THE HURIEZ-SYNDROME - SCLEROATROPHIC PALMOPLANTAR KERATODERMA

We describe a mother and son exhibiting a rare subtype of palmoplantar keratoderma, characterized by scleroatrophy, sclerodactyly, and nail anomalies. In the affected skin of the mother, 3 squamous cell carcino mas had developed at a young age. Retinoid treatment was started proph ylactically. After the original description by Huriez et al. in 1963, six other families and one case report have been described. The palmop lantar keratoderma is usually mild. However, the distinctive feature o f this syndrome is malignant degeneration of the affected skin at a yo ung age. Furthermore, an unusually high mortality rate for this type o f skin cancer of 5% has been reported. This case is described in order to underline the importance of accurate specification of each heredit ary palmoplantar keratoderma. The characteristic clinical hallmarks of Huriez syndrome enable a rapid diagnosis, which is important for prev enting malignant degeneration. Prophylaxis by retinoid treatment is di scussed.