Cicatricial pemphigoid, Brunsting-Perry type (Q104444): Difference between revisions
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Transtorno raro imunobolhoso cicatricial da pele devido a autoanticorpos contra componentes da membrana basal epidérmica, particularmente o componente do filamento de ancoragem laminina 5 e as proteínas associadas ao hemidesmossoma BP180 e BP230. Pode ser demonstrado que uma proporção de pacientes apresenta anticorpos contra o colágeno VII e, portanto, considerado que apresentam uma variante da epidermólise bolhosa adquirida (Epidermólise bolhosa adquirida: tipo Brunsting-Perry). | |||
| description / en | description / en | ||
A rare scarring immunobullous skin disorder due to autoantibodies to components of the epidermal basement membrane, particularly the anchoring filament component laminin 5 and the hemidesmosome-associated proteins BP180 and BP230. A proportion of patients can be shown to have antibodies to collagen VII and are therefore considered to have a variant of epidermolysis bullosa acquisita (Epidermolysis bullosa acquisita: Brunsting-Perry type). | |||
Revision as of 10:18, 17 August 2026
A rare scarring immunobullous skin disorder due to autoantibodies to components of the epidermal basement membrane, particularly the anchoring filament component laminin 5 and the hemidesmosome-associated proteins BP180 and BP230. A proportion of patients can be shown to have antibodies to collagen VII and are therefore considered to have a variant of epidermolysis bullosa acquisita (Epidermolysis bullosa acquisita: Brunsting-Perry type).
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | ID_1148757980 |
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| English | Cicatricial pemphigoid, Brunsting-Perry type |
A rare scarring immunobullous skin disorder due to autoantibodies to components of the epidermal basement membrane, particularly the anchoring filament component laminin 5 and the hemidesmosome-associated proteins BP180 and BP230. A proportion of patients can be shown to have antibodies to collagen VII and are therefore considered to have a variant of epidermolysis bullosa acquisita (Epidermolysis bullosa acquisita: Brunsting-Perry type). |
