ulceration size was only slightly reduced (Figure, F).

The necrosis was treated with debridement and local wound care using collagen matrix dressings (protease modulating matrix). Seven months after attempted scar revision, a deep 4-mm ulcer with a narrow tunnel-like connection to the skin surface remained anteriorly, disconnected from earlier mid posterior ulcerations, which was indicative of delayed onset of necrosis (Figure, G).

				The medial aspect of the left foot sustained a contaminated crush trauma (A). Following reconstruction, the site healed with a hypertrophic scar (B). Six days after treatment with intralesional triamcinolone acetonide and pulsed dye laser, purpura and tissue depression were observed in mid posterior portions of the scar (C). At 14 days posttreatment, deep ulcerations were evident (D). At 21 days posttreatment, exudative fibrinopurulent membrane appeared (E). At 7 weeks posttreatment, ulceration size was slightly reduced (F). At 7 months posttreatment, a delayed-onset, deep ulceration remained anteriorly (G).

Intralesional corticosteroids have been associated with several side effects including pain, dermal atrophy, infection, pigmentation changes, and telangiectases.² Pulsed dye laser therapy is generally described as safe with mild and transient side effects including purpura (resolves in 7–10 days), edema (diminishes in 48 hours), hyperpigmentation, and pain.³ More serious complications such as ulceration and scarring were initially described with PDL for purely vascular cutaneous lesions,⁴ and reports of necrosis and scarring have followed with PDL treatment utilizing higher fluences, shorter pulse durations, and stacking of pulses.⁵

Combination therapy for scar revision with intralesional triamcinolone acetonide and PDL reduces the corticosteroid dose and has been shown to be more efficacious than either modality alone with minimal to no complications.⁶ In our case, we believe the therapeutic combination resulted in several effects including bulk heating, vascular compromise, and inhibition of wound healing that together contributed to protracted necrosis. Triamcinolone acetonide was injected first; the corticosteroid fluid bolus likely conducted heat energy from the PDL spot leading to tissue damage, which corresponded clinically to the deep, delayed-onset necrosis. The PDL’s destructive effect on the vasculature after stacked pulses likely led to the initial necrosis, with wound healing time potentially extended by the anti-inflammatory effect of the corticosteroids. The location on the lower extremity, an area with limited vasculature and subcutaneous tissue, may have further contributed to impaired wound healing. Finally, the underlying scar tissue was by nature devoid of adnexal structures to expedite wound healing. Although the combination of intralesional corticosteroids and PDL is widely used and considered safe, we suggest that dermatologists carefully choose PDL parameters and pulse delivery methods when utilizing these modalities for scar revision.

To the Editor:
Intralesional corticosteroids and pulsed dye laser (PDL)(585–595 nm), either as monotherapy or combination therapy, are commonly used to treat hypertrophic scars.¹ We describe an unusual adverse effect of protracted necrosis following combination therapy with intralesional triamcinolone acetonide and PDL for hypertrophic scar revision.

A 29-year-old healthy man presented after sustaining a contaminated crush trauma to the medial aspect of the left foot (Figure, A), requiring staged reconstruction with split-thickness skin grafting. The site healed with a hypertrophic scar (Figure, B), and treatment was pursued 18 months later with a community dermatologist with laser experience. Intralesional triamcinolone acetonide from a new vial diluted with 2% lidocaine was injected into the hypertrophic portions of the scar (20 mg/mL; 1.5 cc injected). The scar was then treated with a PDL at settings of 595 nm, 7-mm spot size, 10 J/cm², pulse duration of 1.5 milliseconds, 30-millisecond spray with a 20-millisecond delay of cryogen spray, and double-stacked pulses with a 2- to 3-second delay between pulses. The patient reported that purpura was present in treated areas immediately after treatment.

At 6 days posttreatment, the first evidence of possible necrosis with tissue depression in mid posterior portions of the scar appeared (Figure, C). At day 14, deep ulcerations became evident (Figure, D), and by day 21, an exudative, yellow, fibrinopurulent membrane appeared (Figure, E). By 7 weeks posttreatment, ulceration size was only slightly reduced (Figure, F).

The necrosis was treated with debridement and local wound care using collagen matrix dressings (protease modulating matrix). Seven months after attempted scar revision, a deep 4-mm ulcer with a narrow tunnel-like connection to the skin surface remained anteriorly, disconnected from earlier mid posterior ulcerations, which was indicative of delayed onset of necrosis (Figure, G).

				The medial aspect of the left foot sustained a contaminated crush trauma (A). Following reconstruction, the site healed with a hypertrophic scar (B). Six days after treatment with intralesional triamcinolone acetonide and pulsed dye laser, purpura and tissue depression were observed in mid posterior portions of the scar (C). At 14 days posttreatment, deep ulcerations were evident (D). At 21 days posttreatment, exudative fibrinopurulent membrane appeared (E). At 7 weeks posttreatment, ulceration size was slightly reduced (F). At 7 months posttreatment, a delayed-onset, deep ulceration remained anteriorly (G).

Intralesional corticosteroids have been associated with several side effects including pain, dermal atrophy, infection, pigmentation changes, and telangiectases.² Pulsed dye laser therapy is generally described as safe with mild and transient side effects including purpura (resolves in 7–10 days), edema (diminishes in 48 hours), hyperpigmentation, and pain.³ More serious complications such as ulceration and scarring were initially described with PDL for purely vascular cutaneous lesions,⁴ and reports of necrosis and scarring have followed with PDL treatment utilizing higher fluences, shorter pulse durations, and stacking of pulses.⁵

Combination therapy for scar revision with intralesional triamcinolone acetonide and PDL reduces the corticosteroid dose and has been shown to be more efficacious than either modality alone with minimal to no complications.⁶ In our case, we believe the therapeutic combination resulted in several effects including bulk heating, vascular compromise, and inhibition of wound healing that together contributed to protracted necrosis. Triamcinolone acetonide was injected first; the corticosteroid fluid bolus likely conducted heat energy from the PDL spot leading to tissue damage, which corresponded clinically to the deep, delayed-onset necrosis. The PDL’s destructive effect on the vasculature after stacked pulses likely led to the initial necrosis, with wound healing time potentially extended by the anti-inflammatory effect of the corticosteroids. The location on the lower extremity, an area with limited vasculature and subcutaneous tissue, may have further contributed to impaired wound healing. Finally, the underlying scar tissue was by nature devoid of adnexal structures to expedite wound healing. Although the combination of intralesional corticosteroids and PDL is widely used and considered safe, we suggest that dermatologists carefully choose PDL parameters and pulse delivery methods when utilizing these modalities for scar revision.

To the Editor:
Intralesional corticosteroids and pulsed dye laser (PDL)(585–595 nm), either as monotherapy or combination therapy, are commonly used to treat hypertrophic scars.¹ We describe an unusual adverse effect of protracted necrosis following combination therapy with intralesional triamcinolone acetonide and PDL for hypertrophic scar revision.

A 29-year-old healthy man presented after sustaining a contaminated crush trauma to the medial aspect of the left foot (Figure, A), requiring staged reconstruction with split-thickness skin grafting. The site healed with a hypertrophic scar (Figure, B), and treatment was pursued 18 months later with a community dermatologist with laser experience. Intralesional triamcinolone acetonide from a new vial diluted with 2% lidocaine was injected into the hypertrophic portions of the scar (20 mg/mL; 1.5 cc injected). The scar was then treated with a PDL at settings of 595 nm, 7-mm spot size, 10 J/cm², pulse duration of 1.5 milliseconds, 30-millisecond spray with a 20-millisecond delay of cryogen spray, and double-stacked pulses with a 2- to 3-second delay between pulses. The patient reported that purpura was present in treated areas immediately after treatment.

At 6 days posttreatment, the first evidence of possible necrosis with tissue depression in mid posterior portions of the scar appeared (Figure, C). At day 14, deep ulcerations became evident (Figure, D), and by day 21, an exudative, yellow, fibrinopurulent membrane appeared (Figure, E). By 7 weeks posttreatment, ulceration size was only slightly reduced (Figure, F).

The necrosis was treated with debridement and local wound care using collagen matrix dressings (protease modulating matrix). Seven months after attempted scar revision, a deep 4-mm ulcer with a narrow tunnel-like connection to the skin surface remained anteriorly, disconnected from earlier mid posterior ulcerations, which was indicative of delayed onset of necrosis (Figure, G).

				The medial aspect of the left foot sustained a contaminated crush trauma (A). Following reconstruction, the site healed with a hypertrophic scar (B). Six days after treatment with intralesional triamcinolone acetonide and pulsed dye laser, purpura and tissue depression were observed in mid posterior portions of the scar (C). At 14 days posttreatment, deep ulcerations were evident (D). At 21 days posttreatment, exudative fibrinopurulent membrane appeared (E). At 7 weeks posttreatment, ulceration size was slightly reduced (F). At 7 months posttreatment, a delayed-onset, deep ulceration remained anteriorly (G).

Intralesional corticosteroids have been associated with several side effects including pain, dermal atrophy, infection, pigmentation changes, and telangiectases.² Pulsed dye laser therapy is generally described as safe with mild and transient side effects including purpura (resolves in 7–10 days), edema (diminishes in 48 hours), hyperpigmentation, and pain.³ More serious complications such as ulceration and scarring were initially described with PDL for purely vascular cutaneous lesions,⁴ and reports of necrosis and scarring have followed with PDL treatment utilizing higher fluences, shorter pulse durations, and stacking of pulses.⁵

Combination therapy for scar revision with intralesional triamcinolone acetonide and PDL reduces the corticosteroid dose and has been shown to be more efficacious than either modality alone with minimal to no complications.⁶ In our case, we believe the therapeutic combination resulted in several effects including bulk heating, vascular compromise, and inhibition of wound healing that together contributed to protracted necrosis. Triamcinolone acetonide was injected first; the corticosteroid fluid bolus likely conducted heat energy from the PDL spot leading to tissue damage, which corresponded clinically to the deep, delayed-onset necrosis. The PDL’s destructive effect on the vasculature after stacked pulses likely led to the initial necrosis, with wound healing time potentially extended by the anti-inflammatory effect of the corticosteroids. The location on the lower extremity, an area with limited vasculature and subcutaneous tissue, may have further contributed to impaired wound healing. Finally, the underlying scar tissue was by nature devoid of adnexal structures to expedite wound healing. Although the combination of intralesional corticosteroids and PDL is widely used and considered safe, we suggest that dermatologists carefully choose PDL parameters and pulse delivery methods when utilizing these modalities for scar revision.

