Aquagenic urticaria

Aquagenic urticaria, also known as water allergy and water urticaria, is a rare 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.

Aquagenic urticaria
Other nameswater itch, water allergy
SpecialtyDermatology Edit this on Wikidata
SymptomsHives after skin contact with water
Usual onsetImmediate
Duration30 to 60 minutes
CausesUnclear (triggered by water)
Diagnostic methodBased on symptoms, confirmed by "water challenge" test
TreatmentMedications, phototherapy, barrier cream
MedicationAntihistamines, propranolol, steroids

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.

In severe cases, drinking water can result in swelling of the oral cavity,[2] swelling of the throat,[3] and in extreme cases, anaphylaxis.[4][5][6]

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.[7]

Water in all forms, such as tap or sea water, swimming pool water, sweat, tears, and saliva can induce the lesions.[8][9][10]

Cause

The cause of aquagenic urticaria is not fully understood; however, several mechanisms have been proposed.[11] 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.[12][13] 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.[14][15]

Diagnosis

Diagnosis of aquagenic urticaria begins with a clinical history and water challenge test.[16] 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.[17]

Once known as a separate, rare disease, aquagenic urticaria is now considered a subtype of general urticaria.[18] The first case was reported by Walter B Shelley et al. in 1964.[19] 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.[20][21][22][23][24][12]

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.[25] Topical application of antihistamines like 1% diphenhydramine before water exposure is reported to reduce the hives.[26] Oil in water emulsion creams, or petrolatum, applied as barrier agents prior to a shower or bath may control symptoms.[27] Therapeutic effectiveness of various classes of drugs differs from case to case.

Treatment

There is currently no treatment that will permanently cure the condition. Avoidance of water is recommended as a first line of defense, and most treatments are palliative in nature rather than curative:

See also

References

  1. NIH. "Aquagenic urticaria". Retrieved 1 March 2020.
  2. Seol, Jung Eun; Kim, Do Hyeong; Park, So Hee; Kang, Jeong Nan; Sung, Ho Suk; Kim, Hyojin (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.
  3. "The woman who is allergic to water".
  4. "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".
  5. "Girl allergic to water". 14 December 2018.
  6. "Here's What Happens when You Develop an Allergy to Water". 15 December 2015.
  7. Park, H; Kim, H. S.; Yoo, D. S.; Kim, J. W.; Kim, C. W.; Kim, S. S.; Hwang, J. I.; Lee, J. Y.; Choi, Y. J. (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.
  8. Sibbald, R. G.; Black, A. K.; Eady, R. A.; James, M; Greaves, M. W. (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.
  9. Harwood, C. A.; 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.
  10. Martínez-Escribano, J. A.; 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.
  11. Lee HG, Lee AY, Lee YS (1990). "A case of aquagenic urticaria". Korean J Dermatol. 28: 456–458.
  12. Shelley, W. B.; Rawnsley, H. M. (1964). "Aquagenic Urticaria. Contact Sensitivity Reaction to Water". JAMA. 189: 895–8. doi:10.1001/jama.1964.03070120017003. PMID 14172902.
  13. Rothbaum, Robert; McGee, Jean S. (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.
  14. Rothbaum, Robert; McGee, Jean S. (29 November 2016). "Aquagenic urticaria: Diagnostic and management challenges". Journal of Asthma and Allergy. 9: 209–213. doi:10.2147/JAA.S91505. PMC 5136360. PMID 27942227.
  15. Barbaud, A. (May 2003). "[Physical urticarias]". Annales de Dermatologie et de Venereologie. 130 Spec No 1: 1S16–27. PMID 12843805.
  16. Dice, J. P. (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.
  17. Panconesi, E; Lotti, T (1987). "Aquagenic urticaria". Clinics in Dermatology. 5 (3): 49–51. doi:10.1016/s0738-081x(87)80008-1. PMID 3664423.
  18. Beaven, M. A. (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.
  19. Zuberbier, T; Asero, R; Bindslev-Jensen, C; Walter Canonica, G; Church, M. K.; Giménez-Arnau, A; Grattan, C. E.; Kapp, A; Merk, H. F.; Rogala, B; Saini, S; Sánchez-Borges, M; Schmid-Grendelmeier, P; Schünemann, H; Staubach, P; Vena, G. A.; 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.
  20. Yavuz, S. T.; Sahiner, U. M.; Tuncer, A; Sackesen, C (2010). "Aquagenic urticaria in 2 adolescents". Journal of Investigational Allergology & Clinical Immunology. 20 (7): 624–5. PMID 21314009.
  21. Pitarch, G; Torrijos, A; Martínez-Menchón, T; Sánchez-Carazo, J. L.; Fortea, J. M. (2006). "Familial aquagenic urticaria and bernard-soulier syndrome". Dermatology. 212 (1): 96–7. doi:10.1159/000089035. PMID 16319487. S2CID 43153639.
  22. Baptist, A. P.; Baldwin, J. L. (2005). "Aquagenic urticaria with extracutaneous manifestations". Allergy and Asthma Proceedings. 26 (3): 217–20. PMID 16119038.
  23. Park, H; Kim, H. S.; Yoo, D. S.; Kim, J. W.; Kim, C. W.; Kim, S. S.; Hwang, J. I.; Lee, J. Y.; Choi, Y. J. (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.
  24. Treudler, R; Tebbe, B; Steinhoff, M; Orfanos, C. E. (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.
  25. Frances, A. M.; Fiorenza, G; Frances, R. J. (2004). "Aquagenic urticaria: Report of a case". Allergy and Asthma Proceedings. 25 (3): 195–7. PMID 15317326.
  26. 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.
  27. Wong, E; Eftekhari, N; Greaves, M. W.; Ward, A. M. (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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