Epidermolysis Bullosa Acquisita

Roenigk, Henry H.; Ryan, James; Bergfeld, Wilma F.

doi:10.1001/archderm.1971.04000130003001

Cited by 216 publications

(24 citation statements)

References 17 publications

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“…In 1971, Roenigk et al established the first diagnostic criteria for EBA. An EBA diagnosis depends on the following criteria: (i) clinical lesions resembling epidermolysis bullosa dystrophica; (ii) adult onset of disease; (iii) a negative family history of epidermolysis bullosa dystrophica; and (iv) exclusion of other bullous diseases [2]. In 1973, Kushniruk first noted the deposition of IgG and C3 along the dermal-epidermal junction in EBA patients [3].…”

Section: Introductionmentioning

confidence: 99%

Meta-analysis of the clinical and immunopathological characteristics and treatment outcomes in epidermolysis bullosa acquisita patients

Iwata

Vorobyev

Koga

et al. 2018

Orphanet J Rare Dis

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BackgroundEpidermolysis bullosa acquisita (EBA) is an orphan autoimmune disease. Several clinical phenotypes have been described, but subepidermal blistering is characteristic of all variants. Limited data on clinical and immunopathological characteristics and treatment outcomes in EBA are available. To fill this gap, we collected this information from EBA cases, meeting current diagnostic criteria, published between 1971 and 2016.ResultsWe identified 1159 EBA cases. This number must be, however, interpreted with caution, as it is not possible to check for multiple reporting. The analysis of all cases indicated that EBA affects all age groups (median: 50 years, range: 1 to 94 years) at an equal gender distribution. Non-mechanobullous (non-MB) forms of EBA were observed in 55% of patients, whereas the mechanobullous variant (MB-EBA) or a combination of both variants was described in 38 or 7% of patients, respectively. Type VII collagen (COL7)-specific autoantibodies were primarily of the IgG isotype, but anti-COL7 IgA, IgM and IgE were also documented. Comparison of the 2 clinical EBA types showed a higher frequency of IgA deposits in non-MB EBA as opposed to MB EBA. Mucous membrane involvement was observed in 23% of patients, and 4.4% of cases were associated with other chronic inflammatory diseases. Of note, IgA deposits were more frequently observed in cases with mucous membrane involvement. Our analysis indicated that EBA is difficult to treat and that the choice of treatment varies widely. Chi square was applied to identify medications associated with complete remission (CR). Considering all EBA cases, intravenous immunoglobulin (IVIG, p = 0.0047) and rituximab (p = 0.0114) were associated with CR. Subgroup analysis demonstrated that no treatment was associated with CR for non-MB EBA, while IVIG (p = 0.003) was associated with CR in MB EBA.ConclusionsWithin the limitations of the study, we here document the clinical and immunopathological characteristics and treatment outcomes in a large cohort of EBA patients. The observed associations of single drugs with treatment outcome may serve as a guide to develop clinical trials.Electronic supplementary materialThe online version of this article (10.1186/s13023-018-0896-1) contains supplementary material, which is available to authorized users.

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Section: Introductionmentioning

confidence: 99%

Meta-analysis of the clinical and immunopathological characteristics and treatment outcomes in epidermolysis bullosa acquisita patients

Iwata

Vorobyev

Koga

et al. 2018

Orphanet J Rare Dis

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“…Likewise, blisters, erosions, ulcerations, and scarring have been previously described on the buccal mucosa, tongue, gingiva, palate, and the lips of patients with EBA. 2,5,[8][9][10] It is suggested that either perioral or intraoral blistering leads to microstomia, ankyloglossia, Hakki, Çelenligil-Nazliel, Karaduman, et al 555 Small and noninflammatory subepithelial clefts are seen. Stroma is mildly fibrotic (H&E; original magnification ×100).…”

Section: Discussionmentioning

confidence: 99%

Epidermolysis Bullosa Acquisita: Clinical Manifestations, Microscopic Findings, and Surgical Periodontal Therapy. A Case Report

Hakkı

Çelenligil-Nazliel

Karaduman

et al. 2001

Journal of Periodontology

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These results indicate that gingival tissues may also be involved in EBA. Uneventful wound healing after periodontal surgery in this case suggests that periodontal surgery can be performed in patients with EBA. Moreover, both our clinical and histopathologic findings imply that gingivectomy proves useful in maintaining gingival integrity in these patients. Our data may also suggest that the patients with EBA are highly likely to develop dental caries.

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“…Localized chronic head, neck, + ± pemphigoid [3] mucosa of mouth Disseminated generalized + ± cicatricial pemphigoid [14] show similar clinical and electron micros copical findings [7,11,12,15,16]. 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%