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Schleimhautbeteiligung bei blasenbildenden Autoimmunerkrankungen

Involvement of mucous membranes in autoimmune bullous diseases

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Zusammenfassung

Bei blasenbildenden Autoimmunerkrankungen kommt es durch Autoantikörperablagerung zu einer intra- oder subepidermalen Spaltbildung, die sich klinisch mit Blasen und sekundären Erosionen manifestiert. Eine Schleimhautbeteiligung ist häufig beim Pemphigus vulgaris und obligat beim vernarbenden Schleimhautpemphigoid. Seltener gehen bullöses Pemphigoid oder eine Epidermolysis bullosa acquisita mit einer Schleimhautbeteiligung einher. Für die Diagnose sind die Bestimmung der spezifisch abgelagerten Autoantikörper und das klinische Bild entscheidend. Die Therapie beruht auf einer Immunsuppression, die dem Typ der Erkrankung und der Schwere des Schleimhautbefalls angepasst werden muss. Zumindest die okuläre Beteiligung beim vernarbenden Schleimhautpemphigoid und der Pemphigus vulgaris erfordern eine systemische Therapie.

Abstract

Autoimmune bullous diseases are characterized by intraepidermal or subepidermal autoantibody deposition that leads to blisters and secondary erosion. Mucous membranes are frequently affected in pemphigus vulgaris and always involved in cicatricial and mucosal pemphigoid. Mucosal lesions are detected less frequently in patients with bullous pemphigoid or epidermolysis bullosa acquisita. The diagnosis of autoimmune bullous disorders is based on determination of the subtype of autoantibodies bound in the skin and the clinical picture. Treatment is based on immunosuppression related to the type of disease and severity of the mucosal symptoms. Ocular involvement in mucosal pemphigoid and pemphigus vulgaris requires systemic treatment.

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Danksagung

C. Günther erhielt eine Förderung der Deutschen Forschungsgemeinschaft (GU1212/1-1 und GU 1212/1-2).

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  1. Klinik und Poliklinik für Dermatologie, Universitätsklinikum Dresden, Fetscherstr. 74, 01307, Dresden, Deutschland

    C. Günther

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Dieser Beitrag beinhaltet keine vom Autor durchgeführten Studien an Menschen oder Tieren.

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Günther, C. Schleimhautbeteiligung bei blasenbildenden Autoimmunerkrankungen. Hautarzt 67, 774–779 (2016). https://doi.org/10.1007/s00105-016-3871-6

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  • DOI: https://doi.org/10.1007/s00105-016-3871-6

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