A 67-year-old Sikh male was diagnosed with pemphigus vulgaris in 1996 when he presented with oral erosions. His disease remained controlled on long-term, low-dose prednisolone therapy. In 2004, he developed a verrucous keratotic plaque over the occipital region of his scalp and it had been gradually enlarging (Fig. 1). Biopsies were performed in 2004 and 2008 and histological features in the epidermis of the specimens consist of orthokeratosis, parakeratosis, digitated epidermal hyperplasia, extensive suprabasal clefting, and acantholysis (Fig. 2). A dense infiltrate of plasma cells and lymphocytes were seen in the dermis. Direct immunofluorescence showed intercellular IgG and C3 deposition throughout epidermis. Indirect immunofluorescence was positive with antibody titers of more than 1/160. The levels of anti-desmoglein 3 antibody and anti-desmoglein 1 antibody were also elevated at 206 U/mL (negative if value is less than 7) and 81.4 U/mL (negative if value is less than 14) respectively.
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