Aquagenic urticaria

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Aquagenic urticaria
Other nameswater itch, water allergy
Specialty Dermatology   OOjs UI icon edit-ltr-progressive.svg
Symptoms Hives after skin contact with water
Usual onsetImmediate
Duration30 to 60 minutes
CausesUnclear (triggered by water)
Diagnostic method Based on symptoms, confirmed by "water challenge" test
TreatmentMedications, phototherapy, barrier cream
Medication Antihistamines, propranolol, steroids

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. [1] The condition typically results from contact with water of any type, temperature or additive.

Contents

Signs and symptoms

The main symptom of aquagenic urticaria is the development of physical hives, which may or may not itch. Itching after contact with water, without the development of physical hives, is known as aquagenic pruritus. Aquadynia is a condition in which pain occurs after contact with water. [2]

In severe cases, drinking water can result in swelling of the oral cavity, [3] swelling of the throat, [4] and in extreme cases, anaphylaxis. [5] [6] [7]

The hives associated with aquagenic urticaria are typically small (approximately 1–3 mm), red- or skin-colored welts (called wheals) with clearly defined edges. It most commonly develops on the neck, upper trunk and arms, although it can occur anywhere on the body. Once the water source is removed, the rash generally fades within 30 to 60 minutes. [8]

Water in all forms, such as tap or sea water, swimming pool water, sweat, tears, and saliva can induce the lesions. [9] [10] [11]

Cause

The cause of aquagenic urticaria is not fully understood; however, several mechanisms have been proposed. [12] Interaction between water and a component in or on the skin or sebum has been suggested. This theory suggests that a substance is formed by this interaction, the absorption of which causes perifollicular mast cell degranulation with release of histamine. [13] [14] Another proposed theory is of a water-soluble allergen in the epithelial tissues. Water dissolves the allergen, causing it to diffuse into the tissues, causing histamine release from sensitized mast cells. [15] [16]

Diagnosis

Diagnosis of aquagenic urticaria begins with a clinical history and water challenge test. [17] The water challenge test consists of application of a 35°C water compress to the upper body for 30 minutes. Water of any temperature can provoke aquagenic urticaria; however, keeping the compress at a similar temperature to that of the human body (37 °C) avoids confusion with cold urticaria or cholinergic urticaria. In addition, a forearm or hand can be immersed in water of varying temperatures to determine whether temperature is a factor in the patient's condition. Aquagenic urticaria differs from aquagenic pruritus, in which contact with water evokes intense itching without visible hives or rash. [18] [19] [20]

Once known as a separate, rare disease, aquagenic urticaria is now considered a subtype of general urticaria. [21] The first case was reported by Walter B Shelley et al. in 1964. [22] The condition is more common in women than men, and typically presents for the first time during puberty. Genetics may play a part, and the condition may be related to other sensitivities such as lactose intolerance. [23] [24] [25] [26] [27] [13]

Prevention

Desensitization does not seem to work for aquagenic urticaria; a patient will continue to react to water no matter how gradually or frequently it is introduced. [28] Topical application of antihistamines like 1% diphenhydramine before water exposure is reported to reduce the hives. [29] Oil in water emulsion creams, or petrolatum, applied as barrier agents prior to a shower or bath may control symptoms. [30]

Treatment

Given the rarity of the disease, clinical efficacy data of treatments is limited. However, current recommendations suggest a trial of a second generation anti-histamine up to four-times the standard dosing scheme for initial pharmacology. For patients who cannot be controlled on high dose anti-histamine, other therapeutics, including biologics like Omalizumab, have shown improvements in case reports.

See also

Related Research Articles

H1 antagonists, also called H1 blockers, are a class of medications that block the action of histamine at the H1 receptor, helping to relieve allergic reactions. Agents where the main therapeutic effect is mediated by negative modulation of histamine receptors are termed antihistamines; other agents may have antihistaminergic action but are not true antihistamines.

