Differentiating urticaria from other diseases

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Differentiating Urticaria from other Diseases

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2]


It is critical to differentiate urticaria from other similar disorders to utilize the best approach for the treatment. Hereditary or acquired deficiency of complement factor C1, cutaneous mastocytosis such as urticaria pigmentosa, certain malignancies, connective tissue diseases, angioedema and exercise‐induced anaphylaxis are some of the differential diagnosis of urticaria.

Differentiating Urticaria from other Diseases

Disease name Age of onset Signs/Symptoms Diagnostic feature(s) Other features
Cold Contact Urticaria[3][4]
  • Early adulthood
  • Ice cube test is positive
  • The wheal appears within 5 minutes of cold contact
Familial Cold Autoinflammatory Syndrome[5]
  • Infancy, but may be delayed until adulthood

  • Ice cube test is negative
Schnitzler syndrome[6]
Deficiency in Interleukin-1 Receptor Antagonist[7]
Systemic-Onset Juvenile Idiopathic Arthritis[8]
  • 2-16 years of age
Adult-Onset Still’s Disease[9]
  • 16-35 years
  • May affect all ages
  • More commonly seen among women
  • May cause destructive arthritis


  1. Zuberbier T (2003). "Urticaria". Allergy. 58 (12): 1224–34. doi:10.1046/j.1398-9995.2003.00327.x. PMID 14616095.
  2. "Hives (Urticaria and Angioedema)". 2006-03-01. Retrieved 2007-08-24.
  3. Siebenhaar, F.; Weller, K.; Mlynek, A.; Magerl, M.; Altrichter, S.; Vieira dos Santos, R.; Maurer, M.; Zuberbier, T. (2007). "Acquired cold urticaria: clinical picture and update on diagnosis and treatment". Clinical and Experimental Dermatology. 32 (3): 241–245. doi:10.1111/j.1365-2230.2007.02376.x. ISSN 0307-6938.
  4. Krause, Karoline; Zuberbier, Torsten; Maurer, Marcus (2010). "Modern Approaches to the Diagnosis and Treatment of Cold Contact Urticaria". Current Allergy and Asthma Reports. 10 (4): 243–249. doi:10.1007/s11882-010-0121-3. ISSN 1529-7322.
  5. Kastner, D. L. (2005). "Hereditary Periodic Fever Syndromes". Hematology. 2005 (1): 74–81. doi:10.1182/asheducation-2005.1.74. ISSN 1520-4391.
  6. de Koning, Heleen D.; Bodar, Evelien J.; van der Meer, Jos W.M.; Simon, Anna (2007). "Schnitzler Syndrome: Beyond the Case Reports: Review and Follow-Up of 94 Patients with an Emphasis on Prognosis and Treatment". Seminars in Arthritis and Rheumatism. 37 (3): 137–148. doi:10.1016/j.semarthrit.2007.04.001. ISSN 0049-0172.
  7. Aksentijevich, Ivona; Masters, Seth L.; Ferguson, Polly J.; Dancey, Paul; Frenkel, Joost; van Royen-Kerkhoff, Annet; Laxer, Ron; Tedgård, Ulf; Cowen, Edward W.; Pham, Tuyet-Hang; Booty, Matthew; Estes, Jacob D.; Sandler, Netanya G.; Plass, Nicole; Stone, Deborah L.; Turner, Maria L.; Hill, Suvimol; Butman, John A.; Schneider, Rayfel; Babyn, Paul; El-Shanti, Hatem I.; Pope, Elena; Barron, Karyl; Bing, Xinyu; Laurence, Arian; Lee, Chyi-Chia R.; Chapelle, Dawn; Clarke, Gillian I.; Ohson, Kamal; Nicholson, Marc; Gadina, Massimo; Yang, Barbara; Korman, Benjamin D.; Gregersen, Peter K.; van Hagen, P. Martin; Hak, A. Elisabeth; Huizing, Marjan; Rahman, Proton; Douek, Daniel C.; Remmers, Elaine F.; Kastner, Daniel L.; Goldbach-Mansky, Raphaela (2009). "An Autoinflammatory Disease with Deficiency of the Interleukin-1–Receptor Antagonist". New England Journal of Medicine. 360 (23): 2426–2437. doi:10.1056/NEJMoa0807865. ISSN 0028-4793.
  8. Gurion, R.; Lehman, T. J. A.; Moorthy, L. N. (2012). "Systemic Arthritis in Children: A Review of Clinical Presentation and Treatment". International Journal of Inflammation. 2012: 1–16. doi:10.1155/2012/271569. ISSN 2090-8040.
  9. Efthimiou, P (2006). "Diagnosis and management of adult onset Still's disease". Annals of the Rheumatic Diseases. 65 (5): 564–572. doi:10.1136/ard.2005.042143. ISSN 0003-4967.

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