EPIDERMOLYSIS BULLOSA DYSTROPHICA (RECESSIVE TYPE)

Abstract

Epidermolysis bullosa is probably not a single disease but rather a group of closely related pathologic states which differ both in clinical features and genetic origin. From the clinicogenetic point of view there is the simplex type, which is dominant, and the dystrophic type, which occurs in either a dominant or a recessive form. In addition, in a small number of cases of the dystrophic type peculiar clinical or genetic features are present; i. e., in the cases of Wende¹and Guy,²in that of the macular type described by da Costa and Van der Valk³and in the cases in infants reported by Heinrichsbauer⁴in which the disease is rapidly fatal. The following tabulation illustrates the different clinical features of the chief types⁵: The common feature of all types of epidermolysis bullosa is the formation of bullae. Various theories, based chiefly on the patho