Differentiating urticaria from other diseases

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

Overview

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

Hereditary or acquired deficiency of complement factor C1
Cutaneous mastocytosis/urticaria pigmentosa
Malignancy
Exercise‐induced anaphylaxis^[1]
Connective tissue diseases
Photosensitive diseases
Poison ivy contact dermatitis: The rash that develops from poison ivy, poison oak, and poison sumac contact are commonly mistaken for urticaria. This rash is caused by contact with urushiol and results in a form of contact dermatitis called urushiol-induced contact dermatitis.
Angioedema:
- Although it might be related to urticaria, angioedema occurs due to swelling of the lower layers of dermis.^[2]
- Edema can occur around the mouth, in the throat, in the abdomen, or in other locations. Urticaria and angioedema sometimes occur together in response to an allergen and should be a concern in severe cases because angioedema of the throat can be fatal.
A possible differential diagnosis for diseases that cause urticaria, and rash include:
Table below differentiates some of the aforementioned conditions:


Disease name	Age of onset	Signs/Symptoms	Diagnostic feature(s)	Other features
Cold Contact Urticaria^[3]^[4]	Early adulthood	Urticarial skin eruption after exposure to cold Urticaria and/or angioedema affects areas only in contact with cold Systemic symptoms may be present in case of extensive skin involvement (such as anaphylaxis)	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	Non-pruritic urticarial rash Fever Headache Fatigue Conjunctivitis	Ice cube test is negative	Cold-induced rash Skin biopsy shows increased neutrophils at the eccrine coils
Schnitzler syndrome^[6]	Middle age	Chronic urticarial rash Fever Arthralgia Bone pain	Monoclonal gammopathy	Rash is the presenting sign Neutrophillic infiltrate in dermis Dermographism
Deficiency in Interleukin-1 Receptor Antagonist^[7]	Infancy	Pustular rash Bone pain Swollen joints Aphthous ulcers	Occurs due to mutation in IL1RN gene Pustular rash similar to pustular psoriasis	Epidermal neutrophilic pustules at hair follicles Pathergy Hyperkeratosis and acanthosis High neutrophil infiltrate of the dermis
Systemic-Onset Juvenile Idiopathic Arthritis^[8]	2-16 years of age	High fever Arthralgia Generalized lymphadenopathy Splenomegaly	Evanescent rash Bright salmon-pink Morbilliform, macular rash Rash presents with onset of fever	May cause amyloidosis
Adult-Onset Still’s Disease^[9]	16-35 years May affect all ages	High spiking fever Joint pain Skin rash Lymphadenopathy Splenomegaly	Evanescent salmon-colored bumpy rash Pruritic rash on the proximal limbs and trunk	More commonly seen among women May cause destructive arthritis

References

↑ Zuberbier T (2003). “Urticaria”. Allergy. 58 (12): 1224–34. doi:10.1046/j.1398-9995.2003.00327.x. PMID 14616095.
↑ “Hives (Urticaria and Angioedema)”. 2006-03-01. Retrieved 2007-08-24.
↑ 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.
↑ 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.
↑ Kastner, D. L. (2005). “Hereditary Periodic Fever Syndromes”. Hematology. 2005 (1): 74–81. doi:10.1182/asheducation-2005.1.74. ISSN 1520-4391.
↑ 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.
↑ 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.
↑ 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.
↑ 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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[pmid14616095-1] 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.

[SiebenhaarWeller2007-3] 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.

[KrauseZuberbier2010-4] 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.

[Kastner2005-5] Kastner, D. L. (2005). “Hereditary Periodic Fever Syndromes”. Hematology. 2005 (1): 74–81. doi:10.1182/asheducation-2005.1.74. ISSN 1520-4391.

[de_KoningBodar2007-6] 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.

[AksentijevichMasters2009-7] 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.

[GurionLehman2012-8] 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.

[Efthimiou2006-9] 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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