Cold urticaria

Last updated
Cold Urticaria
Other namesCold hives
Cold Urticaria.jpg
Allergic urticaria on arm in the form of hives induced by cold.
Specialty Dermatology
CausesExposure to cold stimulus

Cold urticaria (essentially meaning cold hives) is a disorder in which large red welts called hives (urticaria) form on the skin after exposure to a cold stimulus. [1] The hives are usually itchy and often the hands, feet and other parts of the body will become itchy and swollen as well. Hives vary in size from about 7  mm in diameter to as big as about 27 mm or larger.

Contents

This disorder, or perhaps two disorders with the same clinical manifestations, can be inherited (familial cold urticaria) or acquired (primary acquired cold urticaria). The acquired form is most likely to begin between ages 18 and 25, although it can occur as early as 5 years old in some cases. Life-threatening risks include suffocation resulting from swollen tissue (pharyngeal angioedema) induced by cold foods or beverages, drowning after shock from swimming in cold water, and anaphylactic shock. [2]

Types

Cold urticaria may be divided into the following types: [3]

Primary cold contact urticaria

Primary cold contact urticaria is a cutaneous condition characterized by wheals, and occurs in rainy, windy weather, and after swimming in cold water and after contact with cold objects, including ice cubes. [3]

Secondary cold contact urticaria

Secondary cold contact urticaria is a cutaneous condition characterized by wheals, due to serum abnormalities such as cryoglobulinemia or cryofibrinogenemia are extremely rare, and are then associated with other manifestations such as Raynaud's phenomenon or purpura. [3]

Reflex cold urticaria

Reflex cold urticaria is a cutaneous condition in which generalized cooling of the body induces widespread welting. [3]

Familial cold urticaria

Familial cold urticaria (also properly known as familial cold autoinflammatory syndrome, FCAS) is an autosomal dominant condition characterized by rash, conjunctivitis, fever/chills and arthralgias [4] elicited by exposure to cold – sometimes temperatures below 22 °C (72 °F). [3] [5]

It has been mapped to CIAS1 [6] and is a slightly milder member of the disease family including Muckle–Wells syndrome and NOMID. It is rare and is estimated as having a prevalence of 1 per million people and mainly affects Americans and Europeans. [7]

FCAS is one of the cryopyrin-associated periodic syndromes (CAPS) caused by mutations in the CIAS1/NALP3 (aka NLRP3) gene at location 1q44. [8] [9] [10] [11]

The effect of FCAS on the quality of life of patients is far reaching. A survey of patients in the United States in 2008 found that "[t]o cope with their underlying disease and to try to avoid symptomatic, painful, flares patients reported limiting their work, school, family, and social activities. Seventy-eight percent of survey participants described an impact of the disease on their work, including absenteeism and impaired job advancement; frequently, they quit their job as a consequence of their disease". [12]

Treatment using anakinra (Kineret) has been shown effective for FCAS, although this does mean daily injections of the immunosuppressant into an area such as the lower abdomen. [13] [14] The monoclonal antibody canakinumab (Ilaris) is also used. [15]

Signs and symptoms

Hives on the back from exposure to cold air on an individual with cold allergy. The hives were induced by riding a stationary bike shirtless for an hour next to a door cracked open on a cool day. The temperature of the air flowing in was around 10 degC (50 degF). The lighter band at chest height was covered by a heart rate monitor strap. Cold allergy symptoms.jpg
Hives on the back from exposure to cold air on an individual with cold allergy. The hives were induced by riding a stationary bike shirtless for an hour next to a door cracked open on a cool day. The temperature of the air flowing in was around 10 °C (50 °F). The lighter band at chest height was covered by a heart rate monitor strap.

When the body is exposed to the cold in individuals affected by the condition, hives appear and the skin in the affected area typically becomes itchy. Hives result from dilation of capillaries which allow fluid to flow out into the surrounding tissue which is the epidermis. They resolve when the body absorbs this fluid.[ citation needed ] The border of a hive is described as polycyclic, or made up of many circles, and changes as fluid leaks out and then is absorbed. [16] Pressing on a hive causes the skin to blanch (turn pale as blood flow is interrupted), distinguishing it from a bruise or papule.

Hives may appear immediately or after a delay. [17] Hives can last for a few minutes or a few days, and vary from person to person. Also, a burning sensation occurs. A serious reaction is most likely to occur for patients where the hives occur with less than three minutes of exposure (during a cold test). [18]

Cause

The hives are a histamine reaction in response to cold stimuli, including a drastic drop in temperature, cold air, and cold water. There are many causes for cold hives, most are idiopathic (meaning they have no known cause). Some rare conditions[ specify ] can cause cold hives, and it can be useful to test for these conditions if the cold hives are in any way unusual.