To the Editor:
A 61-year-old woman with a history of atrial fibrillation, type 2 diabetes mellitus, and hyperlipidemia presented with an erythematous, edematous, pruritic eruption on the chest, neck, and arms of 2 weeks’ duration. The patient had no history of considerable sun exposure or reports of photosensitivity. One month prior to presentation she had started taking dronedarone for improved control of atrial fibrillation. She had no known history of drug allergies. Other medications included valsartan, digoxin, pioglitazone, simvastatin, aspirin, hydrocodone, and zolpidem, all of which were unchanged for years. There were no changes in topical products used.

Physical examination revealed confluent, well-demarcated, erythematous and edematous papules and plaques over the anterior aspect of the neck, bilateral forearms, and dorsal aspect of the hands, with a v-shaped distribution on the chest (Figure). There was notable sparing of the submental region, upper arms, abdomen, back, and legs. Dronedarone was discontinued and she was started on fluocinonide ointment 0.05% and oral hydroxyzine for pruritus. Her rash resolved within the following few weeks.

Confluent, well-demarcated, erythematous and edematous papules and plaques in a v-shaped distribution on the chest (A) and dorsal aspect of the hands (B).

Dronedarone is a noniodinated benzofuran derivative. It is structurally similar to and shares the antiarrhythmic properties of amiodarone,¹ and thus it is used in the treatment of atrial fibrillation and atrial flutter. However, the pulmonary and thyroid toxicities sometimes associated with amiodarone have not been observed with dronedarone. The primary side effect of dronedarone is gastrointestinal distress, specifically nausea, vomiting, and diarrhea. Dronedarone has been associated with severe liver injury and hepatic failure.² Cutaneous reactions appear to be an uncommon side effect of dronedarone therapy. Across 5 clinical studies (N=6285), adverse events involving skin and subcutaneous tissue including eczema, allergic dermatitis, pruritus, and nonspecific rash occurred in 5% of dronedarone and 3% of placebo patients. Photosensitivity reactions occurred in less than 1% of dronedarone recipients.³

Although the lack of a biopsy leaves the possibility of a contact or photocontact dermatitis, our patient demonstrated the potential for dronedarone to cause a photodistributed drug eruption that resolved after cessation of the medication.

To the Editor:
A 61-year-old woman with a history of atrial fibrillation, type 2 diabetes mellitus, and hyperlipidemia presented with an erythematous, edematous, pruritic eruption on the chest, neck, and arms of 2 weeks’ duration. The patient had no history of considerable sun exposure or reports of photosensitivity. One month prior to presentation she had started taking dronedarone for improved control of atrial fibrillation. She had no known history of drug allergies. Other medications included valsartan, digoxin, pioglitazone, simvastatin, aspirin, hydrocodone, and zolpidem, all of which were unchanged for years. There were no changes in topical products used.

Physical examination revealed confluent, well-demarcated, erythematous and edematous papules and plaques over the anterior aspect of the neck, bilateral forearms, and dorsal aspect of the hands, with a v-shaped distribution on the chest (Figure). There was notable sparing of the submental region, upper arms, abdomen, back, and legs. Dronedarone was discontinued and she was started on fluocinonide ointment 0.05% and oral hydroxyzine for pruritus. Her rash resolved within the following few weeks.

Confluent, well-demarcated, erythematous and edematous papules and plaques in a v-shaped distribution on the chest (A) and dorsal aspect of the hands (B).

Dronedarone is a noniodinated benzofuran derivative. It is structurally similar to and shares the antiarrhythmic properties of amiodarone,¹ and thus it is used in the treatment of atrial fibrillation and atrial flutter. However, the pulmonary and thyroid toxicities sometimes associated with amiodarone have not been observed with dronedarone. The primary side effect of dronedarone is gastrointestinal distress, specifically nausea, vomiting, and diarrhea. Dronedarone has been associated with severe liver injury and hepatic failure.² Cutaneous reactions appear to be an uncommon side effect of dronedarone therapy. Across 5 clinical studies (N=6285), adverse events involving skin and subcutaneous tissue including eczema, allergic dermatitis, pruritus, and nonspecific rash occurred in 5% of dronedarone and 3% of placebo patients. Photosensitivity reactions occurred in less than 1% of dronedarone recipients.³

Although the lack of a biopsy leaves the possibility of a contact or photocontact dermatitis, our patient demonstrated the potential for dronedarone to cause a photodistributed drug eruption that resolved after cessation of the medication.

To the Editor:
A 61-year-old woman with a history of atrial fibrillation, type 2 diabetes mellitus, and hyperlipidemia presented with an erythematous, edematous, pruritic eruption on the chest, neck, and arms of 2 weeks’ duration. The patient had no history of considerable sun exposure or reports of photosensitivity. One month prior to presentation she had started taking dronedarone for improved control of atrial fibrillation. She had no known history of drug allergies. Other medications included valsartan, digoxin, pioglitazone, simvastatin, aspirin, hydrocodone, and zolpidem, all of which were unchanged for years. There were no changes in topical products used.

Physical examination revealed confluent, well-demarcated, erythematous and edematous papules and plaques over the anterior aspect of the neck, bilateral forearms, and dorsal aspect of the hands, with a v-shaped distribution on the chest (Figure). There was notable sparing of the submental region, upper arms, abdomen, back, and legs. Dronedarone was discontinued and she was started on fluocinonide ointment 0.05% and oral hydroxyzine for pruritus. Her rash resolved within the following few weeks.

Confluent, well-demarcated, erythematous and edematous papules and plaques in a v-shaped distribution on the chest (A) and dorsal aspect of the hands (B).

Dronedarone is a noniodinated benzofuran derivative. It is structurally similar to and shares the antiarrhythmic properties of amiodarone,¹ and thus it is used in the treatment of atrial fibrillation and atrial flutter. However, the pulmonary and thyroid toxicities sometimes associated with amiodarone have not been observed with dronedarone. The primary side effect of dronedarone is gastrointestinal distress, specifically nausea, vomiting, and diarrhea. Dronedarone has been associated with severe liver injury and hepatic failure.² Cutaneous reactions appear to be an uncommon side effect of dronedarone therapy. Across 5 clinical studies (N=6285), adverse events involving skin and subcutaneous tissue including eczema, allergic dermatitis, pruritus, and nonspecific rash occurred in 5% of dronedarone and 3% of placebo patients. Photosensitivity reactions occurred in less than 1% of dronedarone recipients.³

Although the lack of a biopsy leaves the possibility of a contact or photocontact dermatitis, our patient demonstrated the potential for dronedarone to cause a photodistributed drug eruption that resolved after cessation of the medication.

To the Editor:
Dermal melanocytosis is thought to be the result of a defect in melanoblast migration during embryogenesis and is characterized by the presence of functional fusiform and dendritic melanocytes in the dermis. Congenital dermal melanocytosis is classified into various subtypes based on the distribution, morphology, natural history of lesions, and distinctive histologic findings. We present an unusual case of congenital dermal melanocytosis that might fit the rare entity of dermal melanocyte hamartoma (DMH).

Figure 1. Uniform grayish blue patches over the torso with sharp demarcation lines that followed a dermatomal distribution.

Figure 2. Three conspicuous darker blue macules (arrows) within a bluish patch.

Figure 3. Intradermal dendritic pigmented melanocytes localized in the upper dermis and arranged parallel to the skin surface (H&E, original magnification ×100 [inset, original magnification ×400]).

A 4-month-old girl presented with bilateral bluish patches over the trunk and upper extremities. The lesions were present since birth, and remained entirely unchanged during a follow-up period of 18 months. Mental and physical development was normal. There was no family history of pigmentary disorders. On physical examination uniform grayish blue patches were seen over the torso and upper extremities. The patches seemed to follow a dermatomal distribution along the trunk and extremities (Figure 1). Several well-circumscribed, much darker blue macules were scattered within the bluish patches (Figure 2). The rest of the physical examination was unremarkable. Complete blood cell count and blood chemistry values were within reference range. Skin biopsy specimens from both the grayish blue patches and the conspicuous darker blue macules showed a fair amount of dermal bipolar dendritic pigmented melanocytes arranged parallel to the skin surface with no apparent disturbance of collagen bundles (Figure 3). No melanophages were seen. The clinical, histologic, and laboratory findings were consistent with a diagnosis of congenital dermal melanocytosis. An underlying lysosomal storage disease was ruled out through metabolic screening that included liver function tests, abdominal sonography, and neurologic and ophthalmologic examinations.

The clinical spectrum of congenital dermal melanocytosis includes several clinical entities such as mongolian spots, Ota nevus, Ito nevus, blue nevi, and DMH.¹ The differentiation between these different types of dermal melanocytosis can be challenging, especially when the process of melanocytosis is extensive. Among the several types of congenital dermal melanocytosis, Ota or Ito nevi can be ruled out in our patient based on the clinical presentation.

