Bullous pemphigoid and epidermolysis bullosa acquisita: presentation, prognosis, and immunopathology in 11 children.

Edwards, Scott A.; Wakelin, S. H.; Wojnarowska, Fenella; Marsden, R.A.; Kirtschig, Gudula; Bhogal, B.; Black, M.M.

doi:10.1046/j.1525-1470.1998.1998015184.x

Cited by 66 publications

(41 citation statements)

References 33 publications

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“…Indirect immunofluorescence (IIF) was positive in 10 out of 14 patient sera, and the IgG constituted a predominant serum antibody. Although mucosal involvement is frequent and severe in childhood EBA, the overall prognosis and treatment is more favorable than in adult EBA [15, 39]. …”

Section: Clinical Manifestationsmentioning

confidence: 99%

Epidermolysis bullosa acquisita

Gupta

Woodley

Chen

2012

Clinics in Dermatology

147

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: Clinical Manifestationsmentioning

confidence: 99%

Epidermolysis bullosa acquisita

Gupta

Woodley

Chen

2012

Clinics in Dermatology

147

112

View full text Add to dashboard Cite

show abstract

“…It is unknown whether EBA typically occurs in adulthood. Childhood cases are not so rare, as suggested by recent reports [13, 14]. …”

Section: Introductionmentioning

confidence: 84%

“…Four responded well to this treatment [32]. In children, a therapeutic approach employing dapsone and corticosteroids often resolves the active disease [14]. …”

Section: Discussionmentioning

confidence: 99%

Epidermolysis bullosa acquisita with Combined Features of Bullous Pemphigoid and Cicatricial Pemphigoid

Wieme¹,

Lambert²,

Moerman

et al. 1999

Dermatology

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Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal blistering disease associated with autoantibodies against type VII collagen. The classical or mechanobullous form of EBA is characterized by skin fragility, trauma-induced blisters and erosions with mild mucous membrane involvement and healing with scars. Furthermore, bullous-pemphigoid-like and cicatricial pemphigoid-like features have been described. We report a patient who developed a bullous skin disease with upper airway obstruction requiring tracheotomy. The diagnosis of EBA was established by immunoblot, showing a band at 290 kD (collagen VII), and NaCl-split skin immunofluorescence (IgG deposition at the floor of the split). This case presented with clinical features of both bullous pemphigoid and cicatricial pemphigoid which to our knowledge is the first report of such a combination in EBA. The patient also presented tracheal involvement that has never been described either.

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Section: Clinical Manifestationsmentioning

confidence: 99%

Epidermolysis bullosa acquisita: Autoimmunity to anchoring fibril collagen

et al. 2011

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Epidermolysis bullosa acquisita (EBA) is a rare and acquired autoimmune subepidermal bullous disease of the skin and mucosa. EBA includes various distinct clinical manifestations resembling Bullous Pemphigus, Brunsting-Perry pemphigoid, or cicatricial pemphigoid. These patients have autoantibodies against type VII collagen, an integral component of anchoring fibrils, which are responsible for attaching the dermis to the epidermis. Destruction or perturbation of the normally functioning anchoring fibrils clinically results in 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 a mouse, the mouse develops an EBA-like blistering disease. Currently treatment is often unsatisfactory, however some success has been achieved with colchichine, dapsone, photopheresis, plasmaphresis, infliximab, rituximab and IVIG.

show abstract

Bullous pemphigoid and epidermolysis bullosa acquisita: presentation, prognosis, and immunopathology in 11 children.

Cited by 66 publications

References 33 publications

Epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita with Combined Features of Bullous Pemphigoid and Cicatricial Pemphigoid

Epidermolysis bullosa acquisita: Autoimmunity to anchoring fibril collagen

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