Scientists from the USA National Institutes of Health have identified a genetic mutation in three unrelated families that causes a rare immune disorder characterized by excessive and impaired immune function: immune deficiency, autoimmunity, inflammatory skin disorders and cold-induced hives (cold urticaria).

"The mutation discovered occurs in a gene for phospholipase C-gamma2 (PLCG2), an enzyme involved in the activation of immune cells. The investigators have named the condition PLCG2-associated antibody deficiency and immune dysregulation, or PLAID." [19]

Diagnosis

Diagnosis is typically obtained by an allergist or other licensed practitioner performing a cold test. During the cold test, a piece of ice is placed inside a thin plastic bag and held against the forearm, typically for 3–4 minutes. [20] A positive result is a specific looking mark of raised red hives. The hives may be the shape of the ice, or it may radiate from the contact area of the ice. [21] However, while these techniques assist in diagnosis, they do not provide information about temperature and stimulation time thresholds at which patients will start to develop symptoms, [21] which is essential because it can establish disease severity and monitor the effectiveness of treatment. [21]

Management

Management largely consists of avoiding exposures that could trigger a reaction and taking medicine to manage the reaction. The best treatment for this allergy is avoiding exposure to cold temperatures. [22]

Avoiding triggers

Anything that lowers the skin temperature (not body's core temperature) can trigger a reaction in affected people. Avoiding exposures limits the risk of a reaction. The reaction usually affects the body parts that are exposed, so cold air could trigger a reaction to exposed skin or in the breathing passages if cold air is inhaled. Risky exposures include:

Drugs

The first-line therapy is symptomatic relief with second-generation H1-antihistamines. [23] If standard dosing is ineffective, increasing dosages up to fourfold is recommended to control symptoms. [24]

The second-generation H1-antihistamine rupatadine was found to significantly reduce the development of chronic cold urticaria symptom without an increase in adverse effects at doses of 20  mg and 40 mg. [21]

Allergy medications containing antihistamines such as diphenhydramine (Benadryl), cetirizine (Zyrtec), loratadine (Claritin), cyproheptadine (Periactin), and fexofenadine (Allegra) may be taken orally to prevent and relieve some of the hives. [25] For those who have severe anaphylactic reactions, a prescribed medicine such as doxepin, taken daily, should help to prevent and/or lessen the likelihood of a reaction and thus, anaphylaxis. The effectiveness of topical antihistamine creams against hives induced by cold temperature has not been evaluated.[ citation needed ]

Cold hives can result in a potentially serious or even fatal systemic reaction (anaphylactic shock). People with cold hives may have to carry an injectable form of epinephrine (like Epi-pen or Twinject) for use in the event of a serious reaction. [26]

Studies have found that omalizumab (Xolair) may be an effective and safe treatment for cold urticaria in patients who do not sufficiently respond to standard treatments. [27]

Ebastine has been proposed as an approach to prevent acquired cold urticaria. [28]

See also

Related Research Articles

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Mastocytosis, a type of mast cell disease, is a rare disorder affecting both children and adults caused by the accumulation of functionally defective mast cells and CD34+ mast cell precursors.

<span class="mw-page-title-main">Contact dermatitis</span> Inflammation from allergen or irritant exposure

Contact dermatitis is a type of acute or chronic inflammation of the skin caused by exposure to chemical or physical agents. Symptoms of contact dermatitis can include itchy or dry skin, a red rash, bumps, blisters, or swelling. These rashes are not contagious or life-threatening, but can be very uncomfortable.

<span class="mw-page-title-main">Hives</span> Skin disease characterized by red, raised, and itchy bumps

Hives, also known as urticaria, is a kind of skin rash with red and/or flesh-colored, raised, itchy bumps. Hives may burn or sting. The patches of rash may appear on different body parts, with variable duration from minutes to days, and do not leave any long-lasting skin change. Fewer than 5% of cases last for more than six weeks. The condition frequently recurs.

<span class="mw-page-title-main">Dermatographic urticaria</span> Skin disorder

Dermatographic urticaria is a skin disorder and one of the most common types of urticaria, affecting 2–5% of the population.

Aquagenic pruritus is a skin condition characterized by the development of severe, intense, prickling-like epidermal itching without observable skin lesions and evoked by contact with water.

