Epidermolysis bullosa acquisita

J. H. Kim, S. C. Kim

Research output: Contribution to journalReview article

32 Citations (Scopus)

Abstract

Epidermolysis bullosa acquisita (EBA) is a chronic autoimmune subepidermal bullous disease with clinical features similar to the genetic form of dystrophic epidermolysis bullosa. EBA is characterized by the presence of autoantibodies against type VII collagen which is a major component of the anchoring fibrils at the dermal-epidermal junction. EBA can be divided into two main clinical types; mechanobullous and inflammatory EBA. Mechanobullous EBA, referred to as classic EBA, presents with skin fragility, blisters and dystrophic changes on trauma-prone areas. Inflammatory EBA resembles other autoimmune subepidermal bullous diseases. Compelling evidence from mouse models supports a pathogenic role of autoantibodies against type VII collagen in EBA. Treatment of EBA is often unsatisfactory. The most widely used systemic treatment is corticosteroids. Colchicine and dapsone have been reported to be good treatment modalities when combined with corticosteroids. Some intractable cases of EBA have successfully been treated with intravenous immunoglobulin or rituximab.

Original languageEnglish
Pages (from-to)1204-1213
Number of pages10
JournalJournal of the European Academy of Dermatology and Venereology
Volume27
Issue number10
DOIs
Publication statusPublished - 2013 Oct 1

Fingerprint

Epidermolysis Bullosa Acquisita
Collagen Type VII
Autoantibodies
Adrenal Cortex Hormones
Epidermolysis Bullosa Dystrophica
Dapsone
Skin
Intravenous Immunoglobulins
Colchicine
Blister

All Science Journal Classification (ASJC) codes

  • Dermatology
  • Infectious Diseases

Cite this

@article{b6365bb8a49745f89e191deb60259b2e,
title = "Epidermolysis bullosa acquisita",
abstract = "Epidermolysis bullosa acquisita (EBA) is a chronic autoimmune subepidermal bullous disease with clinical features similar to the genetic form of dystrophic epidermolysis bullosa. EBA is characterized by the presence of autoantibodies against type VII collagen which is a major component of the anchoring fibrils at the dermal-epidermal junction. EBA can be divided into two main clinical types; mechanobullous and inflammatory EBA. Mechanobullous EBA, referred to as classic EBA, presents with skin fragility, blisters and dystrophic changes on trauma-prone areas. Inflammatory EBA resembles other autoimmune subepidermal bullous diseases. Compelling evidence from mouse models supports a pathogenic role of autoantibodies against type VII collagen in EBA. Treatment of EBA is often unsatisfactory. The most widely used systemic treatment is corticosteroids. Colchicine and dapsone have been reported to be good treatment modalities when combined with corticosteroids. Some intractable cases of EBA have successfully been treated with intravenous immunoglobulin or rituximab.",
author = "Kim, {J. H.} and Kim, {S. C.}",
year = "2013",
month = "10",
day = "1",
doi = "10.1111/jdv.12096",
language = "English",
volume = "27",
pages = "1204--1213",
journal = "Journal of the European Academy of Dermatology and Venereology",
issn = "0926-9959",
publisher = "Wiley-Blackwell",
number = "10",

}

Epidermolysis bullosa acquisita. / Kim, J. H.; Kim, S. C.

In: Journal of the European Academy of Dermatology and Venereology, Vol. 27, No. 10, 01.10.2013, p. 1204-1213.

Research output: Contribution to journalReview article

TY - JOUR

T1 - Epidermolysis bullosa acquisita

AU - Kim, J. H.

AU - Kim, S. C.

PY - 2013/10/1

Y1 - 2013/10/1

N2 - Epidermolysis bullosa acquisita (EBA) is a chronic autoimmune subepidermal bullous disease with clinical features similar to the genetic form of dystrophic epidermolysis bullosa. EBA is characterized by the presence of autoantibodies against type VII collagen which is a major component of the anchoring fibrils at the dermal-epidermal junction. EBA can be divided into two main clinical types; mechanobullous and inflammatory EBA. Mechanobullous EBA, referred to as classic EBA, presents with skin fragility, blisters and dystrophic changes on trauma-prone areas. Inflammatory EBA resembles other autoimmune subepidermal bullous diseases. Compelling evidence from mouse models supports a pathogenic role of autoantibodies against type VII collagen in EBA. Treatment of EBA is often unsatisfactory. The most widely used systemic treatment is corticosteroids. Colchicine and dapsone have been reported to be good treatment modalities when combined with corticosteroids. Some intractable cases of EBA have successfully been treated with intravenous immunoglobulin or rituximab.

AB - Epidermolysis bullosa acquisita (EBA) is a chronic autoimmune subepidermal bullous disease with clinical features similar to the genetic form of dystrophic epidermolysis bullosa. EBA is characterized by the presence of autoantibodies against type VII collagen which is a major component of the anchoring fibrils at the dermal-epidermal junction. EBA can be divided into two main clinical types; mechanobullous and inflammatory EBA. Mechanobullous EBA, referred to as classic EBA, presents with skin fragility, blisters and dystrophic changes on trauma-prone areas. Inflammatory EBA resembles other autoimmune subepidermal bullous diseases. Compelling evidence from mouse models supports a pathogenic role of autoantibodies against type VII collagen in EBA. Treatment of EBA is often unsatisfactory. The most widely used systemic treatment is corticosteroids. Colchicine and dapsone have been reported to be good treatment modalities when combined with corticosteroids. Some intractable cases of EBA have successfully been treated with intravenous immunoglobulin or rituximab.

UR - http://www.scopus.com/inward/record.url?scp=84884902879&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84884902879&partnerID=8YFLogxK

U2 - 10.1111/jdv.12096

DO - 10.1111/jdv.12096

M3 - Review article

C2 - 23368767

AN - SCOPUS:84884902879

VL - 27

SP - 1204

EP - 1213

JO - Journal of the European Academy of Dermatology and Venereology

JF - Journal of the European Academy of Dermatology and Venereology

SN - 0926-9959

IS - 10

ER -