Rituximab, a new treatment for difficult-to-treat chronic erythema multiforme major? Five cases

J Eur Acad Dermatol Venereol. 2016 Jul;30(7):1140-3. doi: 10.1111/jdv.13313. Epub 2015 Sep 15.

Abstract

Background: Erythema multiforme major (EMM) is an inflammatory disease affecting skin and mucosae, often triggered by infection with Herpes simplex virus. Some patients have a chronic disease associated with antidesmoplakin autoantibodies, but the pathophysiology remains to be elucidated. First-line treatment is antiviral therapy. With treatment failure or in patients without herpes-triggered disease, thalidomide is effective but has neurological side-effects. Alternatives (dapsone, immunosuppressant agents) are not codified. For many patients, systemic steroids use is chronic. The immunosuppressant drug rituximab (RTX) may be effective.

Objectives: We report five cases of severe chronic EMM treated with rituximab (RTX).

Methods: Five patients with severe chronic EMM for 9-20 years received RTX after failure or side-effects of several treatments, especially antiviral therapy and thalidomide. All had chronic use of steroids. Four patients had antidesmoplakin autoantibodies.

Results: Four patients experienced complete or quasi-complete remission of EMM with withdrawal of steroids and one patient partial remission, for 3-11 months. Disease relapsed in all patients, and three received a second cycle of RTX with shorter duration of efficacy. Two patients received a third cycle, one without efficacy.

Conclusion: The use of RTX for many autoimmune diseases, especially pemphigus, is increasing. Chronic EMM, especially EMM associated to antidesmoplakin autoantibodies, is an inflammatory disease in which the role of B cells is not well understood. However, we report a favourable benefit of RTX treatment for months in five patients with severe disease. RTX could be a therapeutic option in severe, difficult-to-treat EMM.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Erythema Multiforme / drug therapy*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Rituximab / therapeutic use*
  • Young Adult

Substances

  • Rituximab