Epidermolysis Bullosa Acquisita and Inflammatory Bowel Disease

Raab, Benjamin

doi:10.1001/jama.1983.03340130064034

Cited by 23 publications

(7 citation statements)

References 1 publication

(1 reference statement)

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“…We found 42 cases of patients with coexisting EBA and IBD in the literature, from 1969 onwards. Of the 42 cases, individual clinical data could be extracted for 20 cases: 23 patients had CD and EBA, [12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31] (Table 1) and 6 had UC and EBA 6,[30][31][32][33] (Table 2). The demographic features of these cases were collected (Table 3).…”

Section: Resultsmentioning

confidence: 99%

Epidermolysis bullosa acquisita and inflammatory bowel disease: a review of the literature

Reddy

Shipman

Wojnarowska

2013

Clinical and Experimental Dermatology

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SummaryWe present a review of all previously reported cases of epidermolysis bullosa acquisita (EBA) and inflammatory bowel disease (IBD). We found 42 cases of coincident EBA and IBD in the literature: 35 cases of Crohn disease (CD) and 7 of ulcerative colitis (UC). The clinical and immunopathological features of the cases are described and the demographics collected. In the majority of cases, the diagnosis of IBD predated the development of the skin condition. The association between EBA and IBD was more common for CD than for UC. We discuss the immunopathogenesis of IBD and EBA, and also the link between them, namely type VII collagen.

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Section: Resultsmentioning

confidence: 99%

Epidermolysis bullosa acquisita and inflammatory bowel disease: a review of the literature

Reddy

Shipman

Wojnarowska

2013

Clinical and Experimental Dermatology

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“…Full-length type VII collagen is normally expressed in colonic epithelium. 28,29 EB acquisita (EBA) is an autoimmune blistering disease of the skin characterized by IgG autoantibodies against type VII collagen, 30 and is observed with greater than expected frequency in patients with Crohn disease 29,[31][32][33] and is also reported with ulcerative colitis. 34 However, it is not understood why some patients with IBD develop EBA whereas others do not.…”

Section: Discussionmentioning

confidence: 99%

Gastrointestinal complications of epidermolysis bullosa in children

Freeman¹,

Köglmeier²,

Martinéz³

et al. 2008

Br J Dermatol

108

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“…Indirect IF and immunoblotting were repeated and showed IgA at the dermal side of salt split skin and IgA-binding type VII collagen, as observed previously. Because of the appearance of a perianal fissure and the reported association of EBA with inflammatory bowel disease, 5,6 an exploration of the gastrointestinal tract was carried out. Colonoscopy and upper endoscopy with multiple biopsies, were normal.…”

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confidence: 99%

IgA‐epidermolysis bullosa acquisita in a child resulting in blindness

Caux

Kirtschig

Lemarchand-Vénencie

et al. 1997

British Journal of Dermatology

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Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disease characterized by IgG autoantibodies directed against type VII collagen, the major component of anchoring fibrils. The classical phenotype of EBA is a non-inflammatory, mechanobullous disease resembling the dystrophic forms of inherited epidermolysis bullosa. Mucous membrane involvement is frequent but usually mild. We report a 1-year-old girl suffering from IgA-EBA, who presented with an initial eruption of disseminated urticarial lesions and tense blisters of the skin but subsequently developed severe oral and ocular lesions reminiscent of cicatricial pemphigoid. Direct immunofluorescence of the skin and buccal mucosa revealed linear IgA and C3 at the basement membrane zone (BMZ). IgA anti-BMZ autoantibodies stained the dermal side of salt-split skin by indirect immunofluorescence and recognized a dermal protein of 290 kDa co-migrating with type VII collagen by immunoblotting. Direct and indirect immunoelectron microscopy revealed IgA deposits overlying the anchoring fibrils. The ocular involvement led to total blindness in spite of intense treatment. This case of childhood IgA-EBA is particularly striking because of the cicatricial pemphigoid phenotype with severe ocular involvement which resulted in blindness. It reinforces the necessity to use modern immunological methods to classify autoimmune bullous diseases in order to allow early and appropriate treatment.

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Epidermolysis Bullosa Acquisita and Inflammatory Bowel Disease

Cited by 23 publications

References 1 publication

Epidermolysis bullosa acquisita and inflammatory bowel disease: a review of the literature

Epidermolysis bullosa acquisita and inflammatory bowel disease: a review of the literature

Gastrointestinal complications of epidermolysis bullosa in children

IgA‐epidermolysis bullosa acquisita in a child resulting in blindness

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