Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A rare genetic skin disease characterized by the triad of congenital scleroatrophy predominantly of the hands with sclerodactyly, palmoplantar keratoderma, and nail changes (consisting of hypoplasia, ridging, clubbing, and white discoloration). Additional features include palmar hypohidrosis and a high susceptibility to early-onset squamous cell carcinoma of affected skin areas. Disease data Classification Disease Synonyms Palmoplantar hyperkeratosis-sclerodactyly syndrome Scleroatrophic syndrome Zespół hiperkeratoza dłoniowo-podeszwowa - sklerodaktylia Zespół Hurieza Palmoplantar keratoderma-sclerodactyly syndrome Scleroatrophic syndrome Sclerotylosis ORPHA code 384 OMIM code 181600 ICD10 code Q82.8 ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl