*Chronic urticaria
| Chronic autoimmune urticaria | {Chronic urticaria with gammopathy | Chronic urticaria with macroglobulinemia | Schnitzler syndrome}
Summary
*Chronic urticaria
The Global Autoimmune Institute includes “Chronic urticaria” in its list of autoimmune diagnoses with an asterisk, meaning autoimmune associated or suspected autoimmune. Its description is simple:
Urticaria is an allergic skin reaction that results in hives. When these hives appear for longer than six weeks and are recurrent over the course of months or years, the condition is classified as chronic.
The story behind Chronic urticaria is far more complex. Chronic urticaria is an umbrella term for chronic hives that can be the result of a variety of causes, one of which is autoimmune. In their 2009 review article on “chronic autoimmune urticaria” Goh and Tan note
It is well-recognized that 30-40% of chronic Idiopathic {unknown cause} urticaria is autoimmune in nature.
The authors go on to review known autoimmunity mechanisms in Chronic Autoimmune Urticaria and the relevance of diagnostic tests to determine whether an autoimmune cause is detectable. If an autoimmune cause is detectable, then a person’s Chronic Urticaria can now be classified as Chronic Autoimmune Urticaria. If an autoimmune cause is not detectable, then a person’s Chronic Urticaria may be considered Chronic Idiopathic Urticaria, which does not rule out an autoimmune cause, it simply means that the cause is unknown and an autoimmune cause is not detectable at this time with the diagnostic testing that is available.
Autoimmune urticaria
The Autoimmune Association lists “Autoimmune urticaria” in their diagnostic list. In their description section, they give a description of “Schnitzler syndrome” {likely autoimmune} and “Cold urticaria” {unlikely autoimmune}. Their resource for Schnitzler syndrome is the Genetic and Rare Diseases (GARD) Information Center. On the GARD website, their description of Schnitzler syndrome lists synonyms as “chronic urticaria with gammopathy” and “chronic urticaria with macroglobulinemia.” Based on the description of “Cold urticaria” on the Autoimmune Association and GARD websites, it is not known to be associated with an autoimmune process, so I am excluding it from this description.
Chronic urticaria with gammopathy / Chronic urticaria with macroglobulinemia / Schnitzler syndrome
Schnitzler syndrome is a rare autoinflammatory condition. Signs and symptoms of the condition vary but may include urticaria; recurrent fevers; joint pain and inflammation; organomegaly (abnormally enlarged organs); and/or blood abnormalities. The exact underlying cause of the condition is unknown; however, most cases occur sporadically in people with no family history of the condition.
From the Genetic and Rare Diseases Information Center.
Symptoms
Coming Soon
Diagnostic Criteria
Under Investigation
Diagnostic Tests
Under Investigation
Human Leukocyte Antigen (HLA) Associations
Under Investigation
Tissue-Type or Cell-Type Attacked
Under Investigation
Attacking Immune Cell Type or Process
Under Investigation
Treatment Modality
Under Investigation
Managing Specialist(s)
Under Investigation
Associated Infections
Under Investigation
Associated Toxins
Under Investigation
Research Authors
Under Investigation
Research Institutions
Under Investigation
Average Time from Symptom Onset to Diagnosis
Under Investigation
Last Updated
October 24, 2022
References
Autoimmune urticaria. Autoimmune Association. (2022, February 11). Retrieved October 24, 2022, from https://autoimmune.org/disease-information/autoimmune-urticaria/
Chronic urticaria. Global Autoimmune Institute. (2022). Retrieved October 24, 2022, from https://www.autoimmuneinstitute.org/chronic-urticaria/
Goh CL, Tan KT. Chronic autoimmune urticaria: where we stand? Indian J Dermatol. 2009 Jul;54(3):269-74. doi: 10.4103/0019-5154.55640. PMID: 20161862; PMCID: PMC2810697.
Schnitzler syndrome. Genetic and Rare Diseases Information Center, National Center for Advancing Translational Sciences. (2021, November 8). Retrieved October 24, 2022, from https://rarediseases.info.nih.gov/diseases/12390/schnitzler-syndrome