Atypical Presentation of Severe Bullous Pemphigoid: A Case Report

Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disorder. Typically, patients will present with tense bullae and intense generalized pruritus with a skin biopsy demonstrating subepidermal split with eosinophils and a direct immunofluorescence highlighting autoantibodies against the basement membrane zone. Prognosis varies, and treatment involves an assessment of the severity of disease to determine whether to initiate topical or systemic immunosuppressive agents. We present an atypical presentation of BP that presented as a 3-to-4-week duration of pruritic small vesicular lesions in the upper chest, scabbed circular lesions along the upper extremity and pinnas of bilateral ear. Initially thought to be herpes zoster infection initially treated with valacyclovir for a week following a prior concern of a concomitant superficial skin infection with cephalexin and prednisone. With no clinical improvement, tissue biopsy was performed that confirmed bullous pemphigoid and treatment with steroid taper, doxycycline, and triamcinolone acetonide 0.1% cream was started. The aim of this case report is to present an atypical presentation of BP and to highlight maintaining a high index of suspicion of BP in patients presenting with disseminated significantly pruritic lesions.