title: Correlative bone imaging in a case of Schnitzler's syndrome and brief review of the literature.
abstract: Schnitzler's syndrome is a rare disease characterized by a monoclonal IgM (or IgG) paraprotein, a nonpruritic urticarial skin rash, and 2 (or 3) of the following: recurrent fever, objective signs of abnormal bone remodeling, elevated CRP level or leukocytosis, and a neutrophilic infiltrate on skin biopsy. It responds well to treatment with the interleukine-1-inhibitor anakinra. We report the bone scintigraphy and MRI findings in a 45 years old man with this syndrome and compare them with data from the literature.
  
  
  CONCLUSIONS
  None of the imaging findings are specific, but they lead to a differential diagnosis including infiltrative diseases (e.g. systemic mastocytosis or Erdheim-Chester disease) and dysplastic diseases (e.g. melorheostosis, Camurati-Engelmann disease or van Buchem disease). The bone scintigraphy pattern may be very suggestive of the correct diagnosis and of bone involvement in this syndrome.
authors.affiliation: Department of Nuclear Medicine Algemeen Ziekenhuis, Sint-Jan Brugge-Oostende Ruddershove 10, B-8000 Brugge, Belgium. frank.degeeter@azsintjan.be.
authors.forename: Inneke
authors.initials: I
authors.lastname: Willekens
authors.forename: Natascha
authors.initials: N
authors.lastname: Walgraeve
authors.forename: Lode
authors.initials: L
authors.lastname: Goethals
authors.forename: Frank
authors.initials: F
authors.lastname: De Geeter
mesh_terms: D001706:Biopsy; D001842:Bone and Bones / Q000000981:diagnostic imaging* / Q000378:metabolism; D005076:Exanthema; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use; D007964:Leukocytosis / Q000097:blood; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009504:Neutrophils / Q000166:cytology; D011877:Radionuclide Imaging / Q000379:methods*; D019873:Schnitzler Syndrome / Q000175:diagnosis* / Q000000981:diagnostic imaging*; D012867:Skin / Q000473:pathology; D016896:Treatment Outcome; D051598:Whole Body Imaging / Q000379:methods
chemical_list: D053590:Interleukin 1 Receptor Antagonist Protein