A Rare Presentation of Pemphigus Erythematosus as Pustules

Pemphigus erythematosus (PE) is an autoimmune blistering disease that is characterized by erythema and blisters, but rarely pustules, on the upper torso, face, and scalp. A 37-year-old woman presented with a 20 day history of intense pruritus as well as multiple papules and pustules on the trunk and face. Initially, the disease was misdiagnosed as impetigo due to the presence of pustules, nontypical lesions, and pathology results. Histopathology of new bullas showed epidermal hyperkeratosis, blistering and typical acantholysis, and the presence of diffuse inflammatory cells. Direct immunofluorescence showed intraepidermal, intercellular deposition of IgG autoantibodies. A diagnosis of pemphigus erythematosus was made. Systemic steroid therapy was effective in controlling the disease. To the best of our knowledge, this is the first case report on pemphigus erythematosus presenting with pustules.