Diagnosis and disease severity assessment of epidermolysis bullosa acquisita by ELISA for anti-type VII collagen autoantibodies: an Italian multicentre study

Marzano, Angelo Valerio; Cozzani, Emanuele; Fanoni, Daniele; Pità, Ornella De; Vassallo, Camilla; Berti, Emilio; Parodi, Aurora; Crosti, C.; Cugno, Massimo

doi:10.1111/bjd.12011

Cited by 32 publications

(33 citation statements)

References 17 publications

(23 reference statements)

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“…For our SSS‐positive sera, we found a sensitivity of 80% which is lower than the > 93% found in earlier studies . The three patients who tested negative clinically fitted the diagnosis of mechanobullous EBA.…”

Section: Discussioncontrasting

confidence: 79%

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Low sensitivity of type VII collagen enzyme-linked immunosorbent assay in epidermolysis bullosa acquisita: serration pattern analysis on skin biopsy is required for diagnosis

et al. 2013

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Section: Discussioncontrasting

confidence: 79%

“…A commercial ELISA is available that has the recombinant NC1 and NC2 coll VII domains coated to the plate, and high sensitivity (> 93%) and specificity (> 96%) have been reported . A correlation between the coll VII ELISA index and disease severity was also demonstrated . Komorowski et al .…”

mentioning

confidence: 99%

Low sensitivity of type VII collagen enzyme-linked immunosorbent assay in epidermolysis bullosa acquisita: serration pattern analysis on skin biopsy is required for diagnosis

et al. 2013

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“…Based on this insight, the pathogenic relevance of COL7 autoantibodies has been demonstrated (i) in vitro by demonstrating digestion of the dermal–epidermal junction in skin specimen incubated with anti-COL7 IgG or IgA and neutrophils (378, 379), (ii) in vivo by induction of inflammation and blistering in mice by transfer of anti-COL7 IgG (380, 381) or by immunization (382, 383), and (iii) clinically by the observation of a correlation of circulating autoantibody titers with clinical disease severity (384, 385). Subsequently, use of these animal models has greatly contributed to our current understanding of EBA pathogenesis.…”

Section: Autoantibody-induced Inflammationmentioning

confidence: 99%

Mechanisms of Autoantibody-Induced Pathology

et al. 2017

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Autoantibodies are frequently observed in healthy individuals. In a minority of these individuals, they lead to manifestation of autoimmune diseases, such as rheumatoid arthritis or Graves' disease. Overall, more than 2.5% of the population is affected by autoantibody-driven autoimmune disease. Pathways leading to autoantibody-induced pathology greatly differ among different diseases, and autoantibodies directed against the same antigen, depending on the targeted epitope, can have diverse effects. To foster knowledge in autoantibody-induced pathology and to encourage development of urgently needed novel therapeutic strategies, we here categorized autoantibodies according to their effects. According to our algorithm, autoantibodies can be classified into the following categories: (1) mimic receptor stimulation, (2) blocking of neural transmission, (3) induction of altered signaling, triggering uncontrolled (4) microthrombosis, (5) cell lysis, (6) neutrophil activation, and (7) induction of inflammation. These mechanisms in relation to disease, as well as principles of autoantibody generation and detection, are reviewed herein.

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“…EBA is characterized by autoantibodies of the IgGisotype specific to type VII collagen, the main constituent of anchoring fibrils at the dermal-epidermal junction. 154 Binding of autoantibodies to type VII collagen triggers an inflammatory reaction, including fixation of complement and Fc-dependent activation of leucocytes. 155 Activated granulocytes release reactive oxygen intermediates and proteases leading to epithelial injury and blister formation.…”

Section: Epidermolysis Bullosa Acquisitamentioning

confidence: 99%

“…Recently, an immunoenzymatic method has been set up to detect circulating autoantibodies against type VII collagen. 154 Systemic diseases are often associated with EBA, including CD and, less frequently, UC. 158 A study found that the prevalence of IBD in patients with EBA was about 25%.…”

Section: Epidermolysis Bullosa Acquisitamentioning

confidence: 99%

Cutaneous Manifestations in Patients With Inflammatory Bowel Diseases

Marzano

Borghi

Stadnicki

et al. 2014

Inflammatory Bowel Diseases

114

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The skin is one of the most common extraintestinal organ system affected in patients with inflammatory bowel disease (IBD), including both Crohn's disease and ulcerative colitis. The skin manifestations associated with IBD are polymorphic and can be classified into 4 categories according to their pathophysiology: (1) specific, (2) reactive, (3) associated, and (4) induced by IBD treatment. Cutaneous manifestations are regarded as specific if they share with IBD the same granulomatous histopathological pattern: perianal or metastatic Crohn's disease (commonly presenting with abscesses, fistulas or hidradenitis suppurativa-like features) is the prototype of this setting. Reactive cutaneous manifestations are different from IBD in the histopathology but have close physiopathological links: pyoderma gangrenosum, a neutrophil-mediated autoinflammatory skin disease typically manifesting as painful ulcers, is the paradigm of this group. Among the cutaneous diseases associated with IBD, the most commonly seen are erythema nodosum, a form of panniculitis most commonly involving bilateral pretibial areas, and psoriasis, a T helper 1/T helper 17-mediated erythematous squamous inflammatory disease. Finally, the number of cutaneous adverse reactions because of IBD therapies is progressively increasing. The most frequent drug-induced cutaneous manifestations are psoriasis-like, eczema-like, and lichenoid eruptions, as well as cutaneous lupus erythematosus for biologics, and nonmelanoma skin cancer, mainly basal cell and squamous cell carcinomas for thiopurines.

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Diagnosis and disease severity assessment of epidermolysis bullosa acquisita by ELISA for anti-type VII collagen autoantibodies: an Italian multicentre study

Abstract: This NC1+NC2 ELISA can be a practical assay for the diagnosis of EBA. The correlation between autoantibody titres and disease severity suggests its usefulness as a marker of disease activity in EBA However, this should be confirmed by studies on larger series of patients.

Cited by 32 publications

References 17 publications

Low sensitivity of type VII collagen enzyme-linked immunosorbent assay in epidermolysis bullosa acquisita: serration pattern analysis on skin biopsy is required for diagnosis

Low sensitivity of type VII collagen enzyme-linked immunosorbent assay in epidermolysis bullosa acquisita: serration pattern analysis on skin biopsy is required for diagnosis

Mechanisms of Autoantibody-Induced Pathology

Cutaneous Manifestations in Patients With Inflammatory Bowel Diseases

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