A case of mucosal dominant pemphigus vulgaris mainly comprising oral lesions in childhood

Abstract

Pemphigus vulgaris (PV) is a rare autoimmune disease that causes blistering of the skin and mucous membranes. The condition commonly develops in middle-aged adults, and childhood onset is extremely rare. The present case was a 9-year-old girl who suffered from intractable blisters and erosions in the oral cavity. No skin lesioning was observed. Serological ELISA testing was positive against desmoglein (Dsg) 3, and direct immunofluorescence study revealed IgG and C3 deposits in the epidermal intercellular space. From the above results, we diagnosed pemphigus vulgaris. The clinical phenotype was mucosal dominant, consistent with the ELISA results. The patient’s oral mucosal lesions improved after treatment with prednisolone followed by high-dose intravenous immunoglobulin. The antibody titer to Dsg-3 decreased as symptoms improved.