<span class="mw-page-title-main">Latex allergy</span> Medical condition

Latex allergy is a medical term encompassing a range of allergic reactions to the proteins present in natural rubber latex. It generally develops after repeated exposure to products containing natural rubber latex. When latex-containing medical devices or supplies come in contact with mucous membranes, the membranes may absorb latex proteins. In some susceptible people, the immune system produces antibodies that react immunologically with these antigenic proteins. Many items contain or are made from natural rubber, including shoe soles, pen grips, hot water bottles, elastic bands, rubber gloves, condoms, baby-bottle nipples, and balloons; consequently, there are many possible routes of exposure that may trigger a reaction. People with latex allergies may also have or develop allergic reactions to some fruits, such as bananas.

<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">Cold urticaria</span> Allergic reaction to low temperatures

Cold urticaria is a disorder in which large red welts called hives (urticaria) form on the skin after exposure to a cold stimulus. 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.

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">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.

<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.

<span class="mw-page-title-main">Notalgia paresthetica</span> Neuropathy causing itching between the shoulder blades

Notalgia paresthetica or Notalgia paraesthetica (NP) (also known as "Hereditary localized pruritus", "Posterior pigmented pruritic patch", and "subscapular pruritus") is a chronic sensory neuropathy. Notalgia paresthetica is a common localized itch, affecting mainly the area between the shoulder blades (especially the T2–T6 dermatomes) but occasionally with a more widespread distribution, involving the shoulders, back, and upper chest. The characteristic symptom is pruritus (itch or sensation that makes a person want to scratch) on the back, usually on the left hand side below the shoulder blade (mid to upper back). It is occasionally accompanied by pain, paresthesia (pins and needles), or hyperesthesia (unusual or pathologically increased sensitivity of the skin to sensory stimuli, such as pain, heat, cold, or touch), which results in a well circumscribed hyperpigmentation of a skin patch in the affected area.

Autoimmune progesterone dermatitis(APD) occurs during the luteal phase of a woman's menstrual cycle and is an uncommon cyclic premenstrual reaction to progesterone. It can present itself in several ways, including eczema, erythema multiforme, urticaria, angioedema, and progesterone-induced anaphylaxis. The first case of autoimmune progesterone dermatitis was identified in 1964. Reproductive function may be impacted by APD.

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.

Adrenergic urticaria is a skin condition characterized by an eruption consisting of small (1-5mm) red macules and papules with a pale halo, appearing within 10 to 15 min after emotional upset. There have been 10 cases described in medical literature, and involve a trigger followed by a rise in catecholamine and IgE. Treatment involves propranolol and trigger avoidance.

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

Pressure urticaria or delayed pressure urticaria is a physical urticaria caused by pressure applied to the skin, and is characterized by the development of swelling and pain that usually occurs 3 to 12 hours after local pressure has been applied.

<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.

Mast cell activation syndrome (MCAS) is a term referring to one of two types of mast cell activation disorder (MCAD); the other type is idiopathic MCAD. MCAS is an immunological condition in which mast cells, a type of white blood cell, inappropriately and excessively release chemical mediators, such as histamine, resulting in a range of chronic symptoms, sometimes including anaphylaxis or near-anaphylaxis attacks. Primary symptoms include cardiovascular, dermatological, gastrointestinal, neurological, and respiratory problems.

A sweat allergy is the exacerbation of atopic dermatitis associated with an elevated body temperature and resulting increases in the production of sweat. It appears as small reddish welts that become visible in response to increased temperature and resulting production of sweat. It can affect all ages. Sweating can trigger intense itching or cholinergic urticaria. The protein MGL_1304 secreted by mycobiota (fungi) present on the skin such as Malassezia globosa acts as a histamine or antigen. People can be desensitized using their own samples of sweat that have been purified that contains small amounts of the allergen. The allergy is not due to the sweat itself but instead to an allergy-producing protein secreted by microorganisms found on the skin.

Exercise-induced anaphylaxis is a rare condition in which anaphylaxis, a serious or life-threatening allergic response, is brought on by physical activity. Approximately 5–15% of all reported cases of anaphylaxis are thought to be exercise-induced.

