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Genetic and Rare Diseases Information Center (GARD)

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Aquagenic pruritus


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Your Question

My 5-year-old daughter has just been told she has aquagenic pruritus. It is a very stressful time for us. Our main problem is getting her dressed in the morning, the change of clothes. We were told this is very rare for a child my daughter's age. Normally it is found in adults and not children. What can you tell us about this condition?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is aquagenic pruritus?

Aquagenic pruritus is a condition in which contact with water of any temperature causes intense itching without any visible skin changes.[1]  The episodes can begin anywhere from 1 to 15 minutes (or more) after contact with water and typically lasts 10 to 120 minutes (average: 40 minutes).[2] The cause of aquagenic pruritus is unknown; however, familial cases have been described.[1][2] Although the condition occurs more commonly in middle-aged and elderly males, aquagenic pruritus has also been observed in women and children. The symptoms of the condition are similar to those seen in patients with other conditions; therefore, a thorough evaluation should be performed to rule out other more serious conditions. Overall, treatment for aquagenic pruritus has been disappointing.[1]
Last updated: 11/10/2008

What symptoms are observed in patients who have aquagenic pruritus?

Aquagenic pruritus causes intense itching in the parts of the body that come in contact with water.  However, the head, palms, soles, and mucosa are not usually affected. No other systemic findings have been noted.[1]
Last updated: 11/12/2008

How is aquagenic pruritus diagnosed?

Criteria for diagnosis include [1]:

  • Severe itching, prickling, stinging, or burning that consistently develops after skin contact with water, regardless of water temperature or salinity;
  • Lack of visible skin manifestations;
  • Reaction within minutes of exposure and lasting anywhere between 10 minutes to 2 hours;
  • Lack of a other skin disease, internal condition, or medication to account for the reaction; and
  • Exclusion of all other physical urticarias, symptomatic dermographism, and polycythemia rubra vera.
Last updated: 11/12/2008

What causes aquagenic pruritus?

The exact cause of aquagenic pruritus is unknown, but increased mast cell degranulation (release of granules rich in histamine and other compounds into the body by mast cells, a special type of cell that plays a role in the immune system), increased circulating histamine, release of acetylcholine (a chemical in the body which sends signals from nerves to muscles and between nerves in the brain), and increased skin fibrinolytic activity (activity that controls clot size by promoting the breakdown of clots) have all been named as possible causes of the condition.[1]
Last updated: 11/12/2008

What underlying conditions and medications might cause the same symptoms as aquagenic pruritus?

The following list includes the names of some of the conditions and medications that cause symptoms similar to aquagenic pruritus:[3]

Last updated: 6/20/2011

What treatment has been attempted in patients who have aquagenic pruritus?

Treatment for aquagenic pruritus has been disappointing. Using water that is less acidic and applying petrolatum have had limited success. Antihistamines are rarely effective, despite the association with increased levels of histamine in the blood and skin. Several of forms of treatment (i.e., anticholinergics, aspirin, iron, serotonin antagonists, propanolol, recombinant interferon-alpha, and triamcinolone) are under investigation and have produced inconclusive results. Topical capsaicin has been reported beneficial. [1] Although UVB phototherapy is effective in some patients, PUVA is the preferred treatment. At least 50 percent of patients experience sustained improvement, although the treatment may have to be repeated every 6 or 12 months. In the cases of aquagenic pruritus associated with polycythemia vera, blood-drawing often relieves the pruritus in these cases. [1][4]
Last updated: 11/12/2008

References
  • Auerbach PS. Auerbach: Wilderness Medicine, 5th ed.. Philadelphia: Mosby, An Imprint of Elsevier; 2007;
  • Habif TP. Physical Urticarias. Habif: Clinical Dermatology, 4th ed. Ebinburgh: Mosby, Inc.; 2004;
  • Reich A, et al.. Drug-induced Pruritus: A Review. Acta Derm Venereol. 2009; 89:236-244. http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-0650&html=1. Accessed 6/20/2011.
  • Greaves MW. Chapter 41 - Pathophysiology and Clinical Aspects of Pruritus. In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, . Fitzpatrick's Dermatology in General Medicine. PA: PA; 2003;
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.