title: First-line treatment of pemphigus vulgaris with a combination of rituximab and high-potency topical corticosteroids.
abstract: OBJECTIVE
  The main component of the first-line treatment of pemphigus vulgaris is high doses of systemic corticosteroids, but adverse effects of these drugs are frequent and sometimes severe. Rituximab has shown effectiveness as a corticosteroid-sparing agent or in case of relapse. To our knowledge, the effectiveness of rituximab as a first-line treatment without systemic corticosteroids has not been evaluated.
  
  
  METHODS
  Five women in their 50s, 60s, or 70s with pemphigus vulgaris (Pemphigus Disease Area Index score, 15-84 at diagnosis) and contraindications to systemic corticosteroid treatment received rituximab with high-potency topical corticosteroids as first-line treatment. All patients experienced a favorable response, with a mean time to healing of skin and mucosal lesions of 15 weeks. Two patients, with 42- and 48-month follow-up evaluations, did not experience relapse. Three patients developed 2 to 4 relapses, with effective retreatment achieved using rituximab and topical corticosteroids. No severe adverse effects were observed.
  
  
  CONCLUSIONS
  Considering the high rate of severe adverse effects induced by prolonged administration of high doses of systemic corticosteroids, new therapeutic options are warranted in the treatment of pemphigus vulgaris. The combination of rituximab and topical corticosteroids could be considered in mild to severe cutaneous disease. Larger long-term studies are needed to evaluate the optimal treatment strategies according to the severity of the disease and the benefit-risk ratio of rituximab.
authors.affiliation: Department of Dermatology, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France.
authors.forename: Saskia
authors.initials: S
authors.lastname: Ingen-Housz-Oro
authors.affiliation: Department of Dermatology, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Valeyrie-Allanore
authors.affiliation: Department of Dermatology, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Cosnes
authors.affiliation: Department of Pathology, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France3Université Paris-Est Créteil Val de Marne, Créteil, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Ortonne
authors.affiliation: Université Paris-Est Créteil Val de Marne, Créteil, France4Department of Immunology, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Hüe
authors.affiliation: Department of Pharmacy, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France.
authors.forename: Muriel
authors.initials: M
authors.lastname: Paul
authors.affiliation: Department of Dermatology, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France3Université Paris-Est Créteil Val de Marne, Créteil, France6Institut National de la Santé et de la Recherche Médicale, Centre d'Investigation Clinique.
authors.forename: Pierre
authors.initials: P
authors.lastname: Wolkenstein
authors.affiliation: Department of Dermatology, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France3Université Paris-Est Créteil Val de Marne, Créteil, France6Institut National de la Santé et de la Recherche Médicale, Centre d'Investigation Clinique.
authors.forename: Olivier
authors.initials: O
authors.lastname: Chosidow
mesh_terms: D000287:Administration, Topical; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage*; D018951:Antigens, CD20; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage; D007262:Infusions, Intravenous; D008875:Middle Aged; D010392:Pemphigus / Q000188:drug therapy*; D012074:Remission Induction; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D005938:Glucocorticoids; D007155:Immunologic Factors; D000069283:Rituximab