<span class="mw-page-title-main">Urticaria pigmentosa</span> Most common form of cutaneous mastocytosis

Urticaria pigmentosa (also known as generalized eruption of cutaneous mastocytosis (childhood type) ) is the most common form of cutaneous mastocytosis. It is a rare disease caused by excessive numbers of mast cells in the skin that produce hives or lesions on the skin when irritated.

<span class="mw-page-title-main">Physical urticaria</span> Medical condition

Physical urticaria is a distinct subgroup of urticaria (hives) that are induced by an exogenous physical stimulus rather than occurring spontaneously. There are seven subcategories that are recognized as independent diseases. Physical urticaria is known to be painful, itchy and physically unappealing; it can recur for months to years.

<span class="mw-page-title-main">Muckle–Wells syndrome</span> Medical condition

Muckle–Wells syndrome (MWS) is a rare autosomal dominant disease which causes sensorineural deafness and recurrent hives, and can lead to amyloidosis. Individuals with MWS often have episodic fever, chills, and joint pain. As a result, MWS is considered a type of periodic fever syndrome. MWS is caused by a defect in the CIAS1 gene which creates the protein cryopyrin. MWS is closely related to two other syndromes, familial cold urticaria and neonatal onset multisystem inflammatory disease—in fact, all three are related to mutations in the same gene and subsumed under the term cryopyrin-associated periodic syndromes (CAPS).

<span class="mw-page-title-main">Cholinergic urticaria</span> Medical condition

Cholinergic urticaria or also known as (CholU) and CU, is a rare form of hives (urticaria) that is triggered by an elevation in body temperature, breaking a sweat, or exposure to heat. It is also sometimes called exercise-induced urticaria or heat hives. The condition is considered to be one of the many rarest forms of allergies known to medical science.

A drug allergy is an allergy to a drug, most commonly a medication, and is a form of adverse drug reaction. Medical attention should be sought immediately if an allergic reaction is suspected.

<span class="mw-page-title-main">Solar urticaria</span> Medical condition

Solar urticaria (SU) is a rare condition in which exposure to ultraviolet or UV radiation, or sometimes even visible light, induces a case of urticaria or hives that can appear in both covered and uncovered areas of the skin. It is classified as a type of physical urticaria. The classification of disease types is somewhat controversial. One classification system distinguished various types of SU based on the wavelength of the radiation that causes the breakout; another classification system is based on the type of allergen that initiates a breakout.

Serum sickness in humans is a reaction to proteins in antiserum derived from a non-human animal source, occurring 5–10 days after exposure. Symptoms often include a rash, joint pain, fever, and lymphadenopathy. It is a type of hypersensitivity, specifically immune complex hypersensitivity. The term serum sickness–like reaction (SSLR) is occasionally used to refer to similar illnesses that arise from the introduction of certain non-protein substances, such as penicillin.

Aquagenic urticaria, also known as water allergy and water urticaria, is a form of physical urticaria in which hives develop on the skin after contact with water, regardless of its temperature. The condition typically results from contact with water of any type, temperature or additive.

Schnitzler syndrome or Schnitzler's syndrome is a rare disease characterised by onset around middle age of chronic hives (urticaria) and periodic fever, bone and joint pain, weight loss, malaise, fatigue, swollen lymph glands and enlarged spleen and liver.

One of the most prevalent forms of adverse drug reactions is cutaneous reactions, with drug-induced urticaria ranking as the second most common type, preceded by drug-induced exanthems. Urticaria, commonly known as hives, manifests as weals, itching, burning, redness, swelling, and angioedema—a rapid swelling of lower skin layers, often more painful than pruritic. These symptoms may occur concurrently, successively, or independently. Typically, when a drug triggers urticaria, symptoms manifest within 24 hours of ingestion, aiding in the identification of the causative agent. Urticaria symptoms usually subside within 1–24 hours, while angioedema may take up to 72 hours to resolve completely.

<span class="mw-page-title-main">Cryopyrin-associated periodic syndrome</span> Medical condition

Cryopyrin-associated periodic syndrome (CAPS) is a group of rare, heterogeneous autoinflammatory disease characterized by interleukin 1β-mediated systemic inflammation and clinical symptoms involving skin, joints, central nervous system, and eyes. It encompasses a spectrum of three clinically overlapping autoinflammatory syndromes including familial cold autoinflammatory syndrome, the Muckle–Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease that were originally thought to be distinct entities, but in fact share a single genetic mutation and pathogenic pathway, and keratoendotheliitis fugax hereditaria in which the autoinflammatory symptoms affect only the anterior segment of the eye.

