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Epidermolysis Bullosa Acquisita

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Blistering Diseases

Abstract

Epidermolysis bullosa acquisita (EBA) is a rare subepidermal autoimmune bullous disease (AIBD) characterized by immune deposits on anchoring fibrils (AFs) of cutaneous and mucosal basement membrane zones (BMZ). It is due to circulating autoantibodies (Ab) directed to type VII collagen (C7). Clinical manifestations include a classical form with skin fragility, blisters and scars on trauma-prone surfaces, and misleading forms mimicking bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), or linear immunoglobulin (Ig) A disease (LAD). Prognosis is related to extent of cutaneous lesions and ocular, laryngeal, and/or esophageal involvements. A frequent association between EBA and Crohn’s disease has been observed. Specialized tests available in only certain laboratories are necessary to confirm its diagnosis such as immunoelectron microscopy (IEM), immunoblotting, or ELISA using recombinant proteins. This orphan disease is often misdiagnosed, and awareness programs for physicians and patients are mandatory.

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Abbreviations

Ab:

Antibodies

AFs:

Anchoring fibrils

AIBD:

Autoimmune bullous disease (AIBD)

BMZ:

Basement membrane zone

BP:

Bullous pemphigoid

C7:

Type VII collagen

EBA:

Epidermolysis bullosa acquisita

EBH:

Epidermolysis bullosa hereditaria

IEM:

Immunoelectron microscopy

IF:

Immunofluorescence

Ig:

Immunoglobulin

LAD:

Linear IgA disease

LD:

Lamina densa

MM:

Mucous membrane

MMP:

Mucous membrane pemphigoid

PCT:

Porphyria cutanea tarda

SSS:

Salt split skin

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Author information

Authors and Affiliations

  1. Department of Dermatology, Reference Center for Autoimmune Bullous Diseases, Avicenne Hospital, Bobigny, France

    Catherine Prost-Squarcioni MD, PhD & Frédéric Caux MD, PhD

  2. Department of Histology, UFR Léonard de Vinci—Paris 13, Bobigny, France

    Catherine Prost-Squarcioni MD, PhD

Authors
  1. Catherine Prost-Squarcioni MD, PhD
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  2. Frédéric Caux MD, PhD
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Corresponding author

Correspondence to Catherine Prost-Squarcioni MD, PhD .

Editor information

Editors and Affiliations

  1. Department of Dermatology, St George Hospital, University of New South Wales, Sydney, New South Wales, Australia

    Dédée F. Murrell

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Prost-Squarcioni, C., Caux, F. (2015). Epidermolysis Bullosa Acquisita. In: Murrell, D. (eds) Blistering Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45698-9_40

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  • DOI: https://doi.org/10.1007/978-3-662-45698-9_40

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