Epidermolysis bullosa acquisita in childhood - a case mimicking chronic bullous dermatosis of childhood

PARK, S.B.; CHO, K.H.; Youn, Jai-Il; Hwang, Dong‐Choon; Kim, S. C.; CHUNG, J.H.

doi:10.1046/j.1365-2230.1997.2560679.x

Cited by 31 publications

(23 citation statements)

References 11 publications

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“…A novel EBA subgroup with a milder clinical presentation was defined in children with tissue-bound and circulating autoantibodies targeting only the triple-helical central domain of C7 [14, 15]. In addition, recent reports showed that the NC2 domain, which may be pertinent in the formation of antiparallel dimers and AF assembly, also contains minor antigenic epitopes.…”

Section: Etiology and Pathogenesismentioning

confidence: 99%

Epidermolysis bullosa acquisita

Gupta

Woodley

Chen

2012

Clinics in Dermatology

146

112

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EBA is a rare, acquired, chronic subepidermal bullous disease of the skin and mucosa characterized by autoantibodies to type VII collagen structures, a major component of anchoring fibrils, that attach the epidermis onto the dermis. EBA patients have tissue-bound as well as circulating anti-type VII collagen autoantibodies that attack type VII collagen and result in a reduction or perturbation of normally functioning anchoring fibrils. Patients with EBA have skin fragility, blisters, erosions, scars, milia, and nail loss: all features reminiscent of genetic dystrophic epidermolysis bullosa. These anti-type VII collagen antibodies are “pathogenic” because when injected into mice, the mice develop an EBA-like blistering disease. In addition to the classical mechanobullous presentation, EBA also has several other distinct clinical syndromes similar to bullous pemphigoid, Brunsting-Perry pemphigoid, or cicatricial pemphigoid. Although treatment for EBA is often unsatisfactory, some therapeutic success has been achieved with colchicine, dapsone, plasmaphoresis, photopheresis, infliximab, and intravenous immunoglobulin.

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Section: Etiology and Pathogenesismentioning

confidence: 99%

Epidermolysis bullosa acquisita

Gupta

Woodley

Chen

2012

Clinics in Dermatology

146

112

View full text Add to dashboard Cite

show abstract

“…Most patients have IgG autoantibodies targeting the non-collagenous domain of type VII collagen in the anchoring fibrils but a small group demonstrate antibodies to the collagenous domain or have IgA antibodies. Park et al (1997) reported an example in a 6 year old girl with genital involvement. In Marren's study of 140 patients with blistering disorders, one of the two adults and one of the three children with EBA had vulval lesions (Marren et al 1993).…”

Section: Aetiology and Classificationmentioning

confidence: 99%

Non‐Infective Cutaneous Conditions of the Vulva

Neill

Lewis

2009

Ridley's the Vulva

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“…However, a novel EBA subgroup with a milder clinical presentation was defined in children with tissue-bound and circulating autoantibodies targeting only the triple-helical central domain of type VII collagen [21,22]. Furthermore, another study using recombinant type VII collagen fragments as antigens have revealed yet additional patients who recognize epitopes in both the NC1 and the triple-helical domain [22].…”

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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Epidermolysis bullosa acquisita in childhood - a case mimicking chronic bullous dermatosis of childhood

Cited by 31 publications

References 11 publications

Epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita

Non‐Infective Cutaneous Conditions of the Vulva

Autoimmunity to Type VII Collagen: Epidermolysis Bullosa Acquisita

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