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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The first line treatment revolves around administration of dapsone or sulfasalazine. Involvement of the mucous membranes, hair and nails is frequent.

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 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. Summary and related texts.

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Other search option s Alphabetical list. 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. Epidermolysis bullosa acquisita Prevalence: Disease definition Epidermolysis bullosa acquisita EBA ampillar 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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Only comments written in English can be processed. 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. In the classical form, onset occurs in adulthood and the bullae may be soft, tense or haemorrhagic, located on otherwise healthy ampollzr.

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

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. The disease manifests during childhood.

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 apollar immunofluorescence studies, immunoblotting and immune electron microscopy. Management and treatment The first line treatment revolves around administration of dapsone or sulfasalazine.

Orphanet: Epidermolisis ampollar adquirida

Differential diagnosis The differential diagnosis should include other subepidermal, autoimmune bullous diseases. Summary Epidemiology The prevalence is unknown but the incidence is estimated at 1 in 96, new cases per year.

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.

Clinical description The disease manifests in two clinical forms: 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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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.

Check this box if you wish to receive a copy of your message. The inflammatory form was recognised more recently and resembles bullous pemphigoid see this term with bullae developing on erythematous a,pollar lesions, plaques without bullous eruptions and diffuse lesions that are not limited to trauma-prone sites.

For all other comments, please send your remarks via contact us. 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. Diagnosis relies on the results of histological analysis, indirect or direct immunofluorescence studies, immunoblotting and immune electron microscopy. The material is epidermolisus no way intended to replace professional medical care by a qualified epiderkolisis and should not be used as a basis for diagnosis or treatment.

EBA is a chronic disease that resolves slowly and leads to dystrophic scarring and milia. The disease manifests in two clinical forms: Specialised Social Epiderolisis Eurordis directory.