Cyclosporine controls epidermolysis bullosa acquisita co-occuring with acquired factor VIII deficiency

John C. Maize, Jack B. Cohen

Research output: Contribution to journalArticle

20 Citations (Scopus)

Abstract

Background: Epidermolysis bullosa acquisita (EBA) is a rare antibody-mediated autoimmune blistering disease of adults. Likewise, acquired factor VIII deficiency is a rare antibody-mediated disease of adulthood. Both diseases can be exceedingly difficult to treat and refractory to immunomodulatory therapies. Methods: Herein, we report a challenging case of EBA and acquired factor VIII deficiency in the same patient. Results: Cyclosporine 4 mg/kg/day rapidly controlled both disease processes after the patient failed to respond to prednisone, colchicine, and pulse cyclophosphamide. The EBA relapsed when the cyclosporine was decreased to 2 mg/kg/day and the patient wore snuggly fitting new shoes, but it cleared quickly when the dose was increased. Cyclosporine has since been decreased gradually to 1.5 mg/kg/day without relapse of either condition or detectable side-effects. Conclusions: Cyclosporine should be considered when EBA and/or acquired factor VIII deficiency fail to respond to conservative therapy. Both conditions usually respond rapidly to cyclosporine 4-6 mg/kg/day.

Original languageEnglish (US)
Pages (from-to)692-694
Number of pages3
JournalInternational Journal of Dermatology
Volume44
Issue number8
DOIs
StatePublished - Aug 2005

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Epidermolysis Bullosa Acquisita
Cyclosporine
Shoes
Immunomodulation
Antibodies
Colchicine
Prednisone
Cyclophosphamide
Autoimmune Diseases
Acquired Factor 8 deficiency
Recurrence

ASJC Scopus subject areas

  • Dermatology

Cite this

Cyclosporine controls epidermolysis bullosa acquisita co-occuring with acquired factor VIII deficiency. / Maize, John C.; Cohen, Jack B.

In: International Journal of Dermatology, Vol. 44, No. 8, 08.2005, p. 692-694.

Research output: Contribution to journalArticle

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