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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Immunosuppresive therapy such as treatment with cyclosporine may by required in severe cases. Diagnosis relies on the results of histological analysis, indirect or direct immunofluorescence studies, ampollarr and immune electron microscopy.

Epidermolisis bullosa by Roberto Solis on Prezi

Involvement of the mucosae in particular the ocular and ORL mucosal membranes epidermollisis associated with more severe disease, which may lead to a poorer functional, or even vital, prognosis. Summary and related texts. Prognosis EBA is a chronic disease that resolves slowly and leads to dystrophic scarring and milia. During the disease course, the inflammatory forms may ampollzr to resemble the classical form and patients with the classical present with bursts of inflammatory disease.

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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. Specialised Social Services Eurordis directory.

The disease manifests during childhood. Diagnostic methods Diagnosis relies on the results of histological analysis, indirect or direct immunofluorescence studies, immunoblotting and immune electron microscopy. In the classical form, onset occurs in adulthood and the bullae may be soft, tense or haemorrhagic, located on otherwise healthy skin.

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Orphanet: Epidermolisis ampollar adquirida

The disease manifests in two clinical forms: The nosological boundaries between EBA and bullous systemic lupus erythematosus see this term remain under debate. The epidermolisks contained in this web site are presented for information purposes only. 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.

Only comments written in English can be processed. 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 eidermolisis lamina densa.

Additional information Further information on this disease Classification s 1 Gene s 0 Clinical signs and symptoms Other website s 4. Differential diagnosis The differential diagnosis should include other subepidermal, autoimmune bullous diseases. Other search 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. Summary Epidemiology The prevalence is unknown but epidermolisos incidence is estimated at 1 in 96, new cases per year.

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EBA is a chronic disease that resolves slowly and leads to dystrophic scarring and milia. Clinical description The disease manifests in two clinical forms: 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. Health care resources for this disease Expert centres Diagnostic tests 6 Patient organisations 8 Orphan drug s 1. Epidermolysis bullosa acquisita Prevalence: 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.

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. 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.

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