Preferred Label : Palmoplantar keratoderma with scleroatrophy of the extremities;
ICD-11 definition : A rare autosomal dominant disorder characterised by atrophy and fibrosis of the skin
of the limbs, nail hypoplasia and palmoplantar keratoderma. Aggressive squamous cell
carcinoma of affected skin occurs in about 1 in 6 affected individuals, mostly in
the third to fourth decade, with early metastasis formation. There is also an increased
risk of bowel cancer.;
ICD-11 synonym : Huriez palmoplantar keratoderma; Palmoplantar keratoderma-sclerodactyly syndrome; Sclerotylosis;
ICD-11 inclusion : Huriez palmoplantar keratoderma;
Origin ID : 1466864301;
Automatic exact mappings (from CISMeF team)
A rare autosomal dominant disorder characterised by atrophy and fibrosis of the skin
of the limbs, nail hypoplasia and palmoplantar keratoderma. Aggressive squamous cell
carcinoma of affected skin occurs in about 1 in 6 affected individuals, mostly in
the third to fourth decade, with early metastasis formation. There is also an increased
risk of bowel cancer.
