Abstract
Case 1 occurred in a 56-year-old Japanese female who developed erythema and erosion on the lips, groin, and toes. The lesions gradually enlarged and evolved into elevated, brownish vegetating plaques. Case 2 occurred in a 36-year-old Japanese female who initially presented with erythema on the umbilicus, followed by blisters and erosions around the lips and vulva that, progressed to grayish-white vegetating plaques.
Histopathological examination in both cases showed intraepidermal abscesses with marked eosinophil infiltration, suprabasal cleft formation, and acantholysis. Direct immunofluorescence showed intercellular deposition of IgG in the epidermis, and indirect immunofluorescence detected circulating anti-intercellular IgG antibodies. Elevated serum levels of anti-desmoglein (Dsg)1 and anti-Dsg3 antibodies were also observed. On the basis of these findings, a diagnosis of pemphigus vegetans was made. Initiation of prednisolone (1.0 mg/kg/day) gradually improved the skin signs.
Pemphigus vegetans should be suspected when blisters or pustules arise in intertriginous areas or the oral cavity and subsequently develop into characteristic vegetating plaques. Histopathological examination, together with direct and indirect immunofluorescence studies and serological evaluation of anti-Dsg antibodies, is essential for accurate diagnosis.
