Post‐orf epidermolysis bullosa acquisita

Daneshpazhooh, Maryam; Mahmoudi, Hamidreza; Toosi, Roja; Tavakolpour, Soheil; Schmidt, Enno; Zillikens, Detlef

doi:10.1111/jdv.15299

Cited by 5 publications

(12 citation statements)

References 3 publications

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“…3,11 All patients with mucosal involvement presented with oral lesions followed by involvement of nostrils (3 of 13 [23%]) and conjunctivae (2 of 13 [15%]). To date, the target antigen in orf-triggered AIBD has remained unknown in all but 3 cases, which included reactivity against Col7 (cases 5 and 12) 6,7 and against laminin 332 (case 4). 5 Here, we describe in detail a patient with orf-induced antilaminin 332 (bullous) pemphigoid and detected serum antilaminin 332 reactivity in another 4 previously reported patients with orf-induced AIBD.…”

Section: Discussionmentioning

confidence: 99%

“…However, human orf has recently generated considerable interest owing to increasing evidence of its role in inducing autoimmune bullous diseases (AIBDs). [1][2][3][4][5][6][7] Previously reported cases of orf-induced AIBDs have all been characterized as subepidermal blistering disorders. Hereby, all but 1 patient showed mucocutaneous lesions with predominant skin involvement.…”

Section: Clinical and Serological Characterization Of Orf-induced Imm...mentioning

confidence: 99%

“…All serum samples except case 12 revealed IgG autoantibodies against laminin 332 (Table ; eFigures 3-6 in the Supplement). 7…”

Section: Reactivity Against Laminin 332mentioning

confidence: 99%

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Clinical and Serological Characterization of Orf-Induced Immunobullous Disease

et al. 2022

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IMPORTANCE Ecthyma contagiosum, or orf, is a viral zoonotic infection caused by Poxviridae. Although human orf infection is considered to follow a self-limited course, various immunological reactions may be triggered, including immunobullous diseases. In the majority of the latter cases, the antigenic target remained enigmatic.OBJECTIVE To characterize the predominant autoantigen in orf-induced immunobullous disease and further describe this clinical entity. DESIGN, SETTING, AND PARTICIPANTSThis multicenter case series sought to provide detailed clinical, histopathological and immunological characteristics of a patient with orf-induced pemphigoid. Based on this index patient, serological analyses were conducted of 4 additional patients with previously reported orf-induced immunobullous disease. Immunoblotting with extracellular matrix and a recently established indirect immunofluorescence assay for detection of serum anti-laminin 332 IgG were performed.EXPOSURES The disease course and clinical characteristics of orf-induced immunobullous disease were observed. MAIN OUTCOMES AND MEASURESOrf-induced immunobullous disease is primarily characterized by anti-laminin 332 autoantibodies, predominant skin involvement, and a self-limiting course. The study provides further details on epidemiological, clinical, immunopathological, diagnostic, and therapeutic aspects of orf-induced immunobullous disease. RESULTSIn all 5 patients, IgG1 and/or IgG3 autoantibodies against laminin 332 were identified. The α3, β3, and γ2 chains were recognized in 2, 4, and 1 patient(s), respectively. CONCLUSIONS AND RELEVANCEIn this case series, laminin 332, a well-known target antigen in mucous membrane pemphigoid, was a major autoantigen in orf-induced immunobullous disease, even though predominant mucosal lesions were lacking in this autoimmune blistering disease. Orf-induced anti-laminin 332 pemphigoid is proposed as distinct clinical entity.

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Section: Discussionmentioning

confidence: 99%

Section: Clinical and Serological Characterization Of Orf-induced Imm...mentioning

confidence: 99%

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Clinical and Serological Characterization of Orf-Induced Immunobullous Disease

et al. 2022

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“…There is a plethora of serological studies with commercial and in F I G U R E 2 Test evaluation: (A) distribution of anti-Dsg3 IgG values for controls (n = 104) and PV (n = 104) sera (cut-off 20 RE/mL; one value located outside the axis limit: 3555 RE/mL) (B) Distribution of POCScan anti-Dsg3 IgG values for controls (n = 104) and PV (n = 96) sera (cut-off 60 mV) (C) ROC analysis based on sensitivity and specificity of POCScan results (D) 10 × 10 dot plots for specificity (control) and sensitivity (PV) for the combination of human observer groups A and B loco developed Dsg3 ELISA for the serological detection of antidesmoglein 3 IgG autoantibodies. [6][7][8][9][10][11][12][13][14] With 78.1% (observers A and B) and 72.9% (POCScan), the sensitivity of the Dsg3 LFIA is lower compared to the available ELISA system, which show a sensitivity between 85 and 100%. The specificity of the Dsg3 LFIA was in the range of 94.2% (POCScan) to 97.1% (observers A + B), while the specificity of the Dsg3 ELISA ranged from 95.7% to 100%.…”

Section: Discussionmentioning

confidence: 99%

“…anti-Dsg3 IgG, lateral flow immunoassay, pemphigus vulgaris detection of anti-Dsg1/3 autoantibodies. [1] As ELISA index correlates with the extent or activity of the disease, [6][7][8][9][10][11][12][13][14] this method represents the "gold standard" for the serological detection of anti-Dsg1/3 IgG, respectively. [15] Nevertheless, due to the rareness of the disease (incidence of 1-5/10 6 ), diagnostic ELISA is only performed in specialized clinical centres and requires an adequately equipped serological laboratory.…”

Section: Introductionmentioning

confidence: 99%

A novel lateral flow immunoassay for the rapid detection of anti‐Dsg3 IgG serum autoantibodies in pemphigus vulgaris

Schmidt

Mauracher

Bender

et al. 2018

Experimental Dermatology

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Pemphigus vulgaris (PV) is a severe autoimmune blistering disease of the skin and mucous membranes. As autoantibodies play an essential role in the disease pathogenesis, the serological detection of anti-desmoglein 3 IgG represents a central tool in the diagnosis of the disease. In this study, we show the validation of a novel lateral flow immunoassay (LFIA) which rapidly detects anti-desmoglein 3 (Dsg3) IgG in human serum. In contrast to other diagnostic procedures, the assay is compact and simple to perform and delivers a fast "yes" or "no" answer within 10 minutes without additional hardware requirements for test evaluation. For validation, a blinded collection of 200 sera including 100 sera from 14 PV patients, 75 sera from 24 bullous pemphigoid patients and 25 sera from 6 patients with pemphigus foliaceus collected at different time points during disease was used. Presence or non-presence of anti-Dsg3 IgG within sera was confirmed using a commercially available Dsg3-ELISA. For qualitative evaluation, Dsg3-LFIA test results were assessed by two independent groups of human observers. Furthermore, quantitative evaluation using POCScan reader was applied. The Dsg3-LFIA demonstrated reliable test results with a sensitivity and specificity of 78.1% and 97.1%, respectively. Test results from POCScan and human observers showed a substantial agreement. The Dsg3-LFIA represents a new diagnostic tool for the immediate and reliable detection of anti-desmoglein 3 serum IgG autoantibodies that does not require additional hardware. Further prospective trials are warranted to validate the Dsg3 LFIA in pemphigus.

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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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Post‐orf epidermolysis bullosa acquisita

Cited by 5 publications

References 3 publications

Clinical and Serological Characterization of Orf-Induced Immunobullous Disease

Clinical and Serological Characterization of Orf-Induced Immunobullous Disease

A novel lateral flow immunoassay for the rapid detection of anti‐Dsg3 IgG serum autoantibodies in pemphigus vulgaris

Immunobullous Diseases

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