Epidermolysis bullosa acquisita with electron microscopical studies

Gibbs, R. B.

doi:10.1001/archderm.111.2.215

Cited by 24 publications

(9 citation statements)

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“…This finding is similar to that of epidermolysis bullosa hereditaria and could explain skin fragility [111,112]. When performed from lesional skin, the cleavage plane can be demonstrated.…”

Section: Immunoelectron Microscopysupporting

confidence: 80%

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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“…This finding is similar to that of epidermolysis bullosa hereditaria and could explain skin fragility [111,112]. When performed from lesional skin, the cleavage plane can be demonstrated.…”

Section: Immunoelectron Microscopysupporting

confidence: 80%

Clinical features and diagnosis of epidermolysis bullosa acquisita

Vorobyev

Ludwig

Schmidt

2016

Expert Review of Clinical Immunology

100

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“…Direct immunofluorescence of perilesional skin biopsies from EBA patients reveals IgG deposits at the dermal-epidermal junction [7][8][9]. EBA antibodies bind to type VII collagen within anchoring fibrils [2].…”

mentioning

confidence: 99%

Autoimmunity to Type VII Collagen: Epidermolysis Bullosa Acquisita

Woodley

Remington

Chen

2007

Clinic Rev Allerg Immunol

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Epidermolysis bullosa acquisita (EBA) is an acquired, autoimmune, mechanobullous disease with clinical features reminiscent of genetic dystrophic epidermolysis bullosa (DEB). EBA patients have skin fragility, blisters, scars, and milia formation. DEB is due to a genetic defect in the gene-encoding type VII collagen, which makes anchoring fibrils, structures that attach the epidermis and its underlying basement membrane zone onto the papillary dermis. DEB patients have a decrease in normally functioning anchoring fibrils. EBA patients have the same problem, but their decrease in normally functioning anchoring fibrils is because of an abnormality in their immune system in which they produce anti-type VII collagen antibodies that attack their anchoring fibrils. These IgG anti-type VII collagen antibodies are "pathogenic" because when injected into a mouse, the mouse develops an EBA-like blistering disease. EBA has several distinct clinical presentations. It can present with features similar to DEB. It can also present with features reminiscent of bullous pemphigoid, cicatricial pemphigoid, Brunsting-Perry pemphigoid, or IgA bullous dermatosis. Treatment for EBA is unsatisfactory. Some therapeutic success has been reported with colchichine, dapsone, photopheresis, infliximab, and IVIG.

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“…In 1971, Roenigk, Ryan & Berfeld (1971) suggested four criteria for the diagnosis of EBA: (i) clinical lesions of trauma-induced bulla occurring over the joints of the hands, feet, elbows and knees, atrophic scars, milia and nail dystrophy; (2) post infancy onset of the disease; (3) no family history of EBA; and (4) exclusion of other bullous diseases. Based on the subsequent immunopathological studies of Kushniruk (1973) and Gibbs & Minus (1975) and more recently, immunoelectronmicroscopic studies of Nieboer et al (1980), Roenigk & Pearson (1981) have added three diagnostic criteria to the above: (i) the finding of IgG at the basement membrane zone (BMZ) by direct immunofluorescence microscopy (IF); (2) demonstration of blister formation beneath the basal lamina with a zone of amorphous material beneath remaining on the epidermal side of the blister by electronmicroscopy; and (3) deposition of IgG in the amorphous zone beneath the basal lamina, demonstrable by immunoelectronmicroscopy.…”

Section: Discussionmentioning

confidence: 99%

Epidermolysis bullosa acquisita with pulmonary tuberculosis: a case report

et al. 1983

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Summary Our case is that of a 67‐year‐old man who presented with a blistering, scarring eruption at sites of trauma. Histologically the blistering was subepidermal. IgG and C3 were present at the basement membrane zone. Electronmicroscopy demonstrated bulla formation to be beneath the basal lamina and that it contained amorphous electron‐dense material. These clinical and investigational findings are consistent with a diagnosis of epidermolysis bullosa acquisita (EBA). The patient also had pulmonary tuberculosis which again highlights the association between EBA and diseases in which the immune system is compromised.

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Epidermolysis bullosa acquisita with electron microscopical studies

Cited by 24 publications

References 0 publications

Clinical features and diagnosis of epidermolysis bullosa acquisita

Clinical features and diagnosis of epidermolysis bullosa acquisita

Autoimmunity to Type VII Collagen: Epidermolysis Bullosa Acquisita

Epidermolysis bullosa acquisita with pulmonary tuberculosis: a case report

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