EPIDERMOLISIS AMPOLLAR PDF

Epidermolisis ampollar adquirida. Roberto Glorio · Authors. Roberto Glorio + 3 · Roberto Glorio. MARÍA JUAREZ. María Juárez. Alberto Woscoff. – Buy Recién nacido con Epidermolisis ampollar: Epidermólisis ampollar: Revisión bibliográfica, a propósito de un caso book online at best prices in. Recién nacido con Epidermolisis ampollar: Epidermólisis ampollar: Revisión bibliográfica, a propósito de un caso (Spanish Edition) [Marcela koroluk.

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Summary and related texts.

Check this box if you wish to receive a copy of your message. Clinical description The disease manifests in two clinical forms: 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.

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

Orphanet: Epidermolisis ampollar adquirida

Only comments written in English can be processed. Prognosis EBA is a chronic disease that resolves slowly and leads to dystrophic scarring and milia.

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.

In the classical form, onset occurs in adulthood and the bullae may be soft, tense or haemorrhagic, located on otherwise healthy skin. Management and treatment The first line treatment revolves around administration of dapsone or sulfasalazine. Lesions are usually triggered by minor trauma and are mainly localised to sites that are easily injured.

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The documents contained in this web site are presented for information purposes only. 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.

The disease manifests in two clinical forms: 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. Involvement of the mucous membranes, hair and nails is frequent. 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.

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.

Health care resources for this disease Expert centres Diagnostic tests 6 Patient organisations 8 Orphan drug s 1. Differential diagnosis The differential diagnosis should include other subepidermal, autoimmune bullous diseases. Other search option s Alphabetical list. Epidermolysis bullosa acquisita Prevalence: Diagnosis relies on the results of histological analysis, indirect or direct immunofluorescence studies, immunoblotting and immune electron microscopy.

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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. Specialised Social Services Eurordis directory.

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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. Additional epideromlisis Further information on this disease Classification s 1 Gene s 0 Clinical signs and symptoms Other website s 4. The nosological boundaries between EBA and bullous systemic lupus erythematosus see this term remain under debate.

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The first line treatment revolves around administration of dapsone or sulfasalazine. 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. Immunosuppresive therapy such as treatment with cyclosporine may by required in severe cases.

Summary Epidemiology The prevalence is unknown but the incidence is estimated at 1 in 96, amlollar cases per year.

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. The disease manifests during childhood. For all other comments, please send your remarks via contact us.

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