ReportsAnti-epiligrin cicatricial pemphigoid and epidermolysis bullosa acquisita: Differentiation by use of indirect immunofluorescence microscopy☆,☆☆,★,★★,♢
Section snippets
Serum characterization
Serum samples were collected from 4 patients with AECP and 6 patients with mechanobullous IgG-mediated EBA. The sera had been characterized by immunoblotting, immunoprecipitation, or both as described previously.8, 12 The AECP sera reacted with the α3 subunit of laminin 5 in immunoblotting. The EBA sera reacted in immunoblot using dermal extract with a 290-kd antigen corresponding with type VII collagen. By IIF, using 1-mol/L salt-split skin,13 all 10 sera showed binding of IgG4 antibodies
Indirect immunofluorescence microscopy on knockout skin
IIF with AECP sera showed negative or faint staining on JEB-H skin (the faint staining being a result of cross-reactivity of the autoantibodies to laminin 6 of the epidermal BMZ in laminin 5-deficient skin) (Fig 1, A).
Discussion
In this study we describe two IIF methods that differentiate AECP and EBA, subepidermal bullous dermatoses characterized by dermal-binding IgG anti-BMZ autoantibodies directed against the dermal side of 1 mol/L salt-split skin. The first IIF method using knockout skin differentiated between AECP and EBA by determining loss of IgG binding to laminin 5- or type VII collagen-deficient skin substrates. Laminin 5-negative skin was obtained from a patient with JEB-H. The loss of laminin 5 in such
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Cited by (25)
Epidermolysis bullosa acquisita
2022, Anais Brasileiros de DermatologiaCitation Excerpt :The sensitivity varies from 20% to 80%, according to the COLVII source and the type of NC domain used.44 False-negative results may occur due to conformational changes in epitopes during the preparation of the recombinant proteins.51 Circulating anti-COLVII IgG can be detected after incubation of the serum with human cells transfected with the NC1 domain.
Mucous membrane pemphigoid
2022, Autoimmunity ReviewsCitation Excerpt :Several other in-house tests are in use in specialized laboratories [55,88,183]. Indirect IF microscopy on laminin 332-deficient skin is another elegant method for the detection of anti-laminin 332 antibodies [184]. This method is, however limited to sera reactive with human skin and those that do not contain antibodies against another antigen of the BMZ.
Epidermolysis bullosa acquisita: A comprehensive review
2019, Autoimmunity ReviewsCitation Excerpt :Using IF and perilesional skin from patients with EBA, digitized color images are formed by staining lamina lucida IgG deposits one color, against collagen VII, a different color. In patients with EBA there is an overlap of the fluorescence whereas in patients with BP, for example, the two stains are seen to be separate [80,92]. With immunoelectron microscopy (IEM), deposition of IgG beneath the basal lamina in the dermis of perilesional skin is visualized [7].
Differentiating antiepiligrin cicatricial pemphigoid from epidermolysis bullosa acquisita by indirect immunofluorescence of skin substrates lacking Type VII collagen or laminin 332: A case report and review of literature
2011, Dermatologica SinicaCitation Excerpt :However, these are often time consuming and expensive. Diagnostic indirect IF on antigen-deficient substrates2,3 of Type VII collagen-deficient or laminin 332-deficient skin from patients with inherited dystrophic and junctional epidermolysis bullosa, respectively, can provide a rapid and simple test to help clinicians differentiate between AECP and EBA. If indirect IF on antigen-deficient skin substrates show positive reaction on Type VII collagen-deficient skin and negative finding on laminin 332-deficient skin, this demonstrates that the patient’s serum contains antilaminin 332 autoantibodies instead of anti-Type VII collagen autoantibodies.
The vesiculobullous reaction pattern
2009, Weedon's Skin Pathology: Third Edition
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Funding sources: Zon/MW.
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Conflict of interest: None identified.
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Reprints not available from authors.
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Correspondence: Prof Dr Marcel F. Jonkman, MD, Department of Dermatology, Groningen University Hospital, PO Box 30.001, 9700 RB Groningen, the Netherlands. E-mail:[email protected].
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