Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition Skin fragility-woolly hair-palmoplantar keratoderma syndrome is a rare, genetic, ectodermal dysplasia syndrome characterized by persistent skin fragility which manifests with blistering and erosions due to minimal trauma, woolly hair with variable alopecia, hyperkeratotic nail dysplasia, diffuse or focal palmoplantar keratoderma with painful fissuring, and no cardiac abnormalities. Perioral hyperkeratosis may also be associated. Disease data Classification Disease Synonyms Skin fragility-woolly hair-palmoplantar hyperkeratosis syndrome Zespół krucha skóra-włosy wełniste-hiperkeratoza dłoniowo-podeszwowa ORPHA code 293165 OMIM code 607655 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