Intractable Epidermolysis bullosa acquisita: Efficacy of Ciclosporin A

Merle, Corinne; Blanc, Dominique; Zultak, M; Landuyt, H. Van; Drobacheff, C.; Laurent, R

doi:10.1159/000247859

Cited by 34 publications

(10 citation statements)

References 19 publications

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“…Although cyclosporin has been used in EBA with some improvement of the disease, this treatment was not successful in our patient in spite of a high dosage. [25][26][27] Azathioprine also did not seem to have been effective. Because of the difficulties in the treatment of EBA, many drugs and therapies have been used, such as colchicine, sulphapyridine, cyclophosphamide, methotrexate and plasmapheresis.…”

Section: Discussionmentioning

confidence: 90%

IgA‐epidermolysis bullosa acquisita in a child resulting in blindness

Caux

Kirtschig

Lemarchand-Vénencie

et al. 1997

British Journal of Dermatology

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Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disease characterized by IgG autoantibodies directed against type VII collagen, the major component of anchoring fibrils. The classical phenotype of EBA is a non-inflammatory, mechanobullous disease resembling the dystrophic forms of inherited epidermolysis bullosa. Mucous membrane involvement is frequent but usually mild. We report a 1-year-old girl suffering from IgA-EBA, who presented with an initial eruption of disseminated urticarial lesions and tense blisters of the skin but subsequently developed severe oral and ocular lesions reminiscent of cicatricial pemphigoid. Direct immunofluorescence of the skin and buccal mucosa revealed linear IgA and C3 at the basement membrane zone (BMZ). IgA anti-BMZ autoantibodies stained the dermal side of salt-split skin by indirect immunofluorescence and recognized a dermal protein of 290 kDa co-migrating with type VII collagen by immunoblotting. Direct and indirect immunoelectron microscopy revealed IgA deposits overlying the anchoring fibrils. The ocular involvement led to total blindness in spite of intense treatment. This case of childhood IgA-EBA is particularly striking because of the cicatricial pemphigoid phenotype with severe ocular involvement which resulted in blindness. It reinforces the necessity to use modern immunological methods to classify autoimmune bullous diseases in order to allow early and appropriate treatment.

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Section: Discussionmentioning

confidence: 90%

IgA‐epidermolysis bullosa acquisita in a child resulting in blindness

Caux

Kirtschig

Lemarchand-Vénencie

et al. 1997

British Journal of Dermatology

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“…Total or partial remissions were maintained, even after drug cessation. [138][139][140][141][142][143][144][145][146] The same agents that are used for the inflammatory form may be used for the classical form. The latter is generally more resistant to treatment.…”

Section: Management Of Epidermolysis Bullosa Acquisitamentioning

confidence: 99%

Management of autoimmune bullous diseases: Pharmacology and therapeutics

Mutasim

2004

Journal of the American Academy of Dermatology

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“…16,26,27,90 In patients with recalcitrant disease or those who have developed side effects from glucocorticoids, cyclosporine may be administered. 90,93,94 A review of the literature identified 11 case reports examining the use of IVIG in the treatment of recalcitrant EBA; efficacy was observed in all but one case. [95][96][97][98][99][100][101][102][103][104] Rapidly progressive, treatment-resistant EBA was therefore considered an indication for IVIG therapy in a recent consensus statement that was published on the use of IVIG in patients with AMBDs.…”

Section: Epidermolysis Bullosa Acquisitamentioning

confidence: 99%