The term dermal melanocyte hamartoma was introduced by Burkhart and Gohara.² It is characterized by congenital dermal melanocytosis that follows a dermatomal pattern. The original case report included speckled, darker blue macules with a background of grayish blue patches similar to our patient.² Melanocytes in DMH typically are located in the upper half of the reticular dermis, as opposed to extensive mongolian spots in which ectopic melanocytes usually are found in the lower half of the dermis. The pigmentation in our patient did not change during 22 months of follow-up, which is more consistent with the diagnosis of DMH versus extensive mongolian spots. Our patient did not present with any systemic anomalies. However, a case of congenital melanocytosis and neuroectodermal malformation has been described in the literature.³

Little is known about the etiology of dermal melanocytosis. Mutations in the guanine nucleotide binding protein q polypeptide gene, GNAQ, or guanine nucleotide binding protein alpha 11 gene, GNA11, were found to cause dermal melanocytosis in mice.⁴ Also, GNAQ mutations have been demonstrated in humans with dermal melanocytosis as well as uveal melanoma.⁵ Progress in our understanding of the pathogenesis of dermal melanocytosis is expected to lead to a more accurate classification of dermal pigmentation disorders.

To the Editor:
Dermal melanocytosis is thought to be the result of a defect in melanoblast migration during embryogenesis and is characterized by the presence of functional fusiform and dendritic melanocytes in the dermis. Congenital dermal melanocytosis is classified into various subtypes based on the distribution, morphology, natural history of lesions, and distinctive histologic findings. We present an unusual case of congenital dermal melanocytosis that might fit the rare entity of dermal melanocyte hamartoma (DMH).

Figure 1. Uniform grayish blue patches over the torso with sharp demarcation lines that followed a dermatomal distribution.

Figure 2. Three conspicuous darker blue macules (arrows) within a bluish patch.

Figure 3. Intradermal dendritic pigmented melanocytes localized in the upper dermis and arranged parallel to the skin surface (H&E, original magnification ×100 [inset, original magnification ×400]).

A 4-month-old girl presented with bilateral bluish patches over the trunk and upper extremities. The lesions were present since birth, and remained entirely unchanged during a follow-up period of 18 months. Mental and physical development was normal. There was no family history of pigmentary disorders. On physical examination uniform grayish blue patches were seen over the torso and upper extremities. The patches seemed to follow a dermatomal distribution along the trunk and extremities (Figure 1). Several well-circumscribed, much darker blue macules were scattered within the bluish patches (Figure 2). The rest of the physical examination was unremarkable. Complete blood cell count and blood chemistry values were within reference range. Skin biopsy specimens from both the grayish blue patches and the conspicuous darker blue macules showed a fair amount of dermal bipolar dendritic pigmented melanocytes arranged parallel to the skin surface with no apparent disturbance of collagen bundles (Figure 3). No melanophages were seen. The clinical, histologic, and laboratory findings were consistent with a diagnosis of congenital dermal melanocytosis. An underlying lysosomal storage disease was ruled out through metabolic screening that included liver function tests, abdominal sonography, and neurologic and ophthalmologic examinations.

The clinical spectrum of congenital dermal melanocytosis includes several clinical entities such as mongolian spots, Ota nevus, Ito nevus, blue nevi, and DMH.¹ The differentiation between these different types of dermal melanocytosis can be challenging, especially when the process of melanocytosis is extensive. Among the several types of congenital dermal melanocytosis, Ota or Ito nevi can be ruled out in our patient based on the clinical presentation.

The term dermal melanocyte hamartoma was introduced by Burkhart and Gohara.² It is characterized by congenital dermal melanocytosis that follows a dermatomal pattern. The original case report included speckled, darker blue macules with a background of grayish blue patches similar to our patient.² Melanocytes in DMH typically are located in the upper half of the reticular dermis, as opposed to extensive mongolian spots in which ectopic melanocytes usually are found in the lower half of the dermis. The pigmentation in our patient did not change during 22 months of follow-up, which is more consistent with the diagnosis of DMH versus extensive mongolian spots. Our patient did not present with any systemic anomalies. However, a case of congenital melanocytosis and neuroectodermal malformation has been described in the literature.³

Little is known about the etiology of dermal melanocytosis. Mutations in the guanine nucleotide binding protein q polypeptide gene, GNAQ, or guanine nucleotide binding protein alpha 11 gene, GNA11, were found to cause dermal melanocytosis in mice.⁴ Also, GNAQ mutations have been demonstrated in humans with dermal melanocytosis as well as uveal melanoma.⁵ Progress in our understanding of the pathogenesis of dermal melanocytosis is expected to lead to a more accurate classification of dermal pigmentation disorders.

To the Editor:
Dermal melanocytosis is thought to be the result of a defect in melanoblast migration during embryogenesis and is characterized by the presence of functional fusiform and dendritic melanocytes in the dermis. Congenital dermal melanocytosis is classified into various subtypes based on the distribution, morphology, natural history of lesions, and distinctive histologic findings. We present an unusual case of congenital dermal melanocytosis that might fit the rare entity of dermal melanocyte hamartoma (DMH).

Figure 1. Uniform grayish blue patches over the torso with sharp demarcation lines that followed a dermatomal distribution.

Figure 2. Three conspicuous darker blue macules (arrows) within a bluish patch.

Figure 3. Intradermal dendritic pigmented melanocytes localized in the upper dermis and arranged parallel to the skin surface (H&E, original magnification ×100 [inset, original magnification ×400]).

A 4-month-old girl presented with bilateral bluish patches over the trunk and upper extremities. The lesions were present since birth, and remained entirely unchanged during a follow-up period of 18 months. Mental and physical development was normal. There was no family history of pigmentary disorders. On physical examination uniform grayish blue patches were seen over the torso and upper extremities. The patches seemed to follow a dermatomal distribution along the trunk and extremities (Figure 1). Several well-circumscribed, much darker blue macules were scattered within the bluish patches (Figure 2). The rest of the physical examination was unremarkable. Complete blood cell count and blood chemistry values were within reference range. Skin biopsy specimens from both the grayish blue patches and the conspicuous darker blue macules showed a fair amount of dermal bipolar dendritic pigmented melanocytes arranged parallel to the skin surface with no apparent disturbance of collagen bundles (Figure 3). No melanophages were seen. The clinical, histologic, and laboratory findings were consistent with a diagnosis of congenital dermal melanocytosis. An underlying lysosomal storage disease was ruled out through metabolic screening that included liver function tests, abdominal sonography, and neurologic and ophthalmologic examinations.

The clinical spectrum of congenital dermal melanocytosis includes several clinical entities such as mongolian spots, Ota nevus, Ito nevus, blue nevi, and DMH.¹ The differentiation between these different types of dermal melanocytosis can be challenging, especially when the process of melanocytosis is extensive. Among the several types of congenital dermal melanocytosis, Ota or Ito nevi can be ruled out in our patient based on the clinical presentation.

The term dermal melanocyte hamartoma was introduced by Burkhart and Gohara.² It is characterized by congenital dermal melanocytosis that follows a dermatomal pattern. The original case report included speckled, darker blue macules with a background of grayish blue patches similar to our patient.² Melanocytes in DMH typically are located in the upper half of the reticular dermis, as opposed to extensive mongolian spots in which ectopic melanocytes usually are found in the lower half of the dermis. The pigmentation in our patient did not change during 22 months of follow-up, which is more consistent with the diagnosis of DMH versus extensive mongolian spots. Our patient did not present with any systemic anomalies. However, a case of congenital melanocytosis and neuroectodermal malformation has been described in the literature.³

Little is known about the etiology of dermal melanocytosis. Mutations in the guanine nucleotide binding protein q polypeptide gene, GNAQ, or guanine nucleotide binding protein alpha 11 gene, GNA11, were found to cause dermal melanocytosis in mice.⁴ Also, GNAQ mutations have been demonstrated in humans with dermal melanocytosis as well as uveal melanoma.⁵ Progress in our understanding of the pathogenesis of dermal melanocytosis is expected to lead to a more accurate classification of dermal pigmentation disorders.

To the Editor:
Cutaneous neurofibromas are a clinical feature of both neurofibromatosis type I (NF-1) and neurofibromatosis type II (NF-2). Neurofibromatosis type I occurs in 1 in 3000 live births and NF-2 occurs in 1 in 40,000 live births.¹ Neurofibromas are discrete masses that arise from peripheral nerves and are composed of Schwann cells, mast cells, fibroblasts, and perineural cells. They commonly appear during or after puberty in the majority of patients and increase in number as patients age.^1,2 Cutaneous neurofibromas can be painful and pruritic as well as cosmetically unappealing. Current treatment of neurofibromas consists primarily of standard surgical excision or laser therapy. Reports of loop electrocoagulation in the operating room, combination erbium:YAG and CO₂ laser, and Nd:YAG laser treatments of neurofibromas have been published in the literature.^3-5 These treatments can be effective, but the bothersome lesions in patients with neurofibromatosis often are multiple, making these procedures time consuming and costly. A removal technique that is time efficient, cost effective, and poses little discomfort or functional impairment for the patient is desirable. We describe the technique of shave removal with electrodesiccation for multiple neurofibromas.

A 24-year-old woman with NF-1 presented to the dermatology clinic for treatment of several pruritic neurofibromas of the trunk, including the bilateral breasts (Figure, A). We performed shave removal of each lesion with electrodesiccation of the lesion base with successful treatment of symptoms and acceptable cosmetic outcome (Figure, B). The patient identified symptomatic and cosmetically bothersome neurofibromas. The lesions were disinfected with an alcohol swab and anesthetized with 1% lidocaine and a 1:200,000 dilution of epinephrine. A shave biopsy blade was used to remove each neurofibroma at the level of the surrounding skin. Toothed forceps were utilized to lift the lesion and provide traction. Electrodesiccation was then used for destruction of the base or any residual gelatinous component of the lesion to the level of the surrounding skin, thus achieving hemostasis. Lastly, petrolatum was applied to the wound and covered with an adhesive dressing. Daily dressing change was performed for 2 weeks or until healed.

A patient with neurofibromatosis type I with neurofibromas on the breast before (A) and approximately 6 months after treatment with shave removal plus electrodesiccation of the lesions (B).

