Correlative bone imaging in a case of Schnitzler's syndrome and brief review of the literature.

ABSTRACT

UNLABELLED 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. CONCLUSION: 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.