Epidermolysis bullosa herpetiformis Dowling-Meara. Report of a case and pathomorphogenesis

Abstract

Report of a 2-year-old girl of Turkish origin with congenital generalized blister formation in herpetiform arrangement. Direct immunofluorescence ruled out juvenile dermatitis herpetiformis Duhring. Ultrastructural investigation of a fresh blister and clinically intact preblistering skin revealed intraepidermal blister formation via cytolysis of basal cells preceded by clumping of tonofilaments and partial attachment to the hemidesmosomes at the dermo-epidermal junction. This type of blister formation is significantly different from all other epidermolysis bullosa types and is a characteristic feature of all further cases studied so far by electron microscopy (n = 20) which correspond to the original cases of Dowling and Meara. Clinical features characteristic of this epidermolysis type are outlined and classification of epidermolysis bullosa herpetiformis Dowling-Meara into the non-scarring epidermolyses with dominant inheritance is discussed.