Pemphigus vulgaris in childhood: clinical features, treatment, and prognosis.

There are 31 reports in the literature of the clinical and immunopathologic features of pemphigus vulgaris in children. Bullous lesions often occur initially in the oral cavity, later spreading to the skin. The diagnosis can be established by means of histologic and immunopathologic criteria. Most patients respond to high doses of corticosteroids. In some instances, cytostatic drugs must be added to the treatment regimen. Two children reported in literature died in the first year of their disease, one child died 3 1/2 years after onset of the disease. Only four had a remission sufficient to discontinue therapy.