Acro‐osteolysis: A complication of Jadassohn–Lewandowsky syndrome

Pachyonychia congenita (PC) is a group of inherited ectodermal dysplasias whose most prominent feature is hypertrophic nail dystrophy. 1 Muller, in 1904, and Wilson, in 1905, described the first cases of PC. One year later the same condition was described in two siblings by Jadassohn and Lewandowsky. 2 It usually develops in early infancy and the onset of nail changes beyond the first few years after infancy is rare. There are two major types of PC: Jadassohn–Lewandowsky syndrome (PC1) and Jackson–Lawler syndrome (PC2). Acro-osteolysis is a crippling deformity usually seen in a wide variety of neurological and vascular disorders. 3 Very rarely the association of PC with acro-osteolysis has been reported in the literature. 4,5 The significance of this association is not known. 5 A case of acroosteolysis as a complication of PC is presented with a review of relevant literature.