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Disease definition Epidermolysis bullosa acquisita EBA is a subepidermal bullous dermatosis of autoimmune origin that was named anpollar a result of its resemblance to hereditary forms of epidermolysis bullosa HEBmost notably dystrophic HEB.

During the disease course, the inflammatory forms may evolve to resemble the classical form and patients with the classical present with bursts of inflammatory disease.

Summary and related texts.

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The first line treatment revolves around administration of dapsone or sulfasalazine. Epidermolysis bullosa acquisita Prevalence: EBA is a chronic disease that resolves slowly and leads to dystrophic scarring and milia. The disease manifests during childhood.

Fpidermolisis nosological boundaries between EBA and bullous systemic lupus erythematosus see this term remain under debate. Specialised Social Services Eurordis directory. Clinical description The disease manifests in two clinical forms: Although further trials are necessary, encouraging results have been obtained with other approaches such as intravenous immunoglobulin therapy, extracorporeal photochemotherapy and, more recently, rituximab therapy.

Diagnostic methods Diagnosis relies on the results of histological analysis, indirect or direct immunofluorescence studies, immunoblotting and immune electron microscopy. Only comments seeking to improve the quality and accuracy of epideemolisis on the Orphanet website are accepted.

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Involvement of the mucous membranes, hair and nails is frequent. Additional information Further information on this disease Classification s 1 Gene s 0 Clinical signs and symptoms Other website s 4. As in dystrophic HEB caused by mutations in the gene encoding collagen VII, the deposition of antibodies on collagen VII leads to cleavage between the epidermis and dermis below the lamina densa.

Involvement of the mucosae in particular the ocular and ORL mucosal membranes is associated with more severe disease, which may lead to a poorer functional, or even vital, prognosis. Summary Epidemiology The prevalence is unknown but the incidence is estimated at 1 in 96, new cases per year. Lesions are usually triggered by minor trauma and are mainly localised to sites that are easily injured. Epidermolysis bullosa acquisita EBA is a subepidermal bullous dermatosis of autoimmune origin that was epldermolisis as a result of its resemblance to hereditary forms of epidermolysis bullosa HEB epidermopisis, most notably dystrophic HEB.

Management and treatment The first line treatment revolves around administration of dapsone or sulfasalazine.

The documents contained in this web site are presented for information purposes only. Differential diagnosis The differential diagnosis should include other subepidermal, autoimmune bullous diseases. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.

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Other search option s Alphabetical list. Diagnosis relies on the results of histological analysis, indirect or direct immunofluorescence studies, immunoblotting and immune electron microscopy. Only comments written in English can be processed. Prognosis EBA is a chronic disease that resolves slowly and leads to dystrophic scarring and milia. EBA is caused by the production of antibodies against the skin basement membrane collagen VII, the major component of the anchoring fibrils located in the dermal-epidermal junction, under the lamina densa.

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Health care resources for this disease Expert centres Diagnostic tests 6 Patient organisations 8 Orphan drug s 1. Immunosuppresive therapy such as treatment with cyclosporine may by required in severe cases.

Orphanet: Epidermolisis ampollar adquirida

Etiology EBA is caused by the production of antibodies against the skin basement membrane collagen VII, the major component of the anchoring fibrils located in the dermal-epidermal junction, under the lamina densa. Eppidermolisis disease manifests in two clinical forms: In the classical form, onset occurs in adulthood and the bullae may be soft, tense or haemorrhagic, located on otherwise healthy skin.

The inflammatory form was recognised more recently and resembles bullous pemphigoid see this term with bullae developing on erythematous skin lesions, plaques without bullous eruptions and diffuse lesions that are not limited to trauma-prone sites.

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