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

Wieme, N; Lambert, Jo; Moerman, Mieke; Geerts, Maria; Temmerman, Liesbeth; Naeyaert, Jean

doi:10.1159/000018139

Cited by 31 publications

(17 citation statements)

References 21 publications

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“…Similar to CP, the lesions and scars usually involve the mouth, upper esophagus, conjunctivae, anus, and vagina [28, 30] with or without similar lesions on the glabrous skin. There has also been evidence of involvement of the trachea in these patients [31] and of mucosal involvement without scarring [32]. Unlike classical EBA, patients with the CP-like presentation often do not show significant skin fragility, evidence of trauma-induced lesions, or a predilection for blistering on extensor skin surfaces.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Epidermolysis bullosa acquisita

Gupta

Woodley

Chen

2012

Clinics in Dermatology

148

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

148

112

View full text Add to dashboard Cite

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“…Demgegenüber wird als inflammatorische Form der BP-artige Typ mit prallen, von erythematösen Plaques umgebenen Blasen abgegrenzt, der auch makroskopisch an ein solches erinnert, aber ohne Narben und Milien abheilt [9]. Klinisch bestehen auch Ähnlichkei-ten zum bullösen systemischen Lupus erythematodes (SLE), zur Dermatitis herpetiformis Duhring, zum bullösen Arzneimittelexanthem und bullösen Erythema exsudativum multiforme [10].…”

Section: Augenärztliches Konsilunclassified

“…Differenzialdiagnostisch lassen die klinischen Verände-rungen gelegentlich auch an eine IgA-lineare Dermatose denken. Über Schleimhautbefall des Larynx, Oropharynx, Öso-phagus, Anus und der weiblichen Genitalien wird berichtet [3,9]. Die narbigen Strikturen können zu lebensbedrohlichen Komplikationen führen und Bougierungen bzw.…”

Section: Histologieunclassified

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Epidermolysis bullosa acquisita mit facettenreichem klinischen Bild und erfolgreicher Mycophenolatmofetiltherapie

Trebing

Ziemer

2001

Der Hautarzt

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A 60-year-old woman with epidermolysis bullosa acquisita (EBA) presented with a highly variable clinical picture. First she developed oral erosions and ulcers covered with shaggy epithelial rests. Later she developed pustular and target lesions as well as tense cutaneous blisters. Clinically the alterations were strongly suggestive of aphthae or a pustular dermatosis. Histological and immunological examinations revealed subepidermal blister formation, neutrophilic infiltration, linear deposition of IgG and C3 at the basement membrane in the direct immunofluorescence and the evidence of circulating IgG-antibodies against basement membrane (monkey esophagus) in the indirect immunofluorescence. Thus our working diagnosis was a pustular or nodular form of bullous pemphigoid, as a linear IgA dermatosis was excluded. Indirect immunofluorescence using NaCl-separated primate skin localized the basement membrane antibodies in the base of the artificial blister, while immunoblotting of dermal extracts disclosed binding of serum antibodies to a 290-kDa protein. Bullous systemic lupus erythematosus was excluded, leading to the definitive diagnosis of EBA. The clinical variability of this disease is well known; the polymorphic picture in our patient with erosions, pustules, target lesions and blisters is unique in the literature. The similarity in histology and immunofluorescence of the clinically quite different lesions is another unexpected event. Also the speedy response to therapy with the immunosuppressive agent mycophenolate mofetil has only been described once before. The patient remains in remission after 6 months. Because of the poor response to therapy in most cases, the significant skin lesions are a source of physical as well as emotional distress. Additional complications include stenosis of trachea or bronchi injure.

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“…Posteriormente se describió una variedad inflamatoria con características clínicas similares al penfigoide ampolloso y otros subtipos que remedaban el penfigoide de mucosas o el penfigoide cicatricial de Brunsting-Perry 8--- 10 . Durante el curso de la enfermedad puede haber una transformación de una presentación clínica a otra o coexistir rasgos de ambas 11 .…”

Section: Introductionunclassified