Schleimhautpemphigoide is the name of a heterogeneous group of rare chronic autoimmune disease, which tend to be switched on and causing decreasing bullous lesions of the mucous membranes. There is often followed by scarring and morbidity.

(Synonyms of mucous membrane, which are no longer used include cicatricial pemphigoid, ocular cicatricial pemphigoid, and benign mucous membrane.)

Schleimhautpemphigoide is the name of a heterogeneous group of rare chronic autoimmune disease, which tend to be switched on and causing decreasing bullous lesions of the mucous membranes. There is often followed by scarring and morbidity. (Synonyms of mucous membrane, which are no longer used include scarred pemphigoid, ocular cicatricial pemphigoid and benign mucous membrane.) Oral MMP and ocular MMP are typical, but other mucosal sites and skin (usually of the head and upper body) may be involved. The elderly are most often affected women more than men. A mucous membrane is characterized by subepithelial lesions caused by auto-antibodies against molecules of the epithelial basement membrane. The target molecules in MMP lie deep to those of bullous pemphigoid. Several autoantibodies were indentifiziert, including against BPAG2, laminin-332, VII and collagen type. Antibodies against beta-4 integrin have been identified in generalierter MMP and MMP ocular and antibodies to alpha-6-integrin identified in oral MMP. Diagnostic skin biopsy and direct immunofluorescence Prevalent mucosal involvement and scarring lesions help MMP of bullous pemphigoid to distinguish. The diagnosis of a mucous membrane pemphigoid by means of skin biopsy and direct immunofluorescence. Linear basement membrane deposits can include IgG, IgA and C3. Serum autoantibodies are usually not present or at low titer. A forecast Mucosal progresses slowly, rarely goes spontaneously and often responds incompletely to treatment. Depending on the affected place, can the serious consequences of eye damage and blindness, respiratory erosion and degradation, and include narrowing of the esophagus or anogenital regions. Anti-laminin-332 MMP is associated with an increased risk of internal cancers. Therapy for mild disease, corticosteroids and doxycycline and nicotinamide In severe disease systemic Immunsuppressivatherapie treatment of mucosal pemphigoid is similar to that for bullous pemphigoid. Topical or intralesional corticosteroids and a combination of oral doxycycline 100 mg p.o. 2 times a day and nicotinamide 500 mg po can 3 times a day be useful in mild cases. A serious illness can systemic immunosuppression with dapsone or prednisone or sometimes high-dose prednisone with immunosuppressive drugs (eg. As azathioprine, mycophenolate, cyclophosphamide, rituximab) and iv require immunoglobulin.

Health Life Media Team

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