Epidermolysis Bullosa Acquisita-a Sign of Cicatricial Pemphigoid?

Dahl, M.G.C.

doi:10.1111/j.1365-2133.1979.tb00030.x

Cited by 83 publications

(38 citation statements)

References 52 publications

(46 reference statements)

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“…In addition to the mechanobullous classic variant, several inflammatory subtypes of EBA were described, clinically mimicking bullous pemphigoid, linear IgA disease, mucous membrane pemphigoid or Brunsting-Perry pemphigoid. [40][41][42][43][44][45][46][47][48] Although the original diagnostic criteria for EBA stated that disease onset should be in adulthood, several childhood cases have been documented. Interestingly, several previous reports described that some childhood EBA patients with reactivity to the triple-helical collagenous domain, as well as the NC1 and NC2 domains, were of the inflammatory type.…”

Section: Clinical Presentationmentioning

confidence: 99%

Epidermolysis bullosa acquisita: What’s new?

Ishii

Hamada

Dainichi

et al. 2010

The Journal of Dermatology

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Type VII collagen is an adhesion molecule of the extracellular matrix in epithelial basement membranes, and the main constituent of anchoring fibrils at the dermal-epidermal junction (DEJ). Autoimmunity against this protein is causing the rare organ-specific epidermolysis bullosa acquisita (EBA). EBA is a rare acquired, heterogeneous, chronic blistering disease of skin disease of skin and mucous membranes characterized by subepidermal blisters and tissue-bound as well as circulating autoantibodies to the DEJ. EBA has several distinct clinical presentations with other subepidermal bullous diseases, such as mainly dystrophic epidermolysis bullosa or bullous pemphigoid. The circulating immunoglobulin G autoantibodies for EBA react with a 290-kDa dermal protein, type VII collagen, as detected by immunoblot analysis using dermal extracts. The pathogenicity of these autoantibodies has been demonstrated by experimental animal models, in which anti-type VII collagen antibodies injected into a mouse produced an EBA-like blistering disease in the animal. EBA cases often require high doses of systemic corticosteroids and a variety of immunosuppressants. Although treatment for EBA is frequently difficult and unsatisfactory, some therapeutic success has been reported with colchicine, dapsone, infliximab and i.v. immunoglobulin. In this review, we will focus on recent progress in our understanding of the clinical manifestations, the etiopathogenesis as well as the management of EBA.

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Section: Clinical Presentationmentioning

confidence: 99%

Epidermolysis bullosa acquisita: What’s new?

Ishii

Hamada

Dainichi

et al. 2010

The Journal of Dermatology

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“…Five different phenotypes of EBA have been reported so far, including the classical mechanobullous variant described by Roenigk [10] and the four inflammatory forms resembling bullous pemphigoid, mucous membrane pemphigoid, linear IgA-disease, and Brunsting-Perry pemphigoid, respectively [11][12][13][14]. About one-third of EBA patients present with the classical phenotype and about two-thirds with one of the inflammatory variants [26,27].…”

Section: Clinical Presentationmentioning

confidence: 99%

“…All squamous cell epithelia can be affected, including oral and nasal mucosa, larynx, esophagus, anal and genital mucosa as well as conjunctiva [14,[93][94][95]. While 50-65% of EBA patients have mucosal lesions [26,96,97] (Figure 5) in about 5-10% of patients, mucosal involvement predominates and patients may be classified as mucous membrane pemphigoid-variant of EBA [27,85].…”

Section: Mucous Membrane Pemphigoid-like Variantmentioning

confidence: 99%

“…Two major clinical variants have been described, the classical mechanobullous form [10] and the inflammatory variant [11]. Within latter form, subtypes resembling bullous pemphigoid, mucous membrane pemphigoid, linear immunoglobulin A (IgA) disease, and Brunsting-Perry pemphigoid can be differentiated [11][12][13][14]. Diagnosis of EBA can, thus, not be based on the clinical picture alone but requires a combination of laboratory investigations including direct immunofluorescence (IF) microscopy and serological assays.…”

Section: Introductionmentioning

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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“…The rela tions between these two clinical entities however remain controversial and need fur ther investigation [5,12], As far as seen from the relatively few cases, the following criteria to differentiate can be established (table II).…”

Section: [2]mentioning

confidence: 99%