Juvenile hyaline fibromatosis (Q101615): Difference between revisions
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Fibromatose hialina juvenil (FHJ) é uma condição genética caracterizada por lesões cutâneas papulonodulares, massas de tecidos moles, hipertrofia gengival e contraturas em flexão das grandes articulações devido à deposição hialina perivascular. Os critérios diagnósticos típicos são fibroma subcutâneo hialino múltiplo, tumores filamentosos da pele, fibromatose gengival, contraturas musculares das extremidades e múltiplas destruições ósseas osteolíticas. | |||||||||||||||
| description / en | description / en | ||||||||||||||
Juvenile hyaline fibromatosis (JHF) is a genetic condition characterised by papulonodular skin lesions, soft tissue masses, gingival hypertrophy, and flexion contractures of the large joints due to perivascular hyaline deposition. Typical diagnostic criteria are multiple hyaline subcutaneous fibroma, filamentous tumours of the skin, gingival fibromatosis, muscle contractures of the extremities and multiple osteolytic bone destructions. | |||||||||||||||
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| Property / Canonical URI: https://id.who.int/icd/entity/1890146024 / rank | |||||||||||||||
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CID11:ID_1890146024 | |||||||||||||||
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dki-india-ID_1890146024 | |||||||||||||||
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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 18:27, 16 August 2026
Juvenile hyaline fibromatosis (JHF) is a genetic condition characterised by papulonodular skin lesions, soft tissue masses, gingival hypertrophy, and flexion contractures of the large joints due to perivascular hyaline deposition. Typical diagnostic criteria are multiple hyaline subcutaneous fibroma, filamentous tumours of the skin, gingival fibromatosis, muscle contractures of the extremities and multiple osteolytic bone destructions.
| Language | Label | Description | Also known as |
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| default for all languages | ID_1890146024 |
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| English | Juvenile hyaline fibromatosis |
Juvenile hyaline fibromatosis (JHF) is a genetic condition characterised by papulonodular skin lesions, soft tissue masses, gingival hypertrophy, and flexion contractures of the large joints due to perivascular hyaline deposition. Typical diagnostic criteria are multiple hyaline subcutaneous fibroma, filamentous tumours of the skin, gingival fibromatosis, muscle contractures of the extremities and multiple osteolytic bone destructions. |
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CID11:ID_1890146024
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dki-india-ID_1890146024
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Concluído
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15 August 2026
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