Successful use of combined corticosteroids and rituximab in the treatment of recalcitrant epidermolysis bullosa acquisita

Annales de dermatologie et de vénéréologie
A CavailhesF Skowron

Abstract

Epidermolysis bullosa acquisita (EBA) is a rare autoimmune subepidermal blistering disease; it is potentially serious and is often refractory to conventional treatments, including corticosteroids. We report a new case of successful treatment of EBA using rituximab (anti-CD20 antibody) without relapse after 1 year of follow-up. A 76-year-old man was seen for blisters of the skin and mucosa, atrophic scars and milia on areas of friction. The diagnosis of EBA was made on the basis of histological and immunohistochemical criteria. The patient was unsuccessfully treated with topical steroids, dapsone, topical tacrolimus, systemic steroids, mycophenolate mofetil, doxycycline and methotrexate. Four weekly infusions of rituximab of 375 mg/m(2) body area were performed, combined with systemic steroids: they proved beneficial within 3 weeks, with a noticeable improvement and no further blisters at 7 months. After 1 year of follow-up, the skin disease is still stable with 5 mg/day of prednisone alone being given. This is the eighth reported case of treatment of EBA with rituximab and the sixth successful therapeutic outcome, with good steroid sparing effect and undeniable improvement in quality of life within several months and good toler...Continue Reading

References

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Citations

Aug 3, 2010·La Presse médicale·Frédéric Caux
Jan 29, 2019·Frontiers in Medicine·Hiroshi KogaKatja Bieber
Oct 28, 2019·The Journal of Dermatology·Hideyuki UjiieUNKNOWN Committee for Guidelines for the Management of Pemphigoid Diseases (Including Epidermolysis Bullosa Acquisita)
Apr 5, 2012·Oral Diseases·I D O'Neill, C Scully
Mar 23, 2011·Archives of Dermatology·Christelle Le Roux-VilletHervé Bachelez

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