Pityriasis rubra pilaris type I, classical adult (Q96869): Difference between revisions
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Forma mais comum e reconhecível de pitiríase rubra pilar. Apresenta-se como uma erupção eritematosa escamosa de rápida evolução que é inicialmente folicular, mas tipicamente progride para eritrodermia, dentro da qual caracteristicamente permanecem pequenas ilhas de pele não envolvida. Sem tratamento, tende a resolver-se espontaneamente após uma média de três anos. Afeta igualmente os sexos com maior incidência entre os 40 e os 60 anos de idade. A causa é pouco compreendida, embora mutações de ganho de função no gene CARD14 tenham sido relacionadas a formas genéticas raras da doença. | |||||||||||||||
| description / en | description / en | ||||||||||||||
This is the most common and recognisable form of pityriasis rubra pilaris. It presents as a rapidly evolving scaly erythematous rash which is initially follicular but typically progresses to erythroderma, within which small islands of uninvolved skin characteristically remain. Untreated, it tends to resolve spontaneously after an average of three years. It affects the sexes equally with the highest incidence between 40 and 60 years of age. The cause is poorly understood though gain-of-function mutations in the CARD14 gene have been linked to rare genetic forms of the disease. | |||||||||||||||
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| Property / Canonical URI: https://id.who.int/icd/entity/1296778126 / rank | |||||||||||||||
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CID11:ID_1296778126 | |||||||||||||||
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dki-india-ID_1296778126 | |||||||||||||||
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| Property / Knowledge Architect: https://pauloleads.com.br/cases-publicos/ / rank | |||||||||||||||
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15 August 2026
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| Property / Collection date: 15 August 2026 / rank | |||||||||||||||
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Latest revision as of 12:54, 16 August 2026
This is the most common and recognisable form of pityriasis rubra pilaris. It presents as a rapidly evolving scaly erythematous rash which is initially follicular but typically progresses to erythroderma, within which small islands of uninvolved skin characteristically remain. Untreated, it tends to resolve spontaneously after an average of three years. It affects the sexes equally with the highest incidence between 40 and 60 years of age. The cause is poorly understood though gain-of-function mutations in the CARD14 gene have been linked to rare genetic forms of the disease.
| Language | Label | Description | Also known as |
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| default for all languages | ID_1296778126 |
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| English | Pityriasis rubra pilaris type I, classical adult |
This is the most common and recognisable form of pityriasis rubra pilaris. It presents as a rapidly evolving scaly erythematous rash which is initially follicular but typically progresses to erythroderma, within which small islands of uninvolved skin characteristically remain. Untreated, it tends to resolve spontaneously after an average of three years. It affects the sexes equally with the highest incidence between 40 and 60 years of age. The cause is poorly understood though gain-of-function mutations in the CARD14 gene have been linked to rare genetic forms of the disease. |
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CID11:ID_1296778126
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dki-india-ID_1296778126
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Concluído
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15 August 2026
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