Palmoplantar keratoderma with scleroatrophy of the extremities (Q84896): Difference between revisions
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Revision as of 10:15, 15 August 2026
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.
| Language | Label | Description | Also known as |
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| default for all languages | ID_1466864301 |
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| English | Palmoplantar keratoderma with scleroatrophy of the extremities |
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. |
