Skip to main content
SpringerLink
Log in

Epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita

  • Leitthema
  • Published:
Der Hautarzt Aims and scope Submit manuscript

Zusammenfassung

Die Epidermolysis bullosa acquisita (EBA) ist eine seltene bullöse Autoimmundermatose, die assoziiert ist mit Autoantikörpern gegen Kollagen VII, dem Hauptbestandteil der Verankerungsfibrillen der Dermis. Die subepidermale Blasenbildung wird durch Bindung der Autoantikörper an Kollagen VII mit nachfolgender komplementabhängiger Neutrophilenchemotaxis und Freisetzung von Proteasen ausgelöst. Die häufigste klinische Variante der EBA, der mechanobullöse Typ, ist durch pralle Blasen an mechanisch belasteten Körperarealen mit narbiger Abheilung gekennzeichnet. Der zweithäufigste klinische Typ entspricht einer inflammatorischen Variante, die klinisch nicht vom bullösen Pemphigoid unterscheidbar ist und mit Juckreiz einhergeht. Eine Schleimhautbeteiligung oder/und Läsionen an Kopf- und Halsregion können zusätzliche Hinweise auf diese Diagnose geben. Insbesondere der mechanobullöse Typ der EBA sowie die EBA mit ausgeprägten Schleimhautläsionen verlaufen oft immanent chronisch und sind äußerst therapieresistent. Zum Einsatz kommen topische und systemische Glukokortikosteroide, Dapson, Colchicin, klassische Immunsuppressiva, Anti-CD20-Antikörper, Immunadsorption oder intravenöse Immunglobuline.

Abstract

Epidermolysis bullosa acquisita (EBA) is a rare acquired subepidermal bullous autoimmune dermatosis, associated with autoantibodies against collagen type VII, the most important component of dermal anchoring fibrils. Blister induction occurs after binding of autoantibodies to collagen type VII, leading to complement activation, recruitment of neutrophils and secretion of proteases. Clinically, the disease is mostly characterized by tense blisters on trauma-exposed body areas which heal with scarring (mechanobullous form of EBA). The second most frequent subtype of EBA is inflammatory EBA, a bullous pemphigoid-like disease associated with pruritus. Involvement of mucous membranes and/or lesions in the head and neck area additionally point to the diagnosis of EBA. The mechanobullous type of EBA and EBA with intensive mucous membrane lesions display a chronic course and are often extremely resistant to therapy. Topical and systemic glucocorticoids, dapsone, colchicine, classical immunosuppressants, anti-CD20 antibodies, immunoadsorption or intravenous immunoglobulins have been reported as treatments.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1
Abb. 2
Abb. 3

Literatur

  1. Asfour L, Chong H, Mee J et al (2017) Epidermolysis Bullosa Acquisita (Brunsting-Perry Pemphigoid variant) localized to the face and diagnosed with antigen identification using skin deficient in type VII collagen. Am J Dermatopathol 39:e90–e96

    Article  Google Scholar 

  2. Bernard P, Vaillant L, Labeille B et al (1995) Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group. Arch Dermatol 131:48–52

    Article  CAS  Google Scholar 

  3. Delgado L, Aoki V, Santi C et al (2011) Clinical and immunopathological evaluation of epidermolysis bullosa acquisita. Clin Exp Dermatol 36:12–18

    Article  CAS  Google Scholar 

  4. Goletz S, Zillikens D, Schmidt E (2017) Structural proteins of the dermal-epidermal junction targeted by autoantibodies in pemphigoid diseases. Exp Dermatol 26:1154–1162

    Article  CAS  Google Scholar 

  5. Hoffmann K, Hertl M, Sitaru C (2016) Molecular diagnosis of autoimmune dermatoses. Hautarzt 67:33–39

    Article  CAS  Google Scholar 

  6. Hofmann SC, Juratli HA, Eming R (2018) Bullous autoimmune dermatoses. J Dtsch Dermatol Ges 16:1339–1358

    PubMed  Google Scholar 

  7. Hubner F, Recke A, Zillikens D et al (2016) Prevalence and age distribution of pemphigus and pemphigoid diseases in Germany. J Invest Dermatol 136:2495–2498

    Article  Google Scholar 

  8. Intong LR, Murrell DF (2011) Management of epidermolysis bullosa acquisita. Dermatol Clin 29:643–647

    Article  CAS  Google Scholar 

  9. Iwata H, Vorobyev A, Koga H et al (2018) Meta-analysis of the clinical and immunopathological characteristics and treatment outcomes in epidermolysis bullosa acquisita patients. Orphanet J Rare Dis 13:153

