title: 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.
  
  
  OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.affiliation: Department of Dermatology, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France.
authors.forename: G
authors.initials: G
authors.lastname: Hirsch
authors.affiliation: Department of Dermatology, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France.|EA EpiDermE (Epidémiologie en Dermatologie et Evaluation des Thérapeutiques), UPEC, Créteil, France.|Referral center for auto-immune and toxic blistering diseases, Paris, Créteil, France.
authors.forename: S
authors.initials: S
authors.lastname: Ingen-Housz-Oro
authors.affiliation: Referral center for auto-immune and toxic blistering diseases, Paris, Créteil, France.|Department of Dermatology, Assistance Publique-Hôpitaux de Paris, Bichat Hospital, Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: Fite
authors.affiliation: Department of Dermatology, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France.|EA EpiDermE (Epidémiologie en Dermatologie et Evaluation des Thérapeutiques), UPEC, Créteil, France.|Referral center for auto-immune and toxic blistering diseases, Paris, Créteil, France.
authors.forename: L
authors.initials: L
authors.lastname: Valeyrie-Allanore
authors.affiliation: Department of Pathology, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France.|UPEC Université Paris-Est Créteil, Créteil, France.
authors.forename: N
authors.initials: N
authors.lastname: Ortonne
authors.affiliation: Department of Dermatology, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France.
authors.forename: V
authors.initials: V
authors.lastname: Buffard
authors.affiliation: Pharmacy, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France.
authors.forename: M
authors.initials: M
authors.lastname: Verlinde-Carvalho
authors.affiliation: Department of Pathology, Assistance Publique-Hôpitaux de Paris, Bichat Hospital, Paris, France.
authors.forename: E
authors.initials: E
authors.lastname: Marinho
authors.affiliation: Department of Immunology, Rouen University Hospital, Rouen, France.|Normandie University, IRIB, Rouen, France.|INSERM, U905, Rouen, France.
authors.forename: J
authors.initials: J
authors.lastname: Martinet
authors.affiliation: Referral center for auto-immune and toxic blistering diseases, Paris, Créteil, France.|Department of Immunology, Assistance Publique-Hôpitaux de Paris, Bichat Hospital, Paris, France.
authors.forename: S
authors.initials: S
authors.lastname: Grootenboer-Mignot
authors.affiliation: Referral center for auto-immune and toxic blistering diseases, Paris, Créteil, France.|Department of Dermatology, Assistance Publique-Hôpitaux de Paris, Bichat Hospital, Paris, France.
authors.forename: V
authors.initials: V
authors.lastname: Descamps
authors.affiliation: Department of Dermatology, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France.|EA EpiDermE (Epidémiologie en Dermatologie et Evaluation des Thérapeutiques), UPEC, Créteil, France.|Referral center for auto-immune and toxic blistering diseases, Paris, Créteil, France.|UPEC Université Paris-Est Créteil, Créteil, France.
authors.forename: P
authors.initials: P
authors.lastname: Wolkenstein
authors.affiliation: Department of Dermatology, Charles Nicolle Hospital, Rouen, France.
authors.forename: P
authors.initials: P
authors.lastname: Joly
authors.affiliation: Department of Dermatology, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France.|EA EpiDermE (Epidémiologie en Dermatologie et Evaluation des Thérapeutiques), UPEC, Créteil, France.|Referral center for auto-immune and toxic blistering diseases, Paris, Créteil, France.|UPEC Université Paris-Est Créteil, Créteil, France.|INSERM CIC 1430, Créteil, France.
authors.forename: O
authors.initials: O
authors.lastname: Chosidow
mesh_terms: D000328:Adult; D004892:Erythema Multiforme / Q000188:drug therapy*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D000069283:Rituximab / Q000627:therapeutic use*; D055815:Young Adult
chemical_list: D000069283:Rituximab