Recessive dystrophic epidermolysis bullosa, generalised severe (Q96101): Difference between revisions
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Epidermólise bolhosa distrófica recessiva generalizada grave é o subtipo mais grave de epidermólise bolhosa distrófica. Anteriormente era conhecida como o tipo Hallopeau-Siemens. É caracterizada por bolhas e cicatrizes cutâneas e mucosas generalizadas associadas a deformidades graves e envolvimento extracutâneo importante. Indivíduos afetados estão em alto risco de carcinomas de células escamosas agressivos em áreas de ulceração crônica. | |||||||||||||||
| description / en | description / en | ||||||||||||||
Severe generalised recessive dystrophic epidermolysis bullosa is the most severe subtype of dystrophic epidermolysis bullosa. It was formerly known as the Hallopeau-Siemens type. It is characterised by generalised cutaneous and mucosal blistering and scarring associated with severe deformities and major extracutaneous involvement. Affected individuals are at high risk of aggressive squamous cell carcinomas in areas of chronic ulceration. | |||||||||||||||
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| Property / Canonical URI: https://id.who.int/icd/entity/365362994 / rank | |||||||||||||||
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CID11:ID_365362994 | |||||||||||||||
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dki-india-ID_365362994 | |||||||||||||||
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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 11:58, 16 August 2026
Severe generalised recessive dystrophic epidermolysis bullosa is the most severe subtype of dystrophic epidermolysis bullosa. It was formerly known as the Hallopeau-Siemens type. It is characterised by generalised cutaneous and mucosal blistering and scarring associated with severe deformities and major extracutaneous involvement. Affected individuals are at high risk of aggressive squamous cell carcinomas in areas of chronic ulceration.
| Language | Label | Description | Also known as |
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| default for all languages | ID_365362994 |
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| English | Recessive dystrophic epidermolysis bullosa, generalised severe |
Severe generalised recessive dystrophic epidermolysis bullosa is the most severe subtype of dystrophic epidermolysis bullosa. It was formerly known as the Hallopeau-Siemens type. It is characterised by generalised cutaneous and mucosal blistering and scarring associated with severe deformities and major extracutaneous involvement. Affected individuals are at high risk of aggressive squamous cell carcinomas in areas of chronic ulceration. |
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CID11:ID_365362994
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dki-india-ID_365362994
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Concluído
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15 August 2026
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