Patients with neurofibromatoses often present to dermatologists for management of symptomatic or cosmetically bothersome neurofibromas, which are often numerous and diffusely distributed. There are few published practical descriptions of removal techniques to manage these multiple and recurrent manifestations of the disease. The shave removal plus electrodesiccation technique we described is straightforward for practitioners and provides a well-tolerated mechanism for treating multiple lesions at once, while also offering patients acceptable functional and cosmetic results.

To the Editor:
Cutaneous neurofibromas are a clinical feature of both neurofibromatosis type I (NF-1) and neurofibromatosis type II (NF-2). Neurofibromatosis type I occurs in 1 in 3000 live births and NF-2 occurs in 1 in 40,000 live births.¹ Neurofibromas are discrete masses that arise from peripheral nerves and are composed of Schwann cells, mast cells, fibroblasts, and perineural cells. They commonly appear during or after puberty in the majority of patients and increase in number as patients age.^1,2 Cutaneous neurofibromas can be painful and pruritic as well as cosmetically unappealing. Current treatment of neurofibromas consists primarily of standard surgical excision or laser therapy. Reports of loop electrocoagulation in the operating room, combination erbium:YAG and CO₂ laser, and Nd:YAG laser treatments of neurofibromas have been published in the literature.^3-5 These treatments can be effective, but the bothersome lesions in patients with neurofibromatosis often are multiple, making these procedures time consuming and costly. A removal technique that is time efficient, cost effective, and poses little discomfort or functional impairment for the patient is desirable. We describe the technique of shave removal with electrodesiccation for multiple neurofibromas.

A 24-year-old woman with NF-1 presented to the dermatology clinic for treatment of several pruritic neurofibromas of the trunk, including the bilateral breasts (Figure, A). We performed shave removal of each lesion with electrodesiccation of the lesion base with successful treatment of symptoms and acceptable cosmetic outcome (Figure, B). The patient identified symptomatic and cosmetically bothersome neurofibromas. The lesions were disinfected with an alcohol swab and anesthetized with 1% lidocaine and a 1:200,000 dilution of epinephrine. A shave biopsy blade was used to remove each neurofibroma at the level of the surrounding skin. Toothed forceps were utilized to lift the lesion and provide traction. Electrodesiccation was then used for destruction of the base or any residual gelatinous component of the lesion to the level of the surrounding skin, thus achieving hemostasis. Lastly, petrolatum was applied to the wound and covered with an adhesive dressing. Daily dressing change was performed for 2 weeks or until healed.

A patient with neurofibromatosis type I with neurofibromas on the breast before (A) and approximately 6 months after treatment with shave removal plus electrodesiccation of the lesions (B).

Patients with neurofibromatoses often present to dermatologists for management of symptomatic or cosmetically bothersome neurofibromas, which are often numerous and diffusely distributed. There are few published practical descriptions of removal techniques to manage these multiple and recurrent manifestations of the disease. The shave removal plus electrodesiccation technique we described is straightforward for practitioners and provides a well-tolerated mechanism for treating multiple lesions at once, while also offering patients acceptable functional and cosmetic results.

To the Editor:
Cutaneous neurofibromas are a clinical feature of both neurofibromatosis type I (NF-1) and neurofibromatosis type II (NF-2). Neurofibromatosis type I occurs in 1 in 3000 live births and NF-2 occurs in 1 in 40,000 live births.¹ Neurofibromas are discrete masses that arise from peripheral nerves and are composed of Schwann cells, mast cells, fibroblasts, and perineural cells. They commonly appear during or after puberty in the majority of patients and increase in number as patients age.^1,2 Cutaneous neurofibromas can be painful and pruritic as well as cosmetically unappealing. Current treatment of neurofibromas consists primarily of standard surgical excision or laser therapy. Reports of loop electrocoagulation in the operating room, combination erbium:YAG and CO₂ laser, and Nd:YAG laser treatments of neurofibromas have been published in the literature.^3-5 These treatments can be effective, but the bothersome lesions in patients with neurofibromatosis often are multiple, making these procedures time consuming and costly. A removal technique that is time efficient, cost effective, and poses little discomfort or functional impairment for the patient is desirable. We describe the technique of shave removal with electrodesiccation for multiple neurofibromas.

A 24-year-old woman with NF-1 presented to the dermatology clinic for treatment of several pruritic neurofibromas of the trunk, including the bilateral breasts (Figure, A). We performed shave removal of each lesion with electrodesiccation of the lesion base with successful treatment of symptoms and acceptable cosmetic outcome (Figure, B). The patient identified symptomatic and cosmetically bothersome neurofibromas. The lesions were disinfected with an alcohol swab and anesthetized with 1% lidocaine and a 1:200,000 dilution of epinephrine. A shave biopsy blade was used to remove each neurofibroma at the level of the surrounding skin. Toothed forceps were utilized to lift the lesion and provide traction. Electrodesiccation was then used for destruction of the base or any residual gelatinous component of the lesion to the level of the surrounding skin, thus achieving hemostasis. Lastly, petrolatum was applied to the wound and covered with an adhesive dressing. Daily dressing change was performed for 2 weeks or until healed.

A patient with neurofibromatosis type I with neurofibromas on the breast before (A) and approximately 6 months after treatment with shave removal plus electrodesiccation of the lesions (B).

Patients with neurofibromatoses often present to dermatologists for management of symptomatic or cosmetically bothersome neurofibromas, which are often numerous and diffusely distributed. There are few published practical descriptions of removal techniques to manage these multiple and recurrent manifestations of the disease. The shave removal plus electrodesiccation technique we described is straightforward for practitioners and provides a well-tolerated mechanism for treating multiple lesions at once, while also offering patients acceptable functional and cosmetic results.

To the Editor:
Primary malignant melanoma (PMM) of the penis is rare, comprising 1% of melanomas overall and less than 4% of malignancies in the male genitourinary tract.¹ However, regression of PMM is not rare. Melanoma is 6 times more likely to undergo regression compared to other malignancies.² Approximately 10% to 35% of cutaneous PMMs undergo partial regression, but only 42 cases of completely regressed cutaneous PMMs have been reported,^3,4 which may be due to underreporting of completely regressed cutaneous PMMs, as they often are clinically inconspicuous. Additionally, completely regressed cutaneous PMMs may be incorrectly reported as metastatic melanoma of unknown primary.⁵ Clinical characteristics of regression include pink coloration and a lightening or whitening of baseline lesional color. Dermatoscopic features of regression include white areas, blue areas, or vascular structures that translate microscopically to dermal fibrosis, melanophages, and telangiectases.⁵ We report a case of complete clinical regression of a nodular, mucosal, penile PMM with no evidence of metastatic disease.

An 86-year-old man presented with a progressively enlarging, pigmented lesion on the glans penis of 2 years’ duration. His medical history was notable for retinal detachment, macular degeneration, lumbar stenosis, and seizures postneurosurgery for a subdural hematoma. Physical examination revealed a healthy man with a mottled, black-brown, macular, and nodular lesion with irregular margins and irregular shape on the glans penis (Figure 1). No other similar skin lesions or lymphadenopathy were detectable. A lesional deep shave biopsy obtained at presentation demonstrated a nodular-type malignant melanoma with a Breslow thickness of approximately 3.5 mm (Figure 2).

Figure 1. Mottled, black-brown, macular, and nodular lesion with irregular margins and irregular shape on the glans penis.

Figure 2. Histologic section showed a nodular melanocytic proliferation with a dense sheetlike collection of melanocytes, predominantly in the dermis (H&E, original magnification ×20). Prominent cytologic atypia and multiple mitotic figures were consistent with melanoma (inset)(H&E, original magnification ×400).

Figure 3. High-power view of HMB-45 stain showed strong and diffuse staining, including several pagetoid intraepidermal melanocytes (original magnification ×200).

Figure 4. Skin examination 8 years following the initial primary malignant melanoma diagnosis showed no clinical evidence of recurrent or metastatic melanoma and almost complete loss of pigmentation at the prior melanoma site.

Histologic examination showed nodular nests of malignant melanocytes that were dispersed along the dermal-epidermal junction and coalesced into sheets within the dermis. Numerous dermal mitoses were present. The tumor was strongly and diffusely positive with Melan-A and HMB-45, which also highlighted scattered pagetoid intraepidermal cells (Figure 3). These findings were diagnostic of PMM of the mucocutaneous glans penis. The tumor was nonulcerated and invaded to a Breslow thickness of approximately 3.5 mm, corresponding to American Joint Committee on Cancer stage IIA (T3aN0M0) with an expected 5-year survival rate of 79%.⁶

He was referred to the urology department and was offered cystoscopy, urethrography, and phallectomy, which he refused. He also refused a trial of imiquimod. Computed tomography (CT) scans of his brain, chest, abdomen, and pelvis were negative for metastatic disease. Following the initial melanoma diagnosis, he had yearly dermatologic evaluations consisting of total-body skin and lymph node (LN) examinations. At 87 years of age (1 year following the initial diagnosis), the melanoma became dramatically smaller. At 88 years of age (2 years after diagnosis), the melanoma had near-complete clinical resolution. At 89 years of age, the patient reported asymmetric hearing loss. A cranial magnetic resonance imaging study showed no evidence of metastases.

At 92 years of age (6 years after the initial diagnosis), the patient reported bilateral leg pain. A CT scan of the lumbar spine showed no evidence of metastasis. He also reported abdominal pain. A CT scan of the abdomen and pelvis revealed an ileocecal mass. Biopsy of the ileocecal mass showed moderately differentiated invasive adenocarcinoma and no evidence of metastatic melanoma. The adenocarcinoma was resected and he continues to do well. Skin and LN examination 8 years after the initial diagnosis showed no clinical evidence of recurrent penile mucosal melanoma or metastatic melanoma (Figure 4). The PMM appeared to have clinically regressed spontaneously. He refused repeat skin biopsy and additional imaging studies.

