| ID |
http://id.who.int/icd/entity/1148757980 |
| Preferred Name |
pemphigoïde cicatricielle type brunsting-perry |
| Definitions |
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). |
| Type |
http://www.w3.org/2002/07/owl#Class |
| comment | 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). a rare scarring immunobullous skin disorder due to autoantibodies to components of the epidermal υπόγειο membrane, particularly the anchoring filament component laminin 5 and the hemidesmosome-associated proteins bp180 and bp230. ένα ποσοστό των ασθενών μπορεί να αποδειχθεί ότι έχουν αντισώματα για να κολλήσει vii και ως εκ τούτου θεωρούνται ότι έχουν μια παραλλαγή του epidermolysis bullosa εξαγορά (epidermolysis bullosa εξαγορά: τύπος μελατονίνης-perry). une rare affection cutanée immunobulleuse cicatricielle due à des autoanticorps aux composantes de la membrane basale épidermique, en particulier la lamine 5 du filament d'ancrage et les protéines bp180 et bp230 associées à l'hématesmosome. on peut démontrer qu'une proportion de patients ont des anticorps contre le collagène vii et sont donc considérés comme présentant une variante de l'épidermolyse bullosa acquisita (epidermolysis bullosa acquisita : type brunsting-perry). |
| broaderTransitive | |
| prefLabel | pemphigoïde cicatricielle type brunsting-perry |
| browserUrl | NA |
| label | Cicatricial pemphigoid, Brunsting-Perry type pemphigoïde cicatricielle type brunsting-perry cicatricial pemphigoid, τύπος μελάγχρωσης-perry |
| subClassOf |
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