Pemphigus of the eyelids

We report the case of a 56-year-old woman who presented with a 2-month history of widespread oral erosion and a 3-day history of small papules on the lower eyelids. No other skin involvement was found. Histopathological examination revealed suprabasal cleft and acantholysis in the lower epidermis of the papule on the lower eyelid and in the lower mucous membrane of the oral mucosa. Intercellular deposits of IgG and C3 were seen in the whole epidermis of the specimen from the papule on the right lower eyelid by direct immunofluorescence study. These deposits were also observed in the biopsy specimen from erosion on the left buccal membrane. Indirect immunofluorescence study using normal human skin as a substrate showed intercellular antibodies directed to the cell surface of the whole epidermis with a titer of 1:40. The titers of antibodies to desmoglein 3 and 1 were 118 and 25.9, respectively, by enzyme-linked immunosorbent assay. The patient was treated with an oral administration of prednisolone (0.75 mg/kg/day) for 9 days, which improved the skin eruptions and oral erosion. The dose of prednisolone was gradually tapered and it took 10 weeks to cease this treatment. These findings suggest that this patient is an unusual case of pemphigus vulgaris (mucosal dominant type) diagnosed from the clinical and histopathological findings, with positive antibodies to desmoglein 3 and 1.