The criteria for complete melanoma regression were initially described in 1965⁷ and revised in 2005.² Although our patient demonstrated complete clinical regression of his PMM, he did not meet the revised criteria for complete regression because there was no histopathologic confirmation of regression or of the absence of melanoma as well as no lymphatic involvement. It is extremely difficult to quantify the percentage of PMMs that completely regress. A case of a completely regressed untreated PMM with no metastatic disease 4 years after diagnosis has been reported. This case involved a nonulcerated melanoma with a Breslow thickness of 0.7 mm (American Joint Committee on Cancer stage IA).⁴ The prognosis of penile mucosal PMM is comparable to that of cutaneous PMM with a similar Breslow thickness.¹

The prognostic significance of melanoma regression is controversial. Regression may be mediated by host immunity, apoptosis, and/or antiangiogenesis. The lymphocytic infiltrate in regressive melanomas consists of cytotoxic T cells with selective antitumor activity, which induces HLA class I–restricted melanoma lysis.⁸ Lymph node migration may result in T-lymphocyte priming and induction of antitumor immunity.⁹ Therefore, regression may indicate risk for sentinel LN metastasis.

It is possible that complete regression of melanoma does not truly exist, and late recurrence due to cancer dormancy is inevitable. Late recurrence is defined as first metastasis 10 years after complete removal of the PMM.¹⁰ Our patient has only been followed for 8 years, so this possibility cannot be entirely excluded.

To the Editor:
Primary malignant melanoma (PMM) of the penis is rare, comprising 1% of melanomas overall and less than 4% of malignancies in the male genitourinary tract.¹ However, regression of PMM is not rare. Melanoma is 6 times more likely to undergo regression compared to other malignancies.² Approximately 10% to 35% of cutaneous PMMs undergo partial regression, but only 42 cases of completely regressed cutaneous PMMs have been reported,^3,4 which may be due to underreporting of completely regressed cutaneous PMMs, as they often are clinically inconspicuous. Additionally, completely regressed cutaneous PMMs may be incorrectly reported as metastatic melanoma of unknown primary.⁵ Clinical characteristics of regression include pink coloration and a lightening or whitening of baseline lesional color. Dermatoscopic features of regression include white areas, blue areas, or vascular structures that translate microscopically to dermal fibrosis, melanophages, and telangiectases.⁵ We report a case of complete clinical regression of a nodular, mucosal, penile PMM with no evidence of metastatic disease.

An 86-year-old man presented with a progressively enlarging, pigmented lesion on the glans penis of 2 years’ duration. His medical history was notable for retinal detachment, macular degeneration, lumbar stenosis, and seizures postneurosurgery for a subdural hematoma. Physical examination revealed a healthy man with a mottled, black-brown, macular, and nodular lesion with irregular margins and irregular shape on the glans penis (Figure 1). No other similar skin lesions or lymphadenopathy were detectable. A lesional deep shave biopsy obtained at presentation demonstrated a nodular-type malignant melanoma with a Breslow thickness of approximately 3.5 mm (Figure 2).

Figure 1. Mottled, black-brown, macular, and nodular lesion with irregular margins and irregular shape on the glans penis.

Figure 2. Histologic section showed a nodular melanocytic proliferation with a dense sheetlike collection of melanocytes, predominantly in the dermis (H&E, original magnification ×20). Prominent cytologic atypia and multiple mitotic figures were consistent with melanoma (inset)(H&E, original magnification ×400).

Figure 3. High-power view of HMB-45 stain showed strong and diffuse staining, including several pagetoid intraepidermal melanocytes (original magnification ×200).

Figure 4. Skin examination 8 years following the initial primary malignant melanoma diagnosis showed no clinical evidence of recurrent or metastatic melanoma and almost complete loss of pigmentation at the prior melanoma site.

Histologic examination showed nodular nests of malignant melanocytes that were dispersed along the dermal-epidermal junction and coalesced into sheets within the dermis. Numerous dermal mitoses were present. The tumor was strongly and diffusely positive with Melan-A and HMB-45, which also highlighted scattered pagetoid intraepidermal cells (Figure 3). These findings were diagnostic of PMM of the mucocutaneous glans penis. The tumor was nonulcerated and invaded to a Breslow thickness of approximately 3.5 mm, corresponding to American Joint Committee on Cancer stage IIA (T3aN0M0) with an expected 5-year survival rate of 79%.⁶

He was referred to the urology department and was offered cystoscopy, urethrography, and phallectomy, which he refused. He also refused a trial of imiquimod. Computed tomography (CT) scans of his brain, chest, abdomen, and pelvis were negative for metastatic disease. Following the initial melanoma diagnosis, he had yearly dermatologic evaluations consisting of total-body skin and lymph node (LN) examinations. At 87 years of age (1 year following the initial diagnosis), the melanoma became dramatically smaller. At 88 years of age (2 years after diagnosis), the melanoma had near-complete clinical resolution. At 89 years of age, the patient reported asymmetric hearing loss. A cranial magnetic resonance imaging study showed no evidence of metastases.

At 92 years of age (6 years after the initial diagnosis), the patient reported bilateral leg pain. A CT scan of the lumbar spine showed no evidence of metastasis. He also reported abdominal pain. A CT scan of the abdomen and pelvis revealed an ileocecal mass. Biopsy of the ileocecal mass showed moderately differentiated invasive adenocarcinoma and no evidence of metastatic melanoma. The adenocarcinoma was resected and he continues to do well. Skin and LN examination 8 years after the initial diagnosis showed no clinical evidence of recurrent penile mucosal melanoma or metastatic melanoma (Figure 4). The PMM appeared to have clinically regressed spontaneously. He refused repeat skin biopsy and additional imaging studies.

The criteria for complete melanoma regression were initially described in 1965⁷ and revised in 2005.² Although our patient demonstrated complete clinical regression of his PMM, he did not meet the revised criteria for complete regression because there was no histopathologic confirmation of regression or of the absence of melanoma as well as no lymphatic involvement. It is extremely difficult to quantify the percentage of PMMs that completely regress. A case of a completely regressed untreated PMM with no metastatic disease 4 years after diagnosis has been reported. This case involved a nonulcerated melanoma with a Breslow thickness of 0.7 mm (American Joint Committee on Cancer stage IA).⁴ The prognosis of penile mucosal PMM is comparable to that of cutaneous PMM with a similar Breslow thickness.¹

The prognostic significance of melanoma regression is controversial. Regression may be mediated by host immunity, apoptosis, and/or antiangiogenesis. The lymphocytic infiltrate in regressive melanomas consists of cytotoxic T cells with selective antitumor activity, which induces HLA class I–restricted melanoma lysis.⁸ Lymph node migration may result in T-lymphocyte priming and induction of antitumor immunity.⁹ Therefore, regression may indicate risk for sentinel LN metastasis.

It is possible that complete regression of melanoma does not truly exist, and late recurrence due to cancer dormancy is inevitable. Late recurrence is defined as first metastasis 10 years after complete removal of the PMM.¹⁰ Our patient has only been followed for 8 years, so this possibility cannot be entirely excluded.

To the Editor:
Primary malignant melanoma (PMM) of the penis is rare, comprising 1% of melanomas overall and less than 4% of malignancies in the male genitourinary tract.¹ However, regression of PMM is not rare. Melanoma is 6 times more likely to undergo regression compared to other malignancies.² Approximately 10% to 35% of cutaneous PMMs undergo partial regression, but only 42 cases of completely regressed cutaneous PMMs have been reported,^3,4 which may be due to underreporting of completely regressed cutaneous PMMs, as they often are clinically inconspicuous. Additionally, completely regressed cutaneous PMMs may be incorrectly reported as metastatic melanoma of unknown primary.⁵ Clinical characteristics of regression include pink coloration and a lightening or whitening of baseline lesional color. Dermatoscopic features of regression include white areas, blue areas, or vascular structures that translate microscopically to dermal fibrosis, melanophages, and telangiectases.⁵ We report a case of complete clinical regression of a nodular, mucosal, penile PMM with no evidence of metastatic disease.

An 86-year-old man presented with a progressively enlarging, pigmented lesion on the glans penis of 2 years’ duration. His medical history was notable for retinal detachment, macular degeneration, lumbar stenosis, and seizures postneurosurgery for a subdural hematoma. Physical examination revealed a healthy man with a mottled, black-brown, macular, and nodular lesion with irregular margins and irregular shape on the glans penis (Figure 1). No other similar skin lesions or lymphadenopathy were detectable. A lesional deep shave biopsy obtained at presentation demonstrated a nodular-type malignant melanoma with a Breslow thickness of approximately 3.5 mm (Figure 2).

Figure 1. Mottled, black-brown, macular, and nodular lesion with irregular margins and irregular shape on the glans penis.

Figure 2. Histologic section showed a nodular melanocytic proliferation with a dense sheetlike collection of melanocytes, predominantly in the dermis (H&E, original magnification ×20). Prominent cytologic atypia and multiple mitotic figures were consistent with melanoma (inset)(H&E, original magnification ×400).

Figure 3. High-power view of HMB-45 stain showed strong and diffuse staining, including several pagetoid intraepidermal melanocytes (original magnification ×200).

Figure 4. Skin examination 8 years following the initial primary malignant melanoma diagnosis showed no clinical evidence of recurrent or metastatic melanoma and almost complete loss of pigmentation at the prior melanoma site.