<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. ↑ NIH. "Aquagenic urticaria". Genetic and Rare Diseases Information Center. Retrieved January 5, 2025.
  2. ↑ Shelley WB, Shelley E (February 1998). "Aquadynia: Noradrenergic pain induced by bathing and responsive to clonidine". Journal of the American Academy of Dermatology. 38 (2): 357–358. doi:10.1016/s0190-9622(98)70583-3. ISSN   0190-9622. PMID   9486716.
  3. ↑ Seol JE, Kim DH, Park SH, Kang JN, Sung HS, Kim H (June 2017). "Aquagenic Urticaria Diagnosed by the Water Provocation Test and the Results of Histopathologic Examination". Annals of Dermatology. 29 (3): 341–345. doi:10.5021/ad.2017.29.3.341. PMC   5438944 . PMID   28566914.
  4. ↑ "The woman who is allergic to water".
  5. ↑ "JUST ONE CUP OF WATER COULD KILL LITTLE HEIDI; Girl's deadly allergy means she cannot weep, wash, drink, play or even stand in the rain. - Free Online Library".
  6. ↑ "Girl allergic to water". December 14, 2018.
  7. ↑ "Here's What Happens when You Develop an Allergy to Water". December 15, 2015.
  8. ↑ Park H, Kim HS, Yoo DS, Kim JW, Kim CW, Kim SS, Hwang JI, Lee JY, Choi YJ (2011). "Aquagenic urticaria: A report of two cases". Annals of Dermatology. 23 (Suppl 3): S371–4. doi:10.5021/ad.2011.23.S3.S371. PMC   3276800 . PMID   22346281.
  9. ↑ Sibbald RG, Black AK, Eady RA, James M, Greaves MW (1981). "Aquagenic urticaria: Evidence of cholinergic and histaminergic basis". The British Journal of Dermatology. 105 (3): 297–302. doi:10.1111/j.1365-2133.1981.tb01289.x. PMID   7272209. S2CID   37931708.
  10. ↑ Harwood CA, Kobza-Black A (1992). "Aquagenic urticaria masquerading as occupational penicillin allergy". The British Journal of Dermatology. 127 (5): 547–8. doi:10.1111/j.1365-2133.1992.tb14862.x. PMID   1467303. S2CID   27755086.
  11. ↑ Martínez-Escribano JA, Quecedo E, de la Cuadra J, Frías J, Sánchez-Pedreño P, Aliaga A (1997). "Treatment of aquagenic urticaria with PUVA and astemizole". Journal of the American Academy of Dermatology. 36 (1): 118–9. doi:10.1016/s0190-9622(97)70344-x. PMID   8996279.
  12. ↑ Lee HG, Lee AY, Lee YS (1990). "A case of aquagenic urticaria". Korean J Dermatol. 28: 456–458.
  13. 1 2 Shelley WB, Rawnsley HM (1964). "Aquagenic Urticaria. Contact Sensitivity Reaction to Water". JAMA. 189: 895–8. doi:10.1001/jama.1964.03070120017003. PMID   14172902.
  14. ↑ Rothbaum R, McGee JS (2016). "Aquagenic urticaria: diagnostic and management challenges". Journal of Asthma and Allergy. 9: 209–213. doi: 10.2147/JAA.S91505 . ISSN   1178-6965. PMC   5136360 . PMID   27942227.
  15. ↑ Rothbaum R, McGee JS (November 29, 2016). "Aquagenic urticaria: Diagnostic and management challenges". Journal of Asthma and Allergy. 9: 209–213. doi: 10.2147/JAA.S91505 . PMC   5136360 . PMID   27942227.
  16. ↑ Barbaud A (May 2003). "[Physical urticarias]". Annales de Dermatologie et de Venereologie. 130: 1S16–27. PMID   12843805.
  17. ↑ Dice JP (2004). "Physical urticaria". Immunology and Allergy Clinics of North America. 24 (2): 225–46, vi. doi:10.1016/j.iac.2004.01.005. PMID   15120149.
