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Case Reports

Abstract

Acute febrile neutrophilic dermatosis, also known as Sweet syndrome, is an uncommon inflammatory skin condition that presents with the rapid onset of painful erythematous plaques, nodules, or bullae and is characterized microscopically by a dense dermal neutrophilic infiltrate and papillary dermal edema. Although rare, acellular bodies surrounded by vacuolated spaces have been noted within the neutrophilic infiltrate in some cases, mimicking Cryptococcus infection. Here, we present a patient with cryptococcoid Sweet syndrome with concomitant hydralazine-induced ANCA vasculitis, which is rarely reported in the literature. A 67-year-old man with a history of end-stage renal disease (ESRD) and hypertension presented with shortness of breath and extensive bullae formation on the face and upper extremities, which ulcerated when ruptured. The patient’s lab results were positive for p-ANCA and anti-MPO, and his ANA was negative. His elevated p-ANCA levels, pulmonary symptoms, and previous history of GI bleeding raised suspicion for hydralazine-induced ANCA vasculitis, which had been prescribed for his poorly controlled hypertension. Hydralazine was discontinued, and his skin lesions improved. Then, at a subsequent hospitalization, he developed similar skin findings after receiving a dose of hydralazine. A shave biopsy showed dense neutrophilic infiltration with ovoid structures throughout the sampled portion of the dermis. A diastase chromic acid Schiff (DCAS) and Grocott’s methenamine silver (GMS) stains were negative in structures of interest, and tissue cultures and urine cryptococcus antigen were negative. Based on the patient's clinical history and histopathological examination, a diagnosis of hydralazine-associated cryptococcoid Sweet syndrome was favored.