Sclerotylosis (Scleroatrophic and Keratotic Dermatosis of Limbs; Scleroatrophic Syndrome of Huriez)
This rare autosomal dominant condition is characterized by scleroatrophy of the skin of the hands and feet, hypoplastic nails, and palmoplantar keratoderma. Skin cancer (squamous cell carcinoma) and bowel cancer are said to be common in this condition, and squamous cell carcinomas of the tongue and tonsil also occur. Aggressive squamous cell carcinomas develop on the skin with early onset (third to fourth decade) in about 15 % cases.
The linkage has been established with the MN blood group on chromosome 4q28–3. In the scleroatrophic syndrome of Huriez, the histopathologic findings of an almost complete absence of epidermal Langerhans cells in affected skin may explain the susceptibility to squamous cell carcinoma in these lesions (Delaporte et al. 1995; Hamm et al. 1996; Downs and Kennedy 1998; Guerriero et al. 2000; Lee et al. 2000).