  18. ↑ Panconesi E, Lotti T (1987). "Aquagenic urticaria". Clinics in Dermatology. 5 (3): 49–51. doi:10.1016/s0738-081x(87)80008-1. PMID   3664423.
  19. ↑ Rothbaum R, McGee JS (November 29, 2016). "Aquagenic urticaria: diagnostic and management challenges". Journal of Asthma and Allergy. 9: 209–213. doi: 10.2147/JAA.S91505 . PMC   5136360 . PMID   27942227.
  20. ↑ Casale TB, Olsen JA, DelasAlas HC (May 2013). "Aquagenic Urticaria". The Journal of Allergy and Clinical Immunology: In Practice. 1 (3): 295–296. doi:10.1016/j.jaip.2013.02.003. ISSN   2213-2198. PMID   24565488.
  21. ↑ Beaven MA (2009). "Our perception of the mast cell from Paul Ehrlich to now". European Journal of Immunology. 39 (1): 11–25. doi:10.1002/eji.200838899. PMC   2950100 . PMID   19130582.
  22. ↑ Zuberbier T, Asero R, Bindslev-Jensen C, Walter Canonica G, Church MK, Giménez-Arnau A, Grattan CE, Kapp A, Merk HF, Rogala B, Saini S, Sánchez-Borges M, Schmid-Grendelmeier P, Schünemann H, Staubach P, Vena GA, Wedi B, Maurer M, Dermatology Section of the European Academy of Allergology Clinical Immunology., Global Allergy Asthma European Network. (2009). "EAACI/GA(2)LEN/EDF/WAO guideline: Definition, classification and diagnosis of urticaria". Allergy. 64 (10): 1417–26. doi:10.1111/j.1398-9995.2009.02179.x. PMID   19772512. S2CID   21174848.
  23. ↑ Yavuz ST, Sahiner UM, Tuncer A, Sackesen C (2010). "Aquagenic urticaria in 2 adolescents". Journal of Investigational Allergology & Clinical Immunology. 20 (7): 624–5. PMID   21314009.
  24. ↑ Pitarch G, Torrijos A, Martínez-Menchón T, Sánchez-Carazo JL, Fortea JM (2006). "Familial aquagenic urticaria and bernard-soulier syndrome". Dermatology. 212 (1): 96–7. doi:10.1159/000089035. PMID   16319487. S2CID   43153639.
  25. ↑ Baptist AP, Baldwin JL (2005). "Aquagenic urticaria with extracutaneous manifestations". Allergy and Asthma Proceedings. 26 (3): 217–20. PMID   16119038.
  26. ↑ Park H, Kim HS, Yoo DS, Kim JW, Kim CW, Kim SS, Hwang JI, Lee JY, Choi YJ (2011). "Aquagenic urticaria: A report of two cases". Annals of Dermatology. 23 (Suppl 3): S371–4. doi:10.5021/ad.2011.23.S3.S371. PMC   3276800 . PMID   22346281.
  27. ↑ Treudler R, Tebbe B, Steinhoff M, Orfanos CE (2002). "Familial aquagenic urticaria associated with familial lactose intolerance". Journal of the American Academy of Dermatology. 47 (4): 611–3. doi:10.1067/mjd.2002.124599. PMID   12271310.
  28. ↑ Frances AM, Fiorenza G, Frances RJ (2004). "Aquagenic urticaria: Report of a case". Allergy and Asthma Proceedings. 25 (3): 195–7. PMID   15317326.
  29. ↑ Hide M, Yamamura Y, Sanada S, Yamamoto S (2000). "Aquagenic urticaria: A case report". Acta Dermato-Venereologica. 80 (2): 148–9. doi: 10.2340/0001555580150 . PMID   10877142. S2CID   29448803.
  30. ↑ Wong E, Eftekhari N, Greaves MW, Ward AM (1987). "Beneficial effects of danazol on symptoms and laboratory changes in cholinergic urticaria". The British Journal of Dermatology. 116 (4): 553–6. doi:10.1111/j.1365-2133.1987.tb05877.x. PMID   3555598. S2CID   46181271.
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