Huriez syndrome: case report with a detailed analysis of skin dendritic cells

We report a 60-year-old man with familial scleroatrophic syndrome of Huriez who developed squamous cell carcinomas on the affected skin of the right p alm. Immunohistochemical analysis showed a marked reduction in the number o f CD1a+, Lag+ and S100+ epidermal Langerhans cells, but not of CD1b+ and fa ctor XIIIa+ dermal dendritic cells, limited to palmoplantar skin. The Lange rhans cell depletion was not associated with an abnormal skin content of mR NA for factors involved in Langerhans cell development or recruitment in th e epidermis, including granulocyte/macrophage colony-stimulating factor, tr ansforming growth factor-beta1 and macrophage inflammatory protein-3 alpha. The results indicate that other as yet unknown mechanisms may account for the reduced number of Langerhans cells in the affected skin of such patient s.