A Case of Epidermolysis Bullosa Hereditaria

—Dowling Meara’s Type?—

Abstract

A 30-year-old man had blisters which repeatedly appeared on the trunk and extremities, from the third postnatal day. The blisters from 0.3 to 1cm in diameter were partially distributed in a circinate fashion. He also presented linear or reticular pigmentation on the trunk and extremities, severe itching, nail dystrophy, as well as palmo-plantar hyperidrosis and hyperkeratosis, but neither milia nor scar was observed. The blisters, looking subepidermal proved to be intraepidermal by electron-microscopic examination: the basal cells revealed segregation of cytoplasmic constituents and tonofibrillar clumping, followed by cytolysis. Although the family history suggested a recessive trait (no affected members in the family, and the parents are first cousins once removed), this case probably can be considered as belonging to Dowling-Meara’s type of dominant epidermolysis bullosa hereditaria.