Acquired epidermolysis bullosa with the clinical feature of Brunsting-Perry cicatricial bullous pemphigoid

Kurzhals, G.

doi:10.1001/archderm.127.3.391

Cited by 24 publications

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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%

“…In most patients with Brunsting-Perry pemphigoid, antibodies against type VII collagen have been detected, thus classifying as Brunsting-Perry pemphigoid-like variant of EBA [12,26,27,[104][105][106].…”

Section: Brunsting-perry 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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“…A salt split skin test was not performed. Eventually, enzyme-linked immunosorbent assay (ELISA) was performed with native human laminin 332 (affinity-purified protein with ␣ 3 -, Kurzhals et al [9] described a case with clinical features of Brunsting-Perry pemphigoid with an immunofluorescence and immuno-electron microscopy pattern identical to EBA. This finding has also been reported by others [10,11] .…”

Section: Casementioning

confidence: 99%

Brunsting-Perry Pemphigoid of the Scalp with Antibodies against Laminin 332

et al. 2011

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Letter to Dermatology␤ 3 -and ␥ 2 -chains from the squamous carcinoma cell line SCC25), recombinant 230-kDa BP antigen (BP230) and 180-kDa BP antigen (BP180; baculoprotein expression system) as previously described [4] . By ELISA, there was strong IgG reactivity against laminin 332 and reactivity against a distinct BP180 epitope (midportion of the BP180 ectodomain, aa residues 809-1106). The lesion healed with topical corticosteroids. Three years later, the patient was in complete remission, and a control ELISA no longer showed IgG reactivity against laminin 332, BP180 or BP230 antigens. Case 2A 74-year-old man had a 2-year history of erosions and crusts of the scalp. At presentation, he had an erosive and crusty lesion 10 cm in diameter on the top of his head while other parts of the body or mucous membranes were not affected ( fig. 1 b). The biopsy showed actinic damage of the epidermis; the diagnosis of actinic keratosis was established, and the lesion was treated with liquid nitrogen with only temporary effect. A second biopsy of lesional skin showed subepidermal cleavage and an inflammatory infiltrate composed of lymphocytes and histiocytes in the dermis suggestive of CP ( fig. 1 c). DIF of lesional skin was negative, IIF on monkey oesophagus showed granular antinuclear auto-antibodies at a titre of 1: 80 and weak linear positivity with anti-IgG, -A and -M antibodies; salt split skin was negative. By ELISA, the patient's serum showed IgG reactivity against laminin 332. Topical treatment with corticosteroids led to a temporary healing of the lesion. Two years later, the patient still needs intermittent topical corticosteroid treatment. DiscussionBrunsting-Perry pemphigoid typically affects elderly men as multiple erosive and scarring lesions in the head and neck region, rarely as a solitary plaque [5] . In case of a solitary lesion the diagnosis may be extremely difficult. These cases can be misdiagnosed as actinic keratosis, pyoderma or dermatitis artefacta. The lesion is often eroded; thus, no subepidermal blister is detected, eosinophils are usually absent, immunofluorescence studies, if done, can be negative.A case of Brunsting-Perry pemphigoid similar to ours with a solitary lesion of the scalp was described by Poon and McGrath [6] , which was negative by DIF and IIF. Monihan et al. [7] described 3 cases of CP simulating basal cell carcinoma. Murata et al. [8] detected immunoglobulin deposits between basal keratinocytes and the lamina lucida of the dermal-epidermal junction by immuno-electron microscopy in a case of Brunsting-Perry pemphigoid supporting the concept that this disorder is closely related to CP. Key WordsAuto-immune bullous diseases ؒ Brunsting-Perry pemphigoid ؒ Cicatricial pemphigoid ؒ Laminin 332 A scarring disease localized to the head and neck without mucosal involvement, described by Brunsting and Perry in 1957 [1] , is considered as a variant of cicatricial pemphigoid (CP), bullous pemphigoid (BP) or epidermolysis bullosa acquisita (EBA), although the target antigen has not yet be...

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Acquired epidermolysis bullosa with the clinical feature of Brunsting-Perry cicatricial bullous pemphigoid

Cited by 24 publications

References 0 publications

Epidermolysis bullosa acquisita: What’s new?

Epidermolysis bullosa acquisita: What’s new?

Clinical features and diagnosis of epidermolysis bullosa acquisita

Brunsting-Perry Pemphigoid of the Scalp with Antibodies against Laminin 332

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