Childhood linear IgA bullous dermatosis (Q86679): Difference between revisions
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A dermatose bolhosa linear por IgA infantil é uma doença autoimune da pele caracterizada por bolhas na pele e nas mucosas. As causas exatas da doença são desconhecidas, mas a doença é mediada por autoanticorpos IgA para hemidesmossomas e componentes da membrana basal BP180 e seu ectodomínio, BP230 e LAD285. A doença afeta crianças, muitas vezes em idade pré-escolar. Aqueles raros pacientes com envolvimento de mucosa muito proeminente com cicatrizes são agora classificados como penfigoide de membrana mucosa. | |||
| description / en | description / en | ||
Childhood linear IgA bullous dermatosis is an autoimmune skin disease characterised by blisters on the skin and mucous membranes. The exact causes of the disease are unknown but the disease is mediated by IgA autoantibodies to hemidesmosome and basement membrane components BP180 and its shed ectodomain, BP230 and LAD285. The disease affects children, often of preschool age. Those rare patients with very prominent mucosal involvement with scarring are now classified as mucous membrane pemphigoid. | |||
Revision as of 12:07, 15 August 2026
Childhood linear IgA bullous dermatosis is an autoimmune skin disease characterised by blisters on the skin and mucous membranes. The exact causes of the disease are unknown but the disease is mediated by IgA autoantibodies to hemidesmosome and basement membrane components BP180 and its shed ectodomain, BP230 and LAD285. The disease affects children, often of preschool age. Those rare patients with very prominent mucosal involvement with scarring are now classified as mucous membrane pemphigoid.
| Language | Label | Description | Also known as |
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| default for all languages | ID_470720333 |
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| English | Childhood linear IgA bullous dermatosis |
Childhood linear IgA bullous dermatosis is an autoimmune skin disease characterised by blisters on the skin and mucous membranes. The exact causes of the disease are unknown but the disease is mediated by IgA autoantibodies to hemidesmosome and basement membrane components BP180 and its shed ectodomain, BP230 and LAD285. The disease affects children, often of preschool age. Those rare patients with very prominent mucosal involvement with scarring are now classified as mucous membrane pemphigoid. |