Histologic examination showed nodular nests of malignant melanocytes that were dispersed along the dermal-epidermal junction and coalesced into sheets within the dermis. Numerous dermal mitoses were present. The tumor was strongly and diffusely positive with Melan-A and HMB-45, which also highlighted scattered pagetoid intraepidermal cells (Figure 3). These findings were diagnostic of PMM of the mucocutaneous glans penis. The tumor was nonulcerated and invaded to a Breslow thickness of approximately 3.5 mm, corresponding to American Joint Committee on Cancer stage IIA (T3aN0M0) with an expected 5-year survival rate of 79%.⁶

He was referred to the urology department and was offered cystoscopy, urethrography, and phallectomy, which he refused. He also refused a trial of imiquimod. Computed tomography (CT) scans of his brain, chest, abdomen, and pelvis were negative for metastatic disease. Following the initial melanoma diagnosis, he had yearly dermatologic evaluations consisting of total-body skin and lymph node (LN) examinations. At 87 years of age (1 year following the initial diagnosis), the melanoma became dramatically smaller. At 88 years of age (2 years after diagnosis), the melanoma had near-complete clinical resolution. At 89 years of age, the patient reported asymmetric hearing loss. A cranial magnetic resonance imaging study showed no evidence of metastases.

At 92 years of age (6 years after the initial diagnosis), the patient reported bilateral leg pain. A CT scan of the lumbar spine showed no evidence of metastasis. He also reported abdominal pain. A CT scan of the abdomen and pelvis revealed an ileocecal mass. Biopsy of the ileocecal mass showed moderately differentiated invasive adenocarcinoma and no evidence of metastatic melanoma. The adenocarcinoma was resected and he continues to do well. Skin and LN examination 8 years after the initial diagnosis showed no clinical evidence of recurrent penile mucosal melanoma or metastatic melanoma (Figure 4). The PMM appeared to have clinically regressed spontaneously. He refused repeat skin biopsy and additional imaging studies.

The criteria for complete melanoma regression were initially described in 1965⁷ and revised in 2005.² Although our patient demonstrated complete clinical regression of his PMM, he did not meet the revised criteria for complete regression because there was no histopathologic confirmation of regression or of the absence of melanoma as well as no lymphatic involvement. It is extremely difficult to quantify the percentage of PMMs that completely regress. A case of a completely regressed untreated PMM with no metastatic disease 4 years after diagnosis has been reported. This case involved a nonulcerated melanoma with a Breslow thickness of 0.7 mm (American Joint Committee on Cancer stage IA).⁴ The prognosis of penile mucosal PMM is comparable to that of cutaneous PMM with a similar Breslow thickness.¹

The prognostic significance of melanoma regression is controversial. Regression may be mediated by host immunity, apoptosis, and/or antiangiogenesis. The lymphocytic infiltrate in regressive melanomas consists of cytotoxic T cells with selective antitumor activity, which induces HLA class I–restricted melanoma lysis.⁸ Lymph node migration may result in T-lymphocyte priming and induction of antitumor immunity.⁹ Therefore, regression may indicate risk for sentinel LN metastasis.

It is possible that complete regression of melanoma does not truly exist, and late recurrence due to cancer dormancy is inevitable. Late recurrence is defined as first metastasis 10 years after complete removal of the PMM.¹⁰ Our patient has only been followed for 8 years, so this possibility cannot be entirely excluded.

To the Editor:
A 78-year-old man presented with erythematous circular skin papules that were widely scattered over the trunk. He denied recent contact with ill individuals and denied any systemic symptoms indicating internal involvement or malignancy leading to possible paraneoplastic presentation. Physical examination showed erythematous, circular, slightly elevated plaques of varying sizes scattered over the trunk (Figure 1) and right axilla.

Figure 1. Nontender, erythematous, brown nodules scattered over the trunk.

Figure 2. Light microscopy revealed Langerhans cells filling the superficial dermis, abutting the epidermis, and extending into the deep dermis with surrounding inflammatory infiltrates (CD1a, original magnification ×100).

Figure 3. Langerhans cells appeared strongly positive for CD1a (original magnification ×400).

Biopsies of lesions were taken and stained with immunoperoxidase. On light microscopy there was a reticular and papillary dermal dense infiltrate of cells with indented nuclei (Figure 2). At higher magnification, cells appeared strongly positive for CD1a (Figure 3) and S-100 protein, which was histologically consistent with adult-type Langerhans cell histiocytosis (ALCH).

Computed tomography of the head, chest, abdomen, and pelvis were ordered to rule out spread of ALCH to other organ sites. Results were clear of evidence of systemic spread. Additionally, a complete blood cell count and comprehensive metabolic panel were within reference range.

He was started on topical tacrolimus; however, most of the lesions resolved on their own. As a result, tacrolimus was discontinued due to its propensity to cause skin irritation and lack of change in disease progression. At 3-month follow-up, he was prescribed triamcinolone acetonide cream 0.1% for minor outbreaks. After 2 years, he was completely clear of all skin signs of ALCH.

Adult-type Langerhans cell histiocytosis is characterized as a group of disorders associated with abnormal spread and proliferation of dendritic cells of the epidermis. The disease primarily affects children aged 1 to 4 years. It is estimated that only 1 to 2 cases of ALCH per million occur.¹ The pathophysiology of ALCH is unknown; it is speculated that it may be associated with a reactive inflammatory process triggered by proliferation of Langerhans-type dendritic cells. It is possible that the release of multiple cytokines by dendritic cells and T cells in ALCH lesions leads to erythematous eruptions and can contribute to spontaneous remission of the disorder.² Various cases of ALCH have reported high serum levels of IL-17 and IL-10 proinflammatory cytokines, supporting the theory of an inflammatory etiology of ALCH.³

Comparative genomic hybridization with loss of heterozygosity of pulmonary lesions has provided further evidence to suggest that chromosomal aberrations also may contribute to the pathophysiology of ALCH.⁴ One study evaluated 14 cases of pulmonary ALCH for loss of heterozygosity and found allelic loss of 1p, 1q, 3p, 5p, 17p, and 22q.⁵ In addition, allelic loss of 1 or more tumor suppressor genes was identified in 19 of 24 specimens, suggesting a neoplastic type of pathology through uncontrolled cellular proliferation.⁶

Lesions of ALCH can be broad but typically present as red-brown maculopapular lesions with petechiae that erupt over the trunk, axilla, and perivulvar or retroauricular regions.⁷ The papules may unify to form an erythematous, weeping, or crusted eruption that appears similar to seborrheic dermatitis. Typically the lesions remit on their own; however, lesions can recur with the same or decreased severity as the primary eruption. Complications have been noted with lesions, particularly secondary infection and ulceration.⁷

Systemic involvement has been noted in adults, particularly in the lungs. Patients typically present with chronic cough, dyspnea, and chest pain with evidence of a solitary nodular lesion on radiologic testing. In addition, bone involvement has been noted as eosinophilic granulomas that can produce osteolytic lesions that lead to spontaneous fractures. Use of corticosteroids and immunosuppressive agents, as opposed to just observation, is warranted in cases of systemic involvement, according to the National Cancer Institute.⁷

Exact treatment modalities have not yet been elucidated due to the ambiguity of pathogenesis. In addition, ALCH is known to remit and relapse in patients, which increases the difficulty in evaluating the efficacy of particular treatments. Trials conducted by the Histiocyte Society have shown that treatment regimens should be tailored to disease severity. Epidermal involvement of ALCH typically responds to corticosteroid creams, whereas patients with systemic involvement respond well to strong chemotherapeutic agents such as vincristine and prednisone with mercaptopurine.⁸ However, as demonstrated in our case, lesions may remit on their own and use of corticosteroids and immunosuppressive agents may lead to further detriment without treating disease progression.

Because of a low prevalence among adults, ALCH is difficult to recognize and diagnose, and the uncertainty of the pathogenesis of ALCH limits treatment alternatives. Further study into proper treatment modalities is warranted given that the remitting and relapsing course of the disease and cosmetic quandaries are detrimental to patient well-being. Our case illustrates that it is appropriate to simply monitor lesions for cases limited to cutaneous involvement. Systemic agents may be used when there are signs of organ involvement outside the skin, but providers must proceed to do so with caution.

To the Editor:
A 78-year-old man presented with erythematous circular skin papules that were widely scattered over the trunk. He denied recent contact with ill individuals and denied any systemic symptoms indicating internal involvement or malignancy leading to possible paraneoplastic presentation. Physical examination showed erythematous, circular, slightly elevated plaques of varying sizes scattered over the trunk (Figure 1) and right axilla.

Figure 1. Nontender, erythematous, brown nodules scattered over the trunk.

Figure 2. Light microscopy revealed Langerhans cells filling the superficial dermis, abutting the epidermis, and extending into the deep dermis with surrounding inflammatory infiltrates (CD1a, original magnification ×100).

Figure 3. Langerhans cells appeared strongly positive for CD1a (original magnification ×400).

Biopsies of lesions were taken and stained with immunoperoxidase. On light microscopy there was a reticular and papillary dermal dense infiltrate of cells with indented nuclei (Figure 2). At higher magnification, cells appeared strongly positive for CD1a (Figure 3) and S-100 protein, which was histologically consistent with adult-type Langerhans cell histiocytosis (ALCH).

Computed tomography of the head, chest, abdomen, and pelvis were ordered to rule out spread of ALCH to other organ sites. Results were clear of evidence of systemic spread. Additionally, a complete blood cell count and comprehensive metabolic panel were within reference range.

He was started on topical tacrolimus; however, most of the lesions resolved on their own. As a result, tacrolimus was discontinued due to its propensity to cause skin irritation and lack of change in disease progression. At 3-month follow-up, he was prescribed triamcinolone acetonide cream 0.1% for minor outbreaks. After 2 years, he was completely clear of all skin signs of ALCH.

