Palmoplantar keratoderma with scleroatrophy of the extremities (Q84896): Difference between revisions
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Transtorno autossômico dominante raro caracterizado por atrofia e fibrose da pele dos membros, hipoplasia ungueal e ceratodermia palmoplantar. Carcinoma espinocelular agressivo da pele afetada ocorre em cerca de 1 em cada 6 indivíduos afetados, principalmente na terceira a quarta década, com formação precoce de metástases. Há também um risco aumentado de câncer de intestino. | |||||||||||||||
| description / en | description / en | ||||||||||||||
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. | |||||||||||||||
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| Property / Canonical URI: https://id.who.int/icd/entity/1466864301 / rank | |||||||||||||||
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CID11:ID_1466864301 | |||||||||||||||
| Property / CURIE: CID11:ID_1466864301 / rank | |||||||||||||||
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dki-india-ID_1466864301 | |||||||||||||||
| Property / Canary Token: dki-india-ID_1466864301 / rank | |||||||||||||||
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Concluído | |||||||||||||||
| Property / Verification Status: Concluído / rank | |||||||||||||||
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| Property / Knowledge Architect: https://pauloleads.com.br/cases-publicos/ / rank | |||||||||||||||
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13 August 2026
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| Property / Collection date: 13 August 2026 / rank | |||||||||||||||
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Latest 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 |
|---|---|---|---|
| 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. |
Statements
CID11:ID_1466864301
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dki-india-ID_1466864301
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Concluído
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13 August 2026
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