Faculty Bibliography

Kikuchi-Fujimoto's disease (KFD) and adult-onset Still's disease (AOSD) are rare inflammatory conditions with some overlapping features. We encountered a 22-year-old male patient who presented with daily fevers, neck discomfort, and sore throat and subsequently developed rash, arthritis, and cervical lymphadenopathy. Biopsy of the skin rash was consistent with KFD skin involvement. Given that the patient also met criteria for AOSD, a final diagnosis of KFD/AOSD co-occurrence was made. Anti-IL-1beta therapy with anakinra resulted in rapid resolution of all symptoms. A literature search identified eight more cases of KFD/AOSD. Fever, rash, arthritis, and lymphadenopathy were present in all patients. No case report demonstrated an association of rash eruption clearly associated with fever spikes. Duration of symptoms ranged from 3 weeks to 10 years. Seven patients had leukocytosis, six had anemia, and five demonstrated elevated ferritin and/or decreased glycosylated ferritin. Seven patients had elevated erythrocyte sedimentation rate (ESR), and seven had transaminitis. Eight of nine patients had no evidence of infectious disease. Autoantibodies were absent from all patients. KFD and AOSD are very rare diseases, yet they may overlap. The two conditions not only share several clinical and laboratory characteristics but also differ in characteristic ways. Given the rapid response observed with anakinra in the index patient, IL-1beta likely plays a role in both diseases.

Oral allergy syndrome (OAS) or pollen-food allergy syndrome (PFS) is a hypersensitivity reaction to plant-based foods, manifesting most commonly with pruritus of the lips, tongue, and mouth. Unlike simple food allergy, OAS requires prior sensitization to a cross-reacting inhalant allergen rather than direct sensitization to a specific food protein. In this review, we summarize the clinical features and pathophysiology of OAS and provide an overview of known pollen-food associations.

A 24-year-old Bangladeshi man presented with a 12-week history of a pruritic papule on his left elbow that had enlarged and ulcerated. He was without any constitutional or systemic symptoms. He reported a history of extensive travel in the two years prior to presentation that included Bangladesh, South and Central America, and Mexico. Histopathologic features were consistent with leishmaniasis. Speciation by the Centers for Disease Control showed L. brasiliensis.

A 68-year-old woman presented for evaluation of a large, red-brown plaque on her left buttock with irregular borders and prominent overlying verrucous changes. The plaque had been present since childhood but over a three-year period had been enlarging with increasing nodularity and easy bleeding with trauma. Histopathologic examination demonstrated an enlarged papillated and polypoid heterogeneous lesion. In part of the specimen, there are bulbous aggregates of small squamous cells with foci of eccrine ductal differentiation. There are other areas with horn pseudocysts, hypergranulosis, and compact orthokeratosis with parakeratosis. There are scattered enlarged heavily pigmented melanocytes, some of which have long and thick dendrites. This collision tumor consisted of an eccrine poroma, a seborrheic keratosis, and a viral wart. The clinical and histopathologic features of collision tumors and poromas are reviewed.

Pigmented vulvar lesions are present in approximately 1 in 10 women and include melanocytic and nonmelanocytic proliferations. Vulvar nevi, melanosis, and melanoma are particularly challenging because of the similarity of their clinical and/or histopathological presentation. As a result, they are often difficult to diagnose, may result in patient and physician anxiety, and can lead to unneeded, potentially disfiguring surgical procedures. Because it is often detected late, vulvar melanoma carries a poor prognosis with associated significant morbidity and mortality, underscoring the importance of prompt recognition and treatment. In this review, we analyze the distinct epidemiologic, clinical, and histopathologic characteristics of vulvar nevi, melanosis, and melanoma, discuss treatment options, and propose a practical, systematic approach to facilitate formulation of a differential diagnosis and initiation of appropriate management.

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.