title: [Congenital bullous erythroderma ichthyosiforme in 4 generations. Histological, electronmicroscopy findings and HLA typing].
abstract: Epidermolytic hyperkeratosis is a rare hereditary disease characterized by change of the dermatological signs with advancing age of the patients. At birth as well as during childhood, epidermolysis and blister formation are found; later the general picture of the disease presents horny hystrix-like lesions especially on the flexor parts of the extremities. Bullae become rare in adult life, vulnerability, however, remains. Here we report on 21 patients out of a 38-member family showing the above mentioned clinical findings. Electron-optical as well as histological studies are presented. For the first time HLA-type is reported.
mesh_terms: D000328:Adult; D000368:Aged; D006680:HLA Antigens / Q000276:immunology; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D010375:Pedigree; D012872:Skin Diseases, Vesiculobullous / Q000235:genetics* / Q000276:immunology / Q000473:pathology
chemical_list: D006680:HLA Antigens
vernacular_title: Erythrodermia congenitalis ichthyosiformis bullosa in vier Generationen. Histologische, elektronenmikroskopische Befunde und HLA-Typisierung.