Adult-type Langerhans cell histiocytosis is characterized as a group of disorders associated with abnormal spread and proliferation of dendritic cells of the epidermis. The disease primarily affects children aged 1 to 4 years. It is estimated that only 1 to 2 cases of ALCH per million occur.¹ The pathophysiology of ALCH is unknown; it is speculated that it may be associated with a reactive inflammatory process triggered by proliferation of Langerhans-type dendritic cells. It is possible that the release of multiple cytokines by dendritic cells and T cells in ALCH lesions leads to erythematous eruptions and can contribute to spontaneous remission of the disorder.² Various cases of ALCH have reported high serum levels of IL-17 and IL-10 proinflammatory cytokines, supporting the theory of an inflammatory etiology of ALCH.³

Comparative genomic hybridization with loss of heterozygosity of pulmonary lesions has provided further evidence to suggest that chromosomal aberrations also may contribute to the pathophysiology of ALCH.⁴ One study evaluated 14 cases of pulmonary ALCH for loss of heterozygosity and found allelic loss of 1p, 1q, 3p, 5p, 17p, and 22q.⁵ In addition, allelic loss of 1 or more tumor suppressor genes was identified in 19 of 24 specimens, suggesting a neoplastic type of pathology through uncontrolled cellular proliferation.⁶

Lesions of ALCH can be broad but typically present as red-brown maculopapular lesions with petechiae that erupt over the trunk, axilla, and perivulvar or retroauricular regions.⁷ The papules may unify to form an erythematous, weeping, or crusted eruption that appears similar to seborrheic dermatitis. Typically the lesions remit on their own; however, lesions can recur with the same or decreased severity as the primary eruption. Complications have been noted with lesions, particularly secondary infection and ulceration.⁷

Systemic involvement has been noted in adults, particularly in the lungs. Patients typically present with chronic cough, dyspnea, and chest pain with evidence of a solitary nodular lesion on radiologic testing. In addition, bone involvement has been noted as eosinophilic granulomas that can produce osteolytic lesions that lead to spontaneous fractures. Use of corticosteroids and immunosuppressive agents, as opposed to just observation, is warranted in cases of systemic involvement, according to the National Cancer Institute.⁷

Exact treatment modalities have not yet been elucidated due to the ambiguity of pathogenesis. In addition, ALCH is known to remit and relapse in patients, which increases the difficulty in evaluating the efficacy of particular treatments. Trials conducted by the Histiocyte Society have shown that treatment regimens should be tailored to disease severity. Epidermal involvement of ALCH typically responds to corticosteroid creams, whereas patients with systemic involvement respond well to strong chemotherapeutic agents such as vincristine and prednisone with mercaptopurine.⁸ However, as demonstrated in our case, lesions may remit on their own and use of corticosteroids and immunosuppressive agents may lead to further detriment without treating disease progression.

Because of a low prevalence among adults, ALCH is difficult to recognize and diagnose, and the uncertainty of the pathogenesis of ALCH limits treatment alternatives. Further study into proper treatment modalities is warranted given that the remitting and relapsing course of the disease and cosmetic quandaries are detrimental to patient well-being. Our case illustrates that it is appropriate to simply monitor lesions for cases limited to cutaneous involvement. Systemic agents may be used when there are signs of organ involvement outside the skin, but providers must proceed to do so with caution.

To the Editor:
A 78-year-old man presented with erythematous circular skin papules that were widely scattered over the trunk. He denied recent contact with ill individuals and denied any systemic symptoms indicating internal involvement or malignancy leading to possible paraneoplastic presentation. Physical examination showed erythematous, circular, slightly elevated plaques of varying sizes scattered over the trunk (Figure 1) and right axilla.

Figure 1. Nontender, erythematous, brown nodules scattered over the trunk.

Figure 2. Light microscopy revealed Langerhans cells filling the superficial dermis, abutting the epidermis, and extending into the deep dermis with surrounding inflammatory infiltrates (CD1a, original magnification ×100).

Figure 3. Langerhans cells appeared strongly positive for CD1a (original magnification ×400).

Biopsies of lesions were taken and stained with immunoperoxidase. On light microscopy there was a reticular and papillary dermal dense infiltrate of cells with indented nuclei (Figure 2). At higher magnification, cells appeared strongly positive for CD1a (Figure 3) and S-100 protein, which was histologically consistent with adult-type Langerhans cell histiocytosis (ALCH).

Computed tomography of the head, chest, abdomen, and pelvis were ordered to rule out spread of ALCH to other organ sites. Results were clear of evidence of systemic spread. Additionally, a complete blood cell count and comprehensive metabolic panel were within reference range.

He was started on topical tacrolimus; however, most of the lesions resolved on their own. As a result, tacrolimus was discontinued due to its propensity to cause skin irritation and lack of change in disease progression. At 3-month follow-up, he was prescribed triamcinolone acetonide cream 0.1% for minor outbreaks. After 2 years, he was completely clear of all skin signs of ALCH.

Adult-type Langerhans cell histiocytosis is characterized as a group of disorders associated with abnormal spread and proliferation of dendritic cells of the epidermis. The disease primarily affects children aged 1 to 4 years. It is estimated that only 1 to 2 cases of ALCH per million occur.¹ The pathophysiology of ALCH is unknown; it is speculated that it may be associated with a reactive inflammatory process triggered by proliferation of Langerhans-type dendritic cells. It is possible that the release of multiple cytokines by dendritic cells and T cells in ALCH lesions leads to erythematous eruptions and can contribute to spontaneous remission of the disorder.² Various cases of ALCH have reported high serum levels of IL-17 and IL-10 proinflammatory cytokines, supporting the theory of an inflammatory etiology of ALCH.³

Comparative genomic hybridization with loss of heterozygosity of pulmonary lesions has provided further evidence to suggest that chromosomal aberrations also may contribute to the pathophysiology of ALCH.⁴ One study evaluated 14 cases of pulmonary ALCH for loss of heterozygosity and found allelic loss of 1p, 1q, 3p, 5p, 17p, and 22q.⁵ In addition, allelic loss of 1 or more tumor suppressor genes was identified in 19 of 24 specimens, suggesting a neoplastic type of pathology through uncontrolled cellular proliferation.⁶

Lesions of ALCH can be broad but typically present as red-brown maculopapular lesions with petechiae that erupt over the trunk, axilla, and perivulvar or retroauricular regions.⁷ The papules may unify to form an erythematous, weeping, or crusted eruption that appears similar to seborrheic dermatitis. Typically the lesions remit on their own; however, lesions can recur with the same or decreased severity as the primary eruption. Complications have been noted with lesions, particularly secondary infection and ulceration.⁷

Systemic involvement has been noted in adults, particularly in the lungs. Patients typically present with chronic cough, dyspnea, and chest pain with evidence of a solitary nodular lesion on radiologic testing. In addition, bone involvement has been noted as eosinophilic granulomas that can produce osteolytic lesions that lead to spontaneous fractures. Use of corticosteroids and immunosuppressive agents, as opposed to just observation, is warranted in cases of systemic involvement, according to the National Cancer Institute.⁷

Exact treatment modalities have not yet been elucidated due to the ambiguity of pathogenesis. In addition, ALCH is known to remit and relapse in patients, which increases the difficulty in evaluating the efficacy of particular treatments. Trials conducted by the Histiocyte Society have shown that treatment regimens should be tailored to disease severity. Epidermal involvement of ALCH typically responds to corticosteroid creams, whereas patients with systemic involvement respond well to strong chemotherapeutic agents such as vincristine and prednisone with mercaptopurine.⁸ However, as demonstrated in our case, lesions may remit on their own and use of corticosteroids and immunosuppressive agents may lead to further detriment without treating disease progression.

Because of a low prevalence among adults, ALCH is difficult to recognize and diagnose, and the uncertainty of the pathogenesis of ALCH limits treatment alternatives. Further study into proper treatment modalities is warranted given that the remitting and relapsing course of the disease and cosmetic quandaries are detrimental to patient well-being. Our case illustrates that it is appropriate to simply monitor lesions for cases limited to cutaneous involvement. Systemic agents may be used when there are signs of organ involvement outside the skin, but providers must proceed to do so with caution.

To the Editor:
Longitudinal melanonychia encompasses a broad spectrum of diseases and often is a complex diagnostic problem. Differential diagnoses include ethnic-type nail pigmentation, which is more frequently seen in darker-skinned individuals; drug-induced pigmentation; subungual hemorrhage; fungal or bacterial infection; nevus; and melanoma.^1,2 Fungal melan-onychia is an uncommon presentation of onychomycosis. Dermoscopy can assist in the evaluation of nail pigmentation caused by fungi to avoid unnecessary nail biopsies.

A 39-year-old man visited the dermatology clinic with a concern for melanoma because of blackish pigmentation of the toenails of 1 month’s duration. He denied history of trauma and was not taking any medications. On physical examination the second and third toenails revealed a 2-mm longitudinal band of black pigment on the lateral side; the fifth toenail showed diffuse black pigment (Figure 1A). The nail plates were thickened. Dermoscopy revealed prominent subungual hyperkeratosis, a homogeneous brown-black band with wide yellow streaks that were wider in the distal ends, and some focal reddish hue. No visible melanin inclusions were observed (Figure 2). These findings were suggestive of fungal infection. Cultures from the diseased nail grew a fungus identified as Trichophyton rubrum. The patient was treated with itraconazole 200 mg daily for 3 months. Clinical cure with disappearance of pigment was obtained at 5-month follow-up (Figure 1B).

Figure 1. Blackish discoloration of the right second, third, and fifth toenails (A). Resolution of pigmentation and subungual hyperkeratosis was achieved at 5-month follow-up after treatment (B).

Figure 2. Top view (A) and front view (B) of a homogeneous brown-black band with subungual hyperkeratosis and wide intervening yellow streaks. Each ruler mark denotes 1 mm.

Our patient illustrates the value of dermoscopy in evaluating melanonychia. The pigmentation of adult-onset melanonychia involving multiple fingers can be divided into nonmelanocytic or melanocytic origin. Causes of the former include subungual hematoma, fungal or bacterial infection, and exogenous pigmentation. The nonmelanocytic pigment often is homogeneously distributed without melanin inclusions under the dermoscope.²

On the contrary, melanin inclusions can be detected as fine granules in pigmentation of melanocytic origin, either from focal melanocytic activation or melanocyte proliferation. Causes of focal melanocytic activation include ethnic-type nail hyperpigmentation; inflammatory nail diseases; or drug-, radiation-, and friction-induced hyperpigmentation. The characteristic dermoscopic features are thin longitudinal gray lines with regular thickness and spacing in a grayish background.^1-3

Melanocyte proliferation can result in a nevus or melanoma of the nail apparatus. Both share dermoscopic features of brown-black longitudinal lines in a brown background. However, the longitudinal lines in melanoma are irregular in coloration, spacing, thickness, and parallelism, in contrast with the regular pattern of a nevus.^1-4 Although patients often are concerned about melanoma, involvement of multiple fingers at the same time is less likely.

