Schnitzler syndrome
From IDWiki
Background
- Rare multisystem disorder characterized by urticaria and monoclonal gammopathy
- Associated with malignancy
Clinical Manifestations
- Recurrent, non-pruritic urticaria
- Monoclonal IgM gammopathy
- At least two of:
- Intermittent fever
- Bone pain
- Arthralgias or arthritis
- Lymphadenopathy
- Hepatomegaly and/or splenomegaly
- Leukocytosis
Complications and Prognosis
- About 20% of cases progress to lymphoproliferative disorder
Investigations
- Histopathology showed neutrophili urticarial dermatosis, with perivascular and interstitial neutrophilic inflammation and leukocytoclasia, but without true leukocytoclastic vasculitis
Further Reading
* 1
References
- ^ Olayemi Sokumbi, Lisa A. Drage, Margot S. Peters. Clinical and histopathologic review of Schnitzler syndrome: The Mayo Clinic experience (1972-2011). Journal of the American Academy of Dermatology. 2012;67(6):1289-1295. doi:10.1016/j.jaad.2012.04.027.
