Dermochondrocorneal dystrophy (Francois' syndrome).

© 2010 The Authors. doi: 10.2340/00015555-0834 Journal Compilation © 2010 Acta Dermato-Venereologica. ISSN 0001-5555 Sir, Dermochondrocorneal dystrophy (of Francois) is a rare inherited disease. The mode of inheritance is uncertain and both autosomal recessive and dominant traits have been suggested (1). The disease is characterized by corneal dystrophy, and xanthomatous nodules of the hands, elbows and nose, together with deformity of the hands and feet. In addition, ocular changes are present, leading to reduced vision. The disease was initially described in 1949 by Francois (2). The histopathology of the disease is described as a proliferation of fibroblasts, and analysis of metabolites from urinary collagen has suggested increased production of type III collagen (3, 4). Only a few cases have been reported in the literature (5–7). We report here the case of a man presenting with classical skin changes.