title: Report of a case of Schnitzler's syndrome treated successfully with interferon alpha 2b.
abstract: Schnitzler's syndrome (SS) is characterized by the association of generalized chronic urticaria, osteocondensation and monoclonal IgM gammopathy. Nonsteroidal anti-inflammatory drugs and systemic steroids are the most promising treatments. In our patient, they were ineffective. By contrast, during the follow-up period of 18 months, interferon alpha(2b) therapy (IFN-alpha) relieved the patient from its urticarial lesions and bone pain. IFN-alpha was tried to be stopped twice: each time, relapse of urticaria was noticed and, each time, the cutaneous lesions disappeared after IFN-alpha had been reintroduced. Furthermore, our observation supports the idea of the interleukin (IL)-1-mediated pathogenesis of SS as IFN-alpha induces high levels of IL-1 receptor antagonists. IFN-alpha could be an alternative treatment in disabling SS resisting other drugs.
authors.affiliation: Department of Dermatology, University Medical Centre Charité, Humboldt University of Berlin, Germany. schartz@igr.fr
authors.forename: Noël Emile Célestin
authors.initials: NE
authors.lastname: Schartz
authors.forename: Suzanne
authors.initials: S
authors.lastname: Buder
authors.forename: Hans
authors.initials: H
authors.lastname: Sperl
authors.forename: Heike
authors.initials: H
authors.lastname: Audring
authors.forename: Ralph
authors.initials: R
authors.lastname: Paus
authors.forename: Beate
authors.initials: B
authors.lastname: Tebbe
authors.forename: Katharina
authors.initials: K
authors.lastname: Krüger
authors.forename: Wolfram
authors.initials: W
authors.lastname: Sterry
mesh_terms: D001842:Bone and Bones; D006801:Humans; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010146:Pain / Q000150:complications; D011994:Recombinant Proteins; D019873:Schnitzler Syndrome / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D012867:Skin / Q000473:pathology; D014581:Urticaria / Q000150:complications / Q000473:pathology
chemical_list: D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins