Description of the disease * EnglishPolish Pobierz sekcję do PDF Definicja A rare genetic skin disorder characterized by very early-onset of progressive skin thickening over the entire body (except for eyelids, neck and ears), progressively limited joint mobility with gradual freezing of joints, and eventual severe chest and abdomen movement restriction, manifesting with restrictive pulmonary disease, which may lead to death. Additional features include severe growth restriction and osteoporosis. There have been no further descriptions in the literature since 1974. Disease data Klasyfikacja Disease Synonimy Hard skin syndrome, Parana type Zespół twardej skóry, typu Parana Kod ORPHA 2812 Kod OMIM 260530 Kod ICD10 L91.8 Kod ICD11 - *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