    Article  Google Scholar 

  10. Kasperkiewicz M, Sadik CD, Bieber K et al (2016) Epidermolysis Bullosa Acquisita: from pathophysiology to novel therapeutic options. J Invest Dermatol 136:24–33

    Article  CAS  Google Scholar 

  11. Kirtschig G, Murrell D, Wojnarowska F et al (2003) Interventions for mucous membrane pemphigoid and epidermolysis bullosa acquisita. Cochrane Database Syst Rev. https://doi.org/10.1002/14651858.CD004056

    Article  PubMed  Google Scholar 

  12. Koga H, Prost-Squarcioni C, Iwata H et al (2018) Epidermolysis Bullosa Acquisita: the 2019 update. Front Med 5:362

    Article  Google Scholar 

  13. Komorowski L, Muller R, Vorobyev A et al (2013) Sensitive and specific assays for routine serological diagnosis of epidermolysis bullosa acquisita. J Am Acad Dermatol 68:e89–e95

    Article  CAS  Google Scholar 

  14. Ludwig RJ (2017) Signalling and targeted therapy of inflammatory cells in epidermolysis bullosa acquisita. Exp Dermatol 26:1179–1186

    Article  CAS  Google Scholar 

  15. Luke MC, Darling TN, Hsu R et al (1999) Mucosal morbidity in patients with epidermolysis bullosa acquisita. Arch Dermatol 135:954–959

    Article  CAS  Google Scholar 

  16. Meijer JM, Atefi I, Diercks GFH et al (2018) Serration pattern analysis for differentiating epidermolysis bullosa acquisita from other pemphigoid diseases. J Am Acad Dermatol 78:754–759.e6

    Article  Google Scholar 

  17. Prost-Squarcioni C, Caux F, Schmidt E et al (2018) International Bullous Diseases Group: consensus on diagnostic criteria for epidermolysis bullosa acquisita. Br J Dermatol 179:30–41

    Article  CAS  Google Scholar 

  18. Schmidt E, Zillikens D (2011) The diagnosis and treatment of autoimmune blistering skin diseases. Dtsch Arztebl Int 108:399–405

    PubMed  PubMed Central  Google Scholar 

  19. Schmidt T, Hoch M, Lotfi Jad SS et al (2017) Serological diagnostics in the detection of IgG autoantibodies against human collagen VII in epidermolysis bullosa acquisita: a multicentre analysis. Br J Dermatol 177:1683–1692

    Article  CAS  Google Scholar 

  20. Sebaratnam DF, Frew JW, Davatchi F et al (2012) Quality-of-life measurement in blistering diseases. Dermatol Clin 30:301–307

    Article  CAS  Google Scholar 

  21. Van Beek N, Zillikens D, Schmidt E (2018) Diagnostik blasenbildender Autoimmundermatosen. J Dtsch Dermatol Ges 16:1077–1092

    PubMed  Google Scholar 

  22. Vodegel RM, De Jong MC, Pas HH et al (2002) IgA-mediated epidermolysis bullosa acquisita: two cases and review of the literature. J Am Acad Dermatol 47:919–925

    Article  Google Scholar 

  23. Vorobyev A, Ludwig RJ, Schmidt E (2017) Clinical features and diagnosis of epidermolysis bullosa acquisita. Expert Rev Clin Immunol 13:157–169

    Article  CAS  Google Scholar 

  24. Zumelzu C, Le Roux-Villet C, Loiseau P et al (2011) Black patients of African descent and HLA-DRB1*15:03 frequency overrepresented in epidermolysis bullosa acquisita. J Invest Dermatol 131:2386–2393

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Zentrum für Dermatologie, Allergologie und Dermatochirurgie, HELIOS Universitätsklinikum Wuppertal, Universität Witten/Herdecke, Heusnerstr. 40, 42283, Wuppertal, Deutschland

    S. C. Hofmann

  2. Universitäts-Hautklinik Kiel, Klinik für Dermatologie, Venerologie und Allergologie, UKSH Kiel, Kiel, Deutschland

    A. Weidinger

Authors
  1. S. C. Hofmann
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. A. Weidinger
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to S. C. Hofmann.

Ethics declarations

Interessenkonflikt

S.C. Hofmann und A. Weidinger geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren. Für Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts, über die Patienten zu identifizieren sind, liegt von ihnen und/oder ihren gesetzlichen Vertretern eine schriftliche Einwilligung vor.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hofmann, S.C., Weidinger, A. Epidermolysis bullosa acquisita. Hautarzt 70, 265–270 (2019). https://doi.org/10.1007/s00105-019-4387-7

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00105-019-4387-7

Schlüsselwörter

Keywords

Search

Navigation