Schnitzler syndrome: Difference between revisions
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==Clinical Manifestations== |
==Clinical Manifestations== |
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* Recurrent, non-pruritic [[urticaria]] |
* Recurrent, non-pruritic [[urticaria]] |
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* Monoclonal IgM gammopathy |
* [[Monoclonal IgM gammopathy]] |
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* At least two of: |
* At least two of: |
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** Intermittent fever |
** Intermittent fever |
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** Bone pain |
** Bone pain |
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** Arthralgias or arthritis |
** [[Arthralgia|Arthralgias]] or [[arthritis]] |
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** Lymphadenopathy |
** [[Lymphadenopathy]] |
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** Hepatomegaly and/or splenomegaly |
** [[Hepatomegaly]] and/or [[splenomegaly]] |
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** Leukocytosis |
** [[Leukocytosis]] |
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=== Complications and Prognosis === |
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* About 20% of cases progress to [[lymphoproliferative disorder]] |
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== Investigations == |
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* Histopathology showed neutrophili urticarial dermatosis, with perivascular and interstitial neutrophilic inflammation and leukocytoclasia, but without true leukocytoclastic vasculitis |
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== Further Reading == |
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<nowiki>*</nowiki> [[CiteRef::sokumbi2012cl]] |
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[[Category:Rheumatology]] |
[[Category:Rheumatology]] |
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[[Category:Allergy]] |
[[Category:Allergy]] |
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[[Category:Dermatology]] |
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[[Category:Hematology]] |
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Latest revision as of 17:09, 18 February 2022
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.
