Faculty Bibliography

A 64-year-old man presented with a three-year history of an enlarging, pruritic, linear, verrucous plaque on his left lower extremity. Histopathologic examination was consistent with a verrucous epidermal nevus, which is a benign epidermal hamartoma, most commonly observed in the pediatric population. Verrucous epidermal nevi are often refractory to treatment and have high rates of recurrences, causing them to be therapeutic challenges. We review the treatment modalities reported to be effective in verrucous epidermal nevi.

Lichen planus pigmentosus (LPP) is an uncommon variant of lichen planus that tends to occur in middle-aged individuals with darker pigmented skin. Clinical findings include hyperpigmented, brown to gray-brown macules and patches in sun-exposed areas, typically on the head and neck. Histopathologic features include epidermal atrophy, vacuolar degeneration of the basal layer of the epidermis, perivascular lymphohistiocystic infiltrate in the upper dermis, and dermal melanophages. We present a unique case of LPP that was characterized by an atypical initial inflammatory phase and subsequent circinate lesions with central clearing.

BACKGROUND: Levamisole is present as a contaminant or additive in most cocaine sold in the United States. Cases of agranulocytosis attributed to levamisole-tainted cocaine have been widely described. A vasculopathic reaction to levamisole has also been reported; however, diagnostic features such as antineutrophil cytoplasmic antibody (ANCA) and additional autoimmune marker positivity are not well recognized. As such, many patients are given a misdiagnosis, prompting aggressive and often unnecessary treatment. OBJECTIVE: We hope to educate practitioners about the clinical and laboratory features of levamisole-induced vasculopathy to ensure accurate diagnosis and management. METHODS: This was a case series. RESULTS: Six patients were admitted with purpuric lesions and vasculitic changes on biopsy specimen; 5 of them were given the diagnosis of and treated for autoimmune conditions before their true diagnosis was revealed. All patients had ANCA positivity, and 4 had additional abnormalities in autoimmune markers. All patients reported recent cocaine abuse, and were ultimately given the diagnosis of levamisole-induced vasculopathy. LIMITATIONS: This observational study is limited by sample size. CONCLUSIONS: Patients presenting with purpuric lesions with ANCA positivity should be assessed for cocaine exposure. It is important to recognize that levamisole may not only induce ANCA positivity but also other autoimmune marker abnormalities. Patients can often be treated with less aggressive therapeutic strategies than what is used for primary ANCA-associated vasculitides.

Primary infection with varicella-zoster virus (VZV) during pregnancy can lead to devastating outcomes for both the mother and fetus. We describe a case of VZV infection in a pregnant woman who presented at 38 weeks' gestation. We also review the literature regarding management and prevention. Varicella-zoster virus-associated pneumonia in the mother is important to recognize. Outcomes in the newborn largely are dependent on gestational age at the time of infection. Prevention is paramount to management.

The specific dermatoses of pregnancy represent a diverse group of intensely pruritic dermatoses, occurring only in the puerperal state. The relative rarity of these conditions, the often variable clinical appearance, and the lack of definitive diagnostic tests have led to confusion regarding the appropriate diagnosis and management of the specific dermatoses of pregnancy. Herein we review the clinical characteristics, diagnosis and treatment of five dermatoses occurring during pregnancy: pruritic urticarial papules and plaques of pregnancy, atopic eruption of pregnancy, pemphigoid gestationis, intrahepatic cholestasis of pregnancy, and pustular psoriasis of pregnancy.

Multiple endocrine neoplasia type 1 (MEN1) is a familial tumor syndrome with autosomal dominant inheritance. Cutaneous tumors in MEN1, which include multiple angiofibromas, collagenomas, and lipomas can easily be overlooked because of their subtle appearance. As markers of this tumor syndrome, recognition of the mucocutaneous manifestations of MEN1 is important in order to facilitate early interdisciplinary care and diagnosis of associated internal disease in both patients and family members.

Disseminated superficial actinic porokeratosis, which was described in 1966, is characterized by small, atrophic patches with distinctive keratin rims that occur on sun-exposed areas of the extremities, shoulders, and back. The diagnosis is based on the histopathologic finding of a cornoid lamella, absence of a granular layer, and often a thin epidermis. It is associated with exposure to ultraviolet radiation. Gene studies suggest a pathway defect in which several mutations in keratinocyte proliferation and differentiation lead to development of porokeratosis.