title: Therapy of paraneoplastic pemphigus with Rituximab: a case report and review of literature.
abstract: Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease with poor prognosis when associated with malignant neoplasm. We report the case of a patient with PNP associated with a CD20+ non-Hodgkin follicular lymphoma who was treated with Rituximab plus corticosteroids and short courses of cyclosporin. One and a half years after Rituximab therapy, oral ulcerations had cleared and oral methylprednisolone was slowly tapered down without further recurrences. In the course of the disease, the patient developed sepsis due to Listeria monocytogenes and viral infections by human herpes virus 1 and 3. At the end-stage of the disease she developed a cutaneous infection from Mycobacterium chelonae. The patient died 2 years and 7 months after the onset of PNP. Rituximab may be useful for PNP therapy, but further studies are necessary to confirm this hypothesis.
authors.affiliation: Department of Dermatology, Hospital Sta. Creu i St. Pau, Barcelona, Spain. mbarnadas@hsp.santpau.es
authors.forename: Ma
authors.initials: M
authors.lastname: Barnadas
authors.forename: E
authors.initials: E
authors.lastname: Roe
authors.forename: S
authors.initials: S
authors.lastname: Brunet
authors.forename: P
authors.initials: P
authors.lastname: Garcia
authors.forename: P
authors.initials: P
authors.lastname: Bergua
authors.forename: L
authors.initials: L
authors.lastname: Pimentel
authors.forename: L
authors.initials: L
authors.lastname: Puig
authors.forename: A
authors.initials: A
authors.lastname: Francia
authors.forename: R
authors.initials: R
authors.lastname: García
authors.forename: C
authors.initials: C
authors.lastname: Gelpí
authors.forename: J
authors.initials: J
authors.lastname: Sierra
authors.forename: P
authors.initials: P
authors.lastname: Coll
authors.forename: A
authors.initials: A
authors.lastname: Alomar
mesh_terms: D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D010257:Paraneoplastic Syndromes / Q000188:drug therapy* / Q000473:pathology; D010392:Pemphigus / Q000188:drug therapy* / Q000473:pathology; D000069283:Rituximab
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab