Epidermolysis bullosa simplex superficialis. A new variant of epidermolysis bullosa characterized by subcorneal skin cleavage mimicking peeling skin syndrome.
We report a new variant of epidermolysis bullosa simplex (EBS), termed EBS superficialis, which is characterized by the development of skin cleavage just beneath the level of stratum corneum. In two of seven patients identified, a second and more focal cleft within the lower third of the epidermis was also detected. Epidermolysis bullosa simplex superficialis appears to be transmitted as an autosomal dominant trait. It differs from other autosomal dominant forms of EBS by the common findings of milia and atrophic scarring, as well as involvement of oral and/or ocular surfaces. Epidermolysis bullosa simplex superficialis is further differentiated from peeling skin syndrome by the presence of blisters and the absence of spontaneous continual exfoliation or peeling.
Fine
J D
JD
Department of Dermatology, School of Medicine, University of Alabama, Birmingham 35294.
Johnson
L
L
Wright
T
T
D000328:Adult; D001706:Biopsy; D002648:Child; D003937:Diagnosis, Differential; D004817:Epidermis / Q000473:pathology* / Q000648:ultrastructure; D004820:Epidermolysis Bullosa / Q000145:classification / Q000235:genetics / Q000473:pathology*; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D010375:Pedigree; D012621:Seasons; D013577:Syndrome