In our patient, the homogeneous deep brown color without melanin inclusions favored a nonmel-anocytic origin. The distally wider pigmentation suggested fungal infection because most ungual infections extend from the distal to the proximal part of the nail.^5,6 The focal reddish hue may be related with traumatic hemorrhage from subungual hyperkeratosis.

Cases of fungal melanonychia are being reported at an increasing rate. Some fungal strains are capable of synthesizing melanin, which is associated with virulence and acts as a fungal armor against toxic insults.⁵ In T rubrum, the melanoid variant, the diffusible black pigment infiltrates the nail plate and attributes to the black nail clinically.⁴ The most frequently isolated fungi in fungal melanonychia are T rubrum and Scytalidium dimidiatum⁶; however, Candida species,^7,8 dematiaceous fungus,⁹ and other dermatophytes such as Trichophyton soudanense¹⁰ have been reported to be the cause.⁵

Our patient presented with fungal melanonychia due to T rubrum with dermoscopic features. The prominent subungual hyperkeratosis, distally wider homogeneous brown-black pigmented band, and wide yellow streaks with focal reddish hue all suggested fungal melanonychia. The diagnosis was further confirmed by a good response to antifungal agents.

To the Editor:
Longitudinal melanonychia encompasses a broad spectrum of diseases and often is a complex diagnostic problem. Differential diagnoses include ethnic-type nail pigmentation, which is more frequently seen in darker-skinned individuals; drug-induced pigmentation; subungual hemorrhage; fungal or bacterial infection; nevus; and melanoma.^1,2 Fungal melan-onychia is an uncommon presentation of onychomycosis. Dermoscopy can assist in the evaluation of nail pigmentation caused by fungi to avoid unnecessary nail biopsies.

A 39-year-old man visited the dermatology clinic with a concern for melanoma because of blackish pigmentation of the toenails of 1 month’s duration. He denied history of trauma and was not taking any medications. On physical examination the second and third toenails revealed a 2-mm longitudinal band of black pigment on the lateral side; the fifth toenail showed diffuse black pigment (Figure 1A). The nail plates were thickened. Dermoscopy revealed prominent subungual hyperkeratosis, a homogeneous brown-black band with wide yellow streaks that were wider in the distal ends, and some focal reddish hue. No visible melanin inclusions were observed (Figure 2). These findings were suggestive of fungal infection. Cultures from the diseased nail grew a fungus identified as Trichophyton rubrum. The patient was treated with itraconazole 200 mg daily for 3 months. Clinical cure with disappearance of pigment was obtained at 5-month follow-up (Figure 1B).

Figure 1. Blackish discoloration of the right second, third, and fifth toenails (A). Resolution of pigmentation and subungual hyperkeratosis was achieved at 5-month follow-up after treatment (B).

Figure 2. Top view (A) and front view (B) of a homogeneous brown-black band with subungual hyperkeratosis and wide intervening yellow streaks. Each ruler mark denotes 1 mm.

Our patient illustrates the value of dermoscopy in evaluating melanonychia. The pigmentation of adult-onset melanonychia involving multiple fingers can be divided into nonmelanocytic or melanocytic origin. Causes of the former include subungual hematoma, fungal or bacterial infection, and exogenous pigmentation. The nonmelanocytic pigment often is homogeneously distributed without melanin inclusions under the dermoscope.²

On the contrary, melanin inclusions can be detected as fine granules in pigmentation of melanocytic origin, either from focal melanocytic activation or melanocyte proliferation. Causes of focal melanocytic activation include ethnic-type nail hyperpigmentation; inflammatory nail diseases; or drug-, radiation-, and friction-induced hyperpigmentation. The characteristic dermoscopic features are thin longitudinal gray lines with regular thickness and spacing in a grayish background.^1-3

Melanocyte proliferation can result in a nevus or melanoma of the nail apparatus. Both share dermoscopic features of brown-black longitudinal lines in a brown background. However, the longitudinal lines in melanoma are irregular in coloration, spacing, thickness, and parallelism, in contrast with the regular pattern of a nevus.^1-4 Although patients often are concerned about melanoma, involvement of multiple fingers at the same time is less likely.

In our patient, the homogeneous deep brown color without melanin inclusions favored a nonmel-anocytic origin. The distally wider pigmentation suggested fungal infection because most ungual infections extend from the distal to the proximal part of the nail.^5,6 The focal reddish hue may be related with traumatic hemorrhage from subungual hyperkeratosis.

Cases of fungal melanonychia are being reported at an increasing rate. Some fungal strains are capable of synthesizing melanin, which is associated with virulence and acts as a fungal armor against toxic insults.⁵ In T rubrum, the melanoid variant, the diffusible black pigment infiltrates the nail plate and attributes to the black nail clinically.⁴ The most frequently isolated fungi in fungal melanonychia are T rubrum and Scytalidium dimidiatum⁶; however, Candida species,^7,8 dematiaceous fungus,⁹ and other dermatophytes such as Trichophyton soudanense¹⁰ have been reported to be the cause.⁵

Our patient presented with fungal melanonychia due to T rubrum with dermoscopic features. The prominent subungual hyperkeratosis, distally wider homogeneous brown-black pigmented band, and wide yellow streaks with focal reddish hue all suggested fungal melanonychia. The diagnosis was further confirmed by a good response to antifungal agents.

To the Editor:
Longitudinal melanonychia encompasses a broad spectrum of diseases and often is a complex diagnostic problem. Differential diagnoses include ethnic-type nail pigmentation, which is more frequently seen in darker-skinned individuals; drug-induced pigmentation; subungual hemorrhage; fungal or bacterial infection; nevus; and melanoma.^1,2 Fungal melan-onychia is an uncommon presentation of onychomycosis. Dermoscopy can assist in the evaluation of nail pigmentation caused by fungi to avoid unnecessary nail biopsies.

A 39-year-old man visited the dermatology clinic with a concern for melanoma because of blackish pigmentation of the toenails of 1 month’s duration. He denied history of trauma and was not taking any medications. On physical examination the second and third toenails revealed a 2-mm longitudinal band of black pigment on the lateral side; the fifth toenail showed diffuse black pigment (Figure 1A). The nail plates were thickened. Dermoscopy revealed prominent subungual hyperkeratosis, a homogeneous brown-black band with wide yellow streaks that were wider in the distal ends, and some focal reddish hue. No visible melanin inclusions were observed (Figure 2). These findings were suggestive of fungal infection. Cultures from the diseased nail grew a fungus identified as Trichophyton rubrum. The patient was treated with itraconazole 200 mg daily for 3 months. Clinical cure with disappearance of pigment was obtained at 5-month follow-up (Figure 1B).

Figure 1. Blackish discoloration of the right second, third, and fifth toenails (A). Resolution of pigmentation and subungual hyperkeratosis was achieved at 5-month follow-up after treatment (B).

Figure 2. Top view (A) and front view (B) of a homogeneous brown-black band with subungual hyperkeratosis and wide intervening yellow streaks. Each ruler mark denotes 1 mm.

Our patient illustrates the value of dermoscopy in evaluating melanonychia. The pigmentation of adult-onset melanonychia involving multiple fingers can be divided into nonmelanocytic or melanocytic origin. Causes of the former include subungual hematoma, fungal or bacterial infection, and exogenous pigmentation. The nonmelanocytic pigment often is homogeneously distributed without melanin inclusions under the dermoscope.²

On the contrary, melanin inclusions can be detected as fine granules in pigmentation of melanocytic origin, either from focal melanocytic activation or melanocyte proliferation. Causes of focal melanocytic activation include ethnic-type nail hyperpigmentation; inflammatory nail diseases; or drug-, radiation-, and friction-induced hyperpigmentation. The characteristic dermoscopic features are thin longitudinal gray lines with regular thickness and spacing in a grayish background.^1-3

Melanocyte proliferation can result in a nevus or melanoma of the nail apparatus. Both share dermoscopic features of brown-black longitudinal lines in a brown background. However, the longitudinal lines in melanoma are irregular in coloration, spacing, thickness, and parallelism, in contrast with the regular pattern of a nevus.^1-4 Although patients often are concerned about melanoma, involvement of multiple fingers at the same time is less likely.

In our patient, the homogeneous deep brown color without melanin inclusions favored a nonmel-anocytic origin. The distally wider pigmentation suggested fungal infection because most ungual infections extend from the distal to the proximal part of the nail.^5,6 The focal reddish hue may be related with traumatic hemorrhage from subungual hyperkeratosis.

Cases of fungal melanonychia are being reported at an increasing rate. Some fungal strains are capable of synthesizing melanin, which is associated with virulence and acts as a fungal armor against toxic insults.⁵ In T rubrum, the melanoid variant, the diffusible black pigment infiltrates the nail plate and attributes to the black nail clinically.⁴ The most frequently isolated fungi in fungal melanonychia are T rubrum and Scytalidium dimidiatum⁶; however, Candida species,^7,8 dematiaceous fungus,⁹ and other dermatophytes such as Trichophyton soudanense¹⁰ have been reported to be the cause.⁵

Our patient presented with fungal melanonychia due to T rubrum with dermoscopic features. The prominent subungual hyperkeratosis, distally wider homogeneous brown-black pigmented band, and wide yellow streaks with focal reddish hue all suggested fungal melanonychia. The diagnosis was further confirmed by a good response to antifungal agents.