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The disease manifests in two clinical forms: Immunosuppresive therapy such as treatment with cyclosporine may by required in severe cases.
Differential diagnosis The differential diagnosis should include other subepidermal, autoimmune bullous diseases. Only comments epidermolosis in English can be processed.
Disease definition Epidermolysis bullosa acquisita EBA is a subepidermal bullous dermatosis of autoimmune origin that was named as a result of its resemblance to hereditary forms of epidermolysis bullosa HEBmost notably dystrophic HEB.
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.
Health care resources for this disease Expert centres Diagnostic tests 6 Patient organisations 8 Orphan drug s 1. For all ampollat comments, please send your remarks via contact us.
Check this box if you wish to receive a copy of your message. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. Involvement of the mucous membranes, hair and nails is frequent. Epidermolysis bullosa acquisita EBA is a subepidermal bullous dermatosis of autoimmune origin that was named as a result of its resemblance to hereditary forms of epidermolysis bullosa HEBmost notably dystrophic HEB.
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Summary Epidemiology The prevalence is unknown but the incidence is estimated at 1 in 96, new cases per year. Specialised Social Services Eurordis directory. Epidermolysis bullosa acquisita Prevalence: Other search option s Alphabetical list. The first line treatment revolves around administration of dapsone or sulfasalazine.
Diagnosis relies on the results of histological analysis, indirect or direct immunofluorescence studies, immunoblotting and ampolla electron microscopy. As in dystrophic HEB caused by epidsrmolisis 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. Management and treatment The first line treatment revolves around administration of dapsone or sulfasalazine.
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.
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. Etiology EBA is caused by epidermolisie 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.
The documents contained in this web site are presented for information purposes only. Lesions are usually triggered by minor trauma and are mainly localised to sites that are easily injured.
Diagnostic methods Diagnosis relies on the results of histological analysis, indirect or direct immunofluorescence studies, immunoblotting and immune electron microscopy. The nosological boundaries between EBA and bullous systemic epidermolisiss erythematosus see this term remain under debate.
Orphanet: Epidermolisis ampollar adquirida
Additional information Further information on this disease Classification s 1 Gene s 0 Clinical signs and symptoms Other website s 4. Summary and related texts. 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. In the classical form, onset qmpollar in adulthood epideemolisis the bullae may be soft, tense or haemorrhagic, located on otherwise healthy skin.
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.
The disease manifests during childhood. Clinical description The disease manifests in two epidremolisis forms: 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.
Prognosis EBA is a chronic disease that resolves slowly and leads to dystrophic epider,olisis and milia. EBA is a chronic disease that resolves slowly and leads to dystrophic scarring and milia.