<span class="mw-page-title-main">Bilastine</span> Antihistamine medication

Bilastine is an antihistamine medication used to treat hives (urticaria), allergic rhinitis and itchy inflamed eyes (allergic conjunctivitis) caused by an allergy. It is a second-generation antihistamine and takes effect by selectively inhibiting the histamine H1 receptor, preventing these allergic reactions. Bilastine has an effectiveness similar to cetirizine, fexofenadine, and desloratadine.

<span class="mw-page-title-main">Chronic spontaneous urticaria</span> Medical condition

Chronic spontaneous urticaria(CSU) also known as Chronic idiopathic urticaria(CIU) is defined by the presence of wheals, angioedema, or both for more than six weeks. The most common symptoms of chronic spontaneous urticaria are angioedema and hives that are accompanied by itchiness.

<span class="mw-page-title-main">PLAID syndrome</span> Medical condition

PLAID syndrome is an inherited condition characterised by antibody deficiency and immune dysregulation, first described in 2012. The name is an acronym of "PLCG2-associated antibody deficiency and immune dysregulation". It is characterised by cold-induced urticaria, autoimmunity, atopy and humoral immune deficiency.

<span class="mw-page-title-main">Autoimmune urticaria</span> Autoimmune disease causing hives and itching

Autoimmune urticaria, also known as chronic autoimmune urticaria, is a type of chronic urticaria characterized by the presence of autoantibodies in the patient's immune system that target the body's own mast cells, leading to episodes of hives (urticaria). This immunologically distinct type of urticaria is considered autoimmune because the immune system, which normally protects the body from foreign organisms, mistakenly attacks the body's own cells, causing inflammation and other symptoms.

