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Der Hautarzt

, Volume 64, Issue 12, pp 931–945 | Cite as

Bullöses Pemphigoid

  • F. SchulzeEmail author
  • M. Kasperkiewicz
  • D. Zillikens
  • E. Schmidt
CME Zertifizierte Fortbildung

Zusammenfassung

Pemphigoiderkrankungen sind eine Gruppe von Autoimmunerkrankungen, die charakterisiert sind durch eine subepidermale Blasenbildung sowie Autoantikörper gegen Strukturproteine der dermoepidermalen Junktionszone. Beim bullösen Pemphigoid, der häufigsten subepidermal Blasen bildenden Autoimmundermatose, sind die Antikörper gegen die hemidesmosomalen Antigene BP180 (Kollagen Typ XVII) und BP230 gerichtet. Klinisch charakteristisch sind der starke Juckreiz sowie pralle Blasen, Erosionen und Krusten bei älteren Patienten. Diagnostisch wegweisend sind lineare Ablagerungen von IgG und/oder C3 an der dermoepidermalen Junktionszone in der direkten immunfluoreszenzoptischen Untersuchung einer periläsionalen Hautprobe und der serologische Nachweis von Autoantikörpern mittels indirekter Immunfluoreszenz auf humaner Spalthaut sowie im ELISA unter Verwendung von rekombinanten immundominanten Fragmenten von BP180 und BP230. Therapeutische Optionen beinhalten topische (Klasse IV) und/oder systemische Glukokortikosteroide, die meist in Kombination mit Immunmodulatoren wie Dapson und Tetrazyklinen oder Immunsuppressiva wie Methotrexat und Azathioprin angewendet werden.

Schlüsselwörter

Autoantikörper Blasen BP180 ELISA Immunfluoreszenz 

Bullous pemphigoid

Abstract

Pemphigoid diseases are a group of autoimmune disorders characterized by subepidermal blistering and autoantibodies against structural proteins of the dermal-epidermal junction. In bullous pemphigoid, the most common subepidermal blistering autoimmune disease, antibodies are directed against the hemidesmosomal antigens BP180 (collagen type XVII) and BP230. Bullous pemphigoid typically presents with severe pruritus and tense blisters accompanied by erosions and crusts in elderly patients. Diagnostic landmarks are the detection of linear IgG and/or C3 deposits at the dermo-epidermal junction by direct immunofluorescence microscopy of a perilesional biopsy and the detection of serum autoantibodies by indirect immunofluorescence microscopy on human salt-split skin and ELISA employing recombinant immunodominant fragments of BP180 and BP230. Treatment options include topical (class IV) and/or systemic corticosteroids, frequently combined with immunomodulatory agents like dapsone and tetracyclines or immunosuppressants such as methotrexate and azathioprine.

Keywords

Autoantibodies Blister BP180 ELISA Immunofluorescence 

Notes

Danksagung

Diese Arbeit wurde unterstützt durch das Exzellenzcluster Inflammation@Interfaces (DFG EXC 306/2). Für die Überlassung der Fotos zu Abb. 5 bedanken wir uns bei Euroimmun AG, Lübeck.

Einhaltung ethischer Richtlinien

Interessenkonflikt. Wissenschaftliche Kooperationen bestehen mit Euroimmun, Fresenius Medical Care und Miltenyi (D. Zillikens und E. Schmidt), Biotest (D. Zillikens) und Novartis (E. Schmidt). D. Zillikens und E. Schmidt erhielten zudem Honorare von Fresenius Medical Care, Miltenyi, Biotest und Roche. F. Schulze erhielt Honorare von Fresenius Medical Care und Biotest. M. Kasperkiewicz erhielt Honorare von Fresenius Medical Care, Biotest und Miltenyi. Dieser Beitrag beinhaltet keine 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 Erziehungsberechtigen oder des gesetzlich bestellten Betreuers vor.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • F. Schulze
    • 1
    Email author
  • M. Kasperkiewicz
    • 1
  • D. Zillikens
    • 1
  • E. Schmidt
    • 1
  1. 1.Klinik für Dermatologie, Allergologie und VenerologieUniversität zu LübeckLübeckDeutschland

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