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  • Title: Epidermolysis bullosa herpetiformis Dowling-Meara. Report of a case and pathomorphogenesis.
    Author: Anton-Lamprecht I, Schnyder UW.
    Journal: Dermatologica; 1982 Apr; 164(4):221-35. PubMed ID: 7084543.
    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.
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