A 72-year-old man with a history of hypertension, atrial flutter, chronic renal failure, and hyperuricemia consulted with lesions that had appeared approximately 2 months earlier. The lesions began as excrescent plaques in the right inguinal fold and later in the left inguinal fold.

Examination revealed erythematous plaques in both inguinal folds; the plaques were formed by warty, exudative, foul-smelling lesions (Fig. 1).

Figure 1

(0.06MB).

A skin biopsy revealed an epidermis with intense acanthosis and papillomatosis, hyperkeratosis and focal parakeratosis, with a subcorneal intraepidermal bulla that contained shed keratinocytes mixed with lymphocytes, eosinophils, and polymorphonuclear cells. Direct immunofluorescence revealed granular deposits of immune complexes with IgG in the cytoplasmic membrane of the keratinocytes and in the desmosomes.

Levels of anti-intracellular substance antibodies and antidesmoglein 1 and 3 antibodies (1:320, 3.43, and 8.01, respectively) were positive.

Skin symptoms worsened a month later, with an increase in the inguinal excrescent lesions and the appearance of new warty lesions in the axillas and oral commissures, some smaller, isolated lesions on the torso, and erosions on the buccal mucosa.

A diagnosis pemphigus vegetans was established and treatment was instated with a tapered regimen of oral prednisone (initial dosage of 0.5mg/kg/d) and the lesions on the skin and mucosa resolved.