Comparison of 3 type VII collagen (C7) assays for serologic diagnosis of epidermolysis bullosa acquisita (EBA)

Seta, V.; Aucouturier, Françoise; Bonnefoy, Jonathan; Roux–Villet, C. Le; Pendaries, Valérie; Alexandre, M.; Grootenboer‐Mignot, Sabine; Heller, Michel; Lièvre, Nicole; Laroche, Liliane; Caux, F.; Titeux, Matthias; Hovnanian, Alain; Prost-Squarcioni, Catherine

doi:10.1016/j.jaad.2016.01.005

Cited by 28 publications

(39 citation statements)

References 31 publications

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“…Other investigators also showed sensitivities of the type VII collagen-specific BIOCHIP ® comparable with the corresponding ELISA [137,157]. As pointed out above, the sensitivity of detection assays for antitype VII collagen IgG may vary considerably depending on the analyzed patient cohort with circulating autoantibodies reported to be only present in about 60% EBA patients [26].…”

Section: Biochip ® Mosaicmentioning

confidence: 86%

“…In fact, ELISA values were shown to correlate with disease activity in EBA patients ( Figure 10) [150,152]. Recently, an in-house ELISA with a recombinant form of full-length type VII collagen was reported with a sensitivity of 65% [137].…”

Section: Enzyme-linked Immunosorbent Assaymentioning

confidence: 97%

“…Sera were, however, selected based on the clinical picture, and the presence of circulating autoantibodies by indirect IF microscopy on salt-split human skin and/or immunoblotting with cell-derived type VII collagen. When these ELISA were probed with EBA sera diagnosed by serration pattern analysis of direct IF microscopy or direct immunogold electron microscopy, much lower sensitivities of 53% and 30% are reported [137,150]. In EBA sera unreactive by indirect IF microscopy on human salt-split skin, ELISA reactivity could only be detected in 12% and 23% [137,150].…”

Section: Enzyme-linked Immunosorbent Assaymentioning

confidence: 99%

“…In latter two diseases, however, direct IF microscopy reveals an n-serrated pattern. Several studies have reported sensitivities of human salt-split skin for the detection of serum autoantibodies in EBA ranging from 57% to 100% with a mean of 64% in 191 EBA sera [26,27,137,[141][142][143]]. An elegant and relatively simple method for the detection of antitype VII collagen antibodies by indirect IF microscopy is the use of type VII collagen-deficient skin from patient with dystrophic epidermolysis bullosa.…”

Section: Indirect If Microscopymentioning

confidence: 99%

“…When these ELISA were probed with EBA sera diagnosed by serration pattern analysis of direct IF microscopy or direct immunogold electron microscopy, much lower sensitivities of 53% and 30% are reported [137,150]. In EBA sera unreactive by indirect IF microscopy on human salt-split skin, ELISA reactivity could only be detected in 12% and 23% [137,150]. Most recently, two multivariant ELISA systems became widely available (Euroimmun, MBL).…”

Section: Enzyme-linked Immunosorbent Assaymentioning

confidence: 99%

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Clinical features and diagnosis of epidermolysis bullosa acquisita

Vorobyev

Ludwig

Schmidt

2016

Expert Review of Clinical Immunology

100

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Introduction: Epidermolysis bullosa acquisita (EBA) is a rare autoimmune blistering disease of skin and mucous membranes. EBA is caused by autoantibodies against type VII collagen, which is a major component of anchoring fibrils, attaching epidermis to dermis. Binding of autoantibodies to type VII collagen leads to skin fragility and, finally, blister formation. The clinical picture of EBA is polymorphic, with several distinct phenotypes being described. Despite recent progress in understanding the pathophysiology of EBA, its diagnosis is still challenging. Areas covered: This review provides an update on the clinical manifestations and diagnostic methods of EBA. We searched PubMed using the terms 'epidermolysis bullosa acquisita' covering articles in English between 1 January 2005 and 31 May 2016. Relevant older publications were retrieved form cited literature. Expert commentary: While the clinical picture is highly variable, diagnosis relies on direct immunofluorescence (IF) microscopy of a perilesional skin biopsy. Linear deposits of IgG, IgA and/or C3 along the dermal-epidermal junction with an u-serrated pattern are diagnostic for EBA alike the detection of serum autoantibodies against type VII collagen. Several test systems for the serological diagnosis of EBA have recently become widely available. In some patients, sophisticated diagnostic approaches only available in specialized centers are required. ARTICLE HISTORY

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Section: Biochip ® Mosaicmentioning

confidence: 86%

Section: Enzyme-linked Immunosorbent Assaymentioning

confidence: 97%

Section: Enzyme-linked Immunosorbent Assaymentioning

confidence: 99%

Section: Indirect If Microscopymentioning

confidence: 99%

Section: Enzyme-linked Immunosorbent Assaymentioning

confidence: 99%

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Clinical features and diagnosis of epidermolysis bullosa acquisita

Vorobyev

Ludwig

Schmidt

2016

Expert Review of Clinical Immunology

100

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Immunobullous Diseases

Schmidt,

Groves

2024

Rook's Textbook of Dermatology

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The immunobullous disorders represent a group of conditions characterised by antibody‐mediated autoimmune responses against structural elements of the skin resulting in blistering of the skin and/or mucosae. Antibody targets include proteins in the hemidesmosomes basement membrane zone and superficial dermis (pemphigoid group), desmosomes (pemphigus group) and epidermal and tissue‐type transglutaminase (dermatitis herpetiformis). If left untreated, immunobullous disorders are associated with significant morbidity and mortality and thus prompt, accurate diagnosis and treatment are mandatory. Therapeutic options include corticosteroids, steroid‐sparing immunosuppressants such as azathioprine, mycophenolate mofetil and anti‐inflammatory agents such as dapsone and tetracyclines. In severe and/or refractory patients, intravenous immunoglobulin, immunoadsorption and anti‐B‐cell agents such as rituximab may be of benefit. Indeed, recently international and European guidelines have recommended the use of rituximab as first line treatment in moderate and severe pemphigus vulgaris and foliaceous.

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