A woman in her 50s presented with a 7-month history of worsening pruritic papules and bullae on her face, trunk, arms, and axillae. Her medical history was significant for Meniere disease and hyperthyroidism. At an outside clinic, an initial skin biopsy from the arm showed intraepidermal acantholysis with dyskeratosis and she was diagnosed with transient acantholytic dermatosis (Grover disease). Treatments included triamcinolone ointment, doxycycline, antihistamines, and short courses of prednisone without clinical improvement. Over the following two months, her eruption worsened, and she developed painful oral mucosal erosions. Physical examination revealed vegetative scale-crusts overlying erosions on the face, arms, and chest in an armor-like pattern as well as flaccid bullae on the back and erosions of the gingival and labial mucosae. A shave biopsy of the skin from the upper back was performed. Direct immunofluorescence showed intercellular epidermal deposition of IgG and C3. Indirect immunofluorescence was positive on monkey esophagus substrate in an intercellular pattern at a titer of 1:5120 and enzyme-linked immunosorbent assay (ELISA) quantified anti-desmoglein-1 (Dsg1) and anti-desmoglein-3 (Dsg3) antibodies at 620 (negative<20) and 176 units (negative<20), respectively.
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