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Epidermolysis bullosa acquisita Prevalence: Clinical description The disease manifests in two clinical 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.
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EBA is a chronic disease that resolves slowly and leads to dystrophic scarring and milia. The disease manifests during childhood. Summary and related texts. Summary Epidemiology The prevalence is unknown but the incidence is estimated at 1 in 96, new cases per year.
epidermólisis ampollar – English Translation – Word Magic Spanish-English Dictionary
Health care resources for this disease Expert centres Diagnostic tests 6 Patient organisations 8 Orphan drug s 1. 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.
The nosological boundaries between EBA and bullous systemic lupus erythematosus see this term remain under debate. Additional information Further information on this disease Classification s 1 Gene s 0 Clinical signs and symptoms Other website s 4.
Lesions are usually triggered by minor trauma and are mainly localised to sites that are easily injured. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.
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Differential diagnosis The differential diagnosis should include other subepidermal, autoimmune bullous diseases. Only comments written in English can be processed. Diagnostic methods Diagnosis relies on the results of histological analysis, indirect or direct immunofluorescence studies, immunoblotting and immune electron microscopy.
Etiology EBA is caused by epidermoliwis 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. Other dpidermolisis option s Alphabetical list. 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.
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.
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.
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 Ampolllarmost notably dystrophic HEB.
Involvement of the mucous membranes, hair and nails is frequent. In the classical form, onset occurs in adulthood and the bullae may be soft, tense or haemorrhagic, located on otherwise healthy skin. Diagnosis relies on the results of histological analysis, indirect or direct immunofluorescence studies, immunoblotting and immune electron microscopy. The disease manifests in two clinical forms: Specialised Social Services Eurordis epidfrmolisis.
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. For all other comments, please send your remarks via contact us.
Immunosuppresive therapy such as treatment with cyclosporine may by required in severe cases. Prognosis EBA is a chronic disease that resolves slowly and leads ammpollar dystrophic scarring and milia. During the disease course, the inflammatory forms may evolve to resemble the classical form ampollar patients with the classical present with bursts of inflammatory xmpollar. Check this box if you wish to receive a copy of your message.
The documents contained in this web site are presented for information purposes only. Management and treatment The first line treatment revolves around administration of dapsone or sulfasalazine. 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. The first line treatment revolves around administration of dapsone or sulfasalazine.