32677 IgA pemphigus in a patient with rheumatoid arthritis and cardiac amyloid successfully treated with oral dapsone

Abstract

IgA pemphigus is a rare autoimmune blistering disease with overarching features including a predominantly neutrophilic infiltrate, mild acantholysis, and intraepidermal IgA deposition. There is a slight female predominance and age of onset ranges widely from infants to late adulthood, but given its rarity, the incidence of this disease is not well appreciated. As of 2019, only 137 cases have been described from 26 different countries. We report an 84-year-old man with several week history of pruritic, blistering rash that started in the groin and spread to involve the trunk and upper extremities.