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Therapie des bullösen Pemphigoids

Management of bullous pemphigoid

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Zusammenfassung

Hintergrund

Das bullöse Pemphigoid ist die häufigste bullöse Autoimmundermatose des Erwachsenenalters, bei der es, bedingt durch Autoantikörperbildung gegen die hemidesmosomalen Adhäsionsproteine (BP180/Typ-XVII-Kollagen und BP230), zur subepidermalen Spaltbildung kommt. Folglich weisen die Patienten, die zumeist das 70. Lebensjahr überschreiten, klinisch pralle Bullae mit nachfolgenden Erosionen an der Haut und seltener an den hautnahen Schleimhäuten auf.

Problematik

Die Erkrankung zeigt in der Regel einen chronisch schubweisen Verlauf, der häufig den längerfristigen Einsatz topischer Kortikosteroide ggf. in Kombination mit systemischen Steroiden/Immunsuppressiva notwendig macht. Ein kurativer Therapieansatz besteht bislang nicht. Bei langfristig erforderlicher Therapie sind die medikamentenspezifischen Nebenwirkungen sowie altersentsprechende Vorerkrankungen der Patienten zu beachten.

Schlussfolgerung

Die Therapie der Wahl des bullösen Pemphigoids richtet sich in erster Linie nach der Krankheitsaktivität und damit einhergehend nach der Ausprägung der (muko)kutanen Läsionen. Abhängig von der Ausprägung werden aufgrund von geringerer Komorbidität topische Klasse-IV-Kortikosteroide eingesetzt. Bei initial ausgedehnter Manifestation oder in therapierefraktären Fällen sollte eine Systemtherapie mit Kortikosteroiden ggf. mit steroidsparenden adjuvanten Immunmodulatoren wie Dapson oder Doxycyclin oder mit adjuvanten Immunsuppressiva wie Azathioprin, Mycophenolen oder Methotrexat erfolgen. Der CD20-Antikörper Rituximab sowie hoch dosierte intravenöse Immunglobuline (IVIG) stellen ergänzende Therapieoptionen im Off-label-Bereich dar.

Abstract

Background

Bullous pemphigoid, the most common autoimmune blistering disease of the skin in adults, is caused by autoantibodies against hemidesmosomal adhesion proteins (BP180/type XVII collagen and BP230), leading into subepidermal blistering. Therefore patients, mostly older than 70 years, show tight bullae and erosions of the skin and rarely at mucous membranes.

Problem

Usually the disease shows a chronically relapsing course. Thus there is a need for long-term topical corticosteroids and if necessary systemic immunosuppressives. Still there is no curative treatment available. In the context of a long-term treatment, drug-specific side effects and also a patient’s comorbidities have to be taken into account.

Conclusion

The choice of treatment should be based on disease activity and the extent of the muco-cutaneous manifestations. Dependent on this, high-potent topical class IV corticosteroids are used because of fewer side effects compared to systemic steroids. In case of an intense disease extent or in refractory courses treatment with systemic corticosteroids is usually combined with potentially corticosteroid-sparing immunomodulants such as dapsone or doxycycline or adjuvant immunosuppressives such as azathioprine, mycophenoles or methotrexate. Medium- to long-term an attendant immunosuppressant should be applied to reduce the use of corticosteroids. The CD20-antibody rituximab and high-dose intravenous immunoglobulins are also supplemental off-label options in refractory cases.

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Authors and Affiliations

  1. Klinik für Dermatologie und Allergologie, Universitätsklinikum Gießen und Marburg GmbH, Standort Marburg, Baldingerstr., 35043, Marburg, Deutschland

    M. Göbel & R. Eming

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  1. M. Göbel
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Correspondence to M. Göbel.

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Interessenkonflikt

M. Göbel und R. Eming geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren. Alle Patienten, die über Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts zu identifizieren sind, haben hierzu ihre schriftliche Einwilligung gegeben. Im Falle von nicht mündigen Patienten liegt die Einwilligung eines Erziehungsberechtigten oder des gesetzlich bestellten Betreuers vor.

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Göbel, M., Eming, R. Therapie des bullösen Pemphigoids. Hautarzt 70, 236–242 (2019). https://doi.org/10.1007/s00105-019-4390-z

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