References

  1. ↑ Feinberg, Jeff H.; Toner, Charles B. (2008). "Successful Treatment of Disabling Cholinergic Urticaria". Military Medicine. 173 (2): 217–20. doi: 10.7205/MILMED.173.2.217 . PMID   18333501.
  2. ↑ Siebenhaar F, Weller K, : clinical picture and update on diagnosis and treatment (May 2007). "Acquired cold urticaria: Clinical picture and update on diagnosis and treatment". Clin. Exp. Dermatol. 32 (3): 241–5. doi:10.1111/j.1365-2230.2007.02376.x. PMID   17355280. S2CID   32159216.{{cite journal}}: CS1 maint: multiple names: authors list (link)
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  4. ↑ Tunca, Mehmet; Ozdogan, Huri (2005). "Molecular and Genetic Characteristics of Hereditary Autoinflammatory Diseases". Current Drug Targets. Inflammation and Allergy. 4 (1). Bentham Science Publishers: 77–80. doi: 10.2174/1568010053622957 . PMID   15720239.
  5. ↑ James, William D.; Berger, Timothy G.; Elston, Dirk M.; Grekin, Roy C.; Neuhaus, Isaac M.; Wei, Maria L. (2006). "Erythema and Urticaria". Andrews' Diseases of the Skin: Clinical Dermatology (10th ed.). Philadelphia, PA: Saunders, Elsevier. pp. 165–166. ISBN   0-7216-2921-0.
  6. ↑ Hoffman HM, Mueller JL, Broide DH, Wanderer AA, Kolodner RD (November 2001). "Mutation of a new gene encoding a putative pyrin-like protein causes familial cold autoinflammatory syndrome and Muckle-Wells syndrome". Nat. Genet. 29 (3): 301–5. doi:10.1038/ng756. PMC   4322000 . PMID   11687797.
  7. ↑ "Familial cold autoinflammatory syndrome". MedlinePlus. U.S. National Library of Medicine, National Institutes of Health . Retrieved 27 July 2021.
  8. ↑ CAPS, Community (2008-01-01). "Familial Cold Auto-inflammatory Syndrome (FCAS): Fact Sheet". Regeneron Pharmaceuticals. Archived from the original on 2016-03-04. Retrieved 2010-02-01.
  9. ↑ Tweedie, Susan; Braschi, Bryony; Gray, Kristian; Jones, Tamsin E. M.; Seal, Ruth L.; Yates, Bethan; Bruford, Elspeth A. "Symbol report for NLRP3". HGNC . Human Genome Organisation. 16400. Retrieved 27 July 2021.
  10. ↑ Smith, David W.; Johnson, Kenneth A.; Bingman, Craig A.; Aceti, David J.; Blommel, Paul G.; Wrobel, Russell L.; Frederick, Ronnie O.; Zhao, Qin; Sreenath, Hassan; Fox, Brian G.; Volkman, Brian F.; Jeon, Won Bae; Newman, Craig S.; Ulrich, Eldon L.; Hegeman, Adrian D.; Kimball, Todd; Thao, Sandy; Sussman, Michael R.; Markley, John L.; Phillips, George N. Jr. (2003). "1Q44". PDB . Research Collaboratory for Structural Bioinformatics. doi:10.2210/pdb1Q44/pdb . Retrieved 27 July 2021.
  11. ↑ Hoffman, Hal M.; Rosengren, Sanna; Boyle, David L.; Cho, Jae Y.; Nayar, Jyothi; Mueller, James L.; Anderson, Justin P.; Wanderer, Alan A.; Firestein, Gary S. (November 2004). "Prevention of cold-associated acute inflammation in familial cold autoinflammatory syndrome by interleukin-1 receptor antagonist". The Lancet . 364 (9447). Elsevier: 1779–1785. doi:10.1016/S0140-6736(04)17401-1. PMC   4321997 . PMID   15541451.
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  17. ↑ Yasmeen, Jabeen Bhat (2021-03-04). "Immunologically mediated skin disorders". In Sarkar, Rashmi (ed.). Concise Dermatology. CRC Press. ISBN   978-1-000-33966-6. Idiopathic cold urticaria may be immediate, delayed, localized, familial, or acquired.
  18. ↑ Soter Nicholas A.; Wasserman Stephen I.; Austen K. Frank (1976). "Cold Urticaria: Release into the Circulation of Histamine and Eosinophil Chemotactic Factor of Anaphylaxis during Cold Challenge". New England Journal of Medicine. 294 (13): 687–90. doi:10.1056/nejm197603252941302. PMID   55969.
  19. ↑ "NIH scientists find cause of rare immune disease: Genetic mutation leads to cold allergy, immune deficiency and autoimmunity". National Institute of Allergy and Infectious Diseases, National Institutes of Health. 11 January 2012.
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  21. 1 2 3 4 Abajian M.; Curto-Barredo L.; Krause K.; Santamaria E.; Izquierdo I.; Church M.; Maurer M.; Giménez-Arnau A. (2016). "Rupatadine 20 Mg and 40 Mg Are Effective in Reducing the Symptoms of Chronic Cold Urticaria" (PDF). Acta Dermato Venereologica Acta Derm Venerol. 96 (1): 56–59. doi: 10.2340/00015555-2150 . PMID   26038847.
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  23. ↑ Abajian, Marina; Curto Barredo, Laia; Krause, Karoline; Santamaria, Eva; Izquierdo, Iñaki; Church, Martin K.; Maurer, Marcus; Giménez Arnau, Ana (2016). "Rupatadine 20 mg and 40 mg are Effective in Reducing the Symptoms of Chronic Cold Urticaria". Acta Dermato Venereologica. 96 (1). Medical Journals Sweden: 56–59. doi: 10.2340/00015555-2150 . ISSN   0001-5555. PMID   26038847.
  24. ↑ Zuberbier T, Aberer W, Asero R, Bindslev-Jensen C, Brzoza Z, Canonica GW, et al. (2014). "The EAACI/GA(2) LEN/EDF/WAO Guideline for the definition, classification, diagnosis, and management of urticaria: the 2013 revision and update". Allergy. 69 (7): 868–887. doi:10.1111/all.12313. PMID   24785199.
  25. ↑ Mahmoudi, Massoud (May 2001). "Cold-induced urticaria". The Journal of the American Osteopathic Association . 101 (s5). American Osteopathic Association, De Gruyter: S1-4. doi: 10.7556/jaoa.2001.20027 (inactive 1 November 2024). PMID   11409259.{{cite journal}}: CS1 maint: DOI inactive as of November 2024 (link)
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  27. ↑ Brodská P, Schmid-Grendelmeier P (2012). "Treatment of severe cold contact urticaria with omalizumab: case reports". Case Rep Dermatol. 4 (3): 275–80. doi:10.1159/000346284. PMC   3551415 . PMID   23341807.
  28. ↑ Magerl M, Schmolke J, Siebenhaar F, Zuberbier T, Metz M, Maurer M (December 2007). "Acquired cold urticaria symptoms can be safely prevented by ebastine". Allergy. 62 (12): 1465–8. doi:10.1111/j.1398-9995.2007.01500.x. PMID   17900265. S2CID   12051897.
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