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

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.

• Authors: G Hirsch, S Ingen-Housz-Oro, C Fite, L Valeyrie-Allanore, N Ortonne, V Buffard, M Verlinde-Carvalho, E Marinho, J Martinet, S Grootenboer-Mignot, V Descamps, P Wolkenstein, P Joly, O Chosidow
• Journal: Journal of the European Academy of Dermatology and Venereology : JEADV (J Eur Acad Dermatol Venereol) Vol. 30 Issue 7 Pg. 1140-3 (Jul 2016) ISSN: 1468-3083 [Electronic] England
• PMID: 26369288 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2015 European Academy of Dermatology and Venereology.

Chemical References

• Rituximab

Topics

• Adult
• Erythema Multiforme (drug therapy)
• Female
• Humans
• Male
• Middle Aged
• Rituximab (therapeutic use)
• Young Adult