THE SCLEROATROPHIC SYNDROME OF HURIEZ - A CANCER-PRONE GENODERMATOSIS

We report a 24-year-old woman, her 6-year-old son and her 17-month-old daughter, who all suffer from a rare congenital genodermatosis first delineated by Huriez et al, in the 1960s. The clinical features of thi s autosomal dominant condition include scleroatrophy of the hands and feet, nail hypoplasia, mild palmoplantar keratoderma and hypohidrosis. Histological changes are non-specific. but immunohistological and ult rastructural examination in our index patient revealed an almost compl ete absence of epidermal Langerhans cells in the affected skin. This n ew finding may be linked to the cancer proneness of the scleroatrophic skin. In this family, the grandmother had died at the age of 37 years from metastatic squamous cell carcinoma which had arisen on the thena r eminence.