[Congenital bullous erythroderma ichthyosiforme in 4 generations. Histological, electronmicroscopy findings and HLA...

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.