Schnitzler syndrome
MONDO:0018304Mondo
Findings
No curated finding names Schnitzler syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A rare, underdiagnosed disorder in adults characterized by recurrent febrile rash, bone and/or joint pain, enlarged lymph nodes, fatigue, a monoclonal IgM component, leukocytosis and systemic inflammatory response.
Definition from the Mondo Disease Ontology (MONDO:0018304), read 2026-09-29. CC BY 4.0.
Features
21 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- ArthralgiaHPOHP:0002829
- Very frequent (80% to 99% of cases)
- ArthritisHPOHP:0001369
- Very frequent (80% to 99% of cases)
- Bone painHPOHP:0002653
- Very frequent (80% to 99% of cases)
- FeverHPOHP:0001945
- Very frequent (80% to 99% of cases)
- HepatomegalyHPOHP:0002240
- Very frequent (80% to 99% of cases)
- Increased bone mineral densityHPOHP:0011001
- Very frequent (80% to 99% of cases)
- Increased circulating IgM concentrationHPOHP:0003496
- Very frequent (80% to 99% of cases)
- MaculeHPOHP:0012733
- Very frequent (80% to 99% of cases)
- MyalgiaHPOHP:0003326
- Very frequent (80% to 99% of cases)
- PapuleHPOHP:0200034
- Very frequent (80% to 99% of cases)
- Skin rashHPOHP:0000988
- Very frequent (80% to 99% of cases)
- SplenomegalyHPOHP:0001744
- Very frequent (80% to 99% of cases)
Show the remaining 9
- UrticariaHPOHP:0001025
- Very frequent (80% to 99% of cases)
- AnemiaHPOHP:0001903
- Frequent (30% to 79% of cases)
- FatigueHPOHP:0012378
- Frequent (30% to 79% of cases)
- Increased total leukocyte countHPOHP:0001974
- Frequent (30% to 79% of cases)
- LymphadenopathyHPOHP:0002716
- Occasional (5% to 29% of cases)
- LymphomaHPOHP:0002665
- Occasional (5% to 29% of cases)
Where it sits
- A kind of
Other names
2 names
Resolves to: Schnitzler syndrome
- Also called
- chronic urticaria with gammopathychronic urticaria with macroglobulinemia