EPIDERMOLISIS AMPOLLAR PDF

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Clinical description The disease manifests in two clinical forms: Epidermolysis bullosa acquisita Prevalence: Other search option s Alphabetical list. The documents contained in this web site are presented epidegmolisis information purposes only.

The disease manifests during childhood. The disease manifests in two clinical forms: Check this box if you wish to receive a copy of your message. The first line treatment revolves around administration of dapsone or sulfasalazine. 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.

Additional information Epodermolisis information on this disease Classification s 1 Gene s 0 Clinical signs and symptoms Other website s 4. Only comments written in English can be processed. 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.

Health care resources for this disease Expert centres Diagnostic tests 6 Patient organisations 8 Orphan drug s 1. Summary Epidemiology The prevalence is unknown but the incidence is estimated at 1 in 96, new cases per year. Management and treatment The first line treatment revolves around administration of dapsone or sulfasalazine.

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Lesions are usually triggered by minor trauma and are mainly localised to sites that are easily injured. The inflammatory form was recognised more recently and resembles bullous pemphigoid see this term eppidermolisis bullae developing on erythematous skin lesions, plaques without bullous eruptions and diffuse lesions that are not limited to trauma-prone sites.

Diagnostic methods Diagnosis relies on the results of histological analysis, indirect or direct immunofluorescence studies, immunoblotting and immune electron microscopy. 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. EBA is a chronic disease that resolves slowly and leads to dystrophic scarring and milia. 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.

Diagnosis relies on the results of histological analysis, indirect or direct immunofluorescence studies, immunoblotting and immune electron microscopy.

For all other comments, please send your remarks via contact us. Specialised Social Services Eurordis directory. The nosological boundaries between EBA and bullous systemic lupus erythematosus see this term remain under debate.

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peidermolisis 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. 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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Prognosis EBA is a chronic disease that resolves slowly and leads to dystrophic scarring and milia. In the classical form, onset occurs in adulthood and the bullae may be soft, tense or haemorrhagic, located on otherwise healthy skin.

Epidermolisis bullosa by Roberto Solis on Prezi

Involvement of the mucous membranes, hair and nails is frequent. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.

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. Differential diagnosis The differential diagnosis should include other subepidermal, autoimmune bullous diseases. 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.

Although further trials are necessary, encouraging results have epidermolisls obtained with other approaches such as intravenous immunoglobulin therapy, extracorporeal photochemotherapy and, more recently, rituximab therapy.

Immunosuppresive therapy such as treatment with cyclosporine may by required in severe cases.