Pemphigus Vulgaris With Esophageal Involvement in an Atopic Child Successfully Treated With Dupilumab

We report the case of a 3-year-old boy who was diagnosed with childhood pemphigus vulgaris having developed oral lesions, gastrointestinal symptoms with esophageal involvement, and failure to thrive. He had a markedly increased total serum IgE level and peripheral blood eosinophilia. The pemphigus was recalcitrant to conventional therapies and, based on the coexisting characteristics of Th2 immune deviation, he was treated with dupilumab and has had sustained clinical improvement since starting treatment. The case illustrates the importance of recognizing pemphigus vulgaris in childhood, considerations for dupilumab therapy, and a potential pathophysiological links between pemphigus autoantibodies in early life, Th2 inflammation, and atopic disorder.