Pruritus aquagener L29.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Aquagene itching; Aquagene Pruritus; aquagenic pruritus; Bath time itch

History
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Shelley, 1970

Definition
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Intense, localized or even generalized itching (sometimes burning, stinging or pain = aquadynia), which occurs immediately (about 1/3 of the patients) or after a latency period of 2-15 min (about 2/3 of the patients) after contact with water, and subsides after about one hour. The entity is sometimes considered a special form of aquagenic urticaria.

Occurrence/Epidemiology
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Prevalence and incidence are unknown. Familial clustering occurs.

Etiopathogenesis
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Unknown; postulated is a "water-induced" activation of mast cells. The symptom is often described as an accompanying or premonitory symptom in systemic diseases.

The most frequent association is with polycythaemia vera, which is given as 40-50%. In these cases a mutation in the enzyme Januskinase 2(JAK2) occurs in a highly associated manner. The mutation leads to an increase in CD63+ eosinophilic and basophilic granulocytes, which are constitutively activated and induce the degranulation of mast cells in the skin.

Manifestation
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Middle to young age between 30 and 50 years. The duration of the disease is between a few months and 20 years.

Localization
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Preferably on buttocks and thighs (strictly limited to the points of contact with water); head, hands and feet are gutted.

Clinical features
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Severe symptoms, sometimes described as burning or pungent itching without visible skin changes, occurring regularly after contact with water, regardless of the temperature of the water. Myeloproliferative diseases can be detected in about 30% of patients. In addition, a lactose intolerance is often found. Other associated diseases are: essential thrombocythemia, hemochromatosis, infectious diseases such as hepatits C; neoplasia such as uterus carcinoma, acute lymphatic leukemia, T-cell lymphomas, hypereosinophilic syndrome, drugs (antimalarials, bupropion).

Therapy
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  • It is possible to try to develop tolerances by regular, increasing contact times with water.
  • The best results are achieved by a PUVA bath therapy. Alternatively: UVB irradiation, also 311 nm narrow spectrum UV therapy.
  • Local application of a 5% polidocanol cream(1-2 times/day) or capsaicin 0.01-1% as cream, shaking mixture or gel.
S.u. Urticaria, aquagene.

Internal therapy
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About 30% of patients react positively to non-sedating antihistamines such as desloratadine (Aerius) 1-2x2 Tbl.l/day p.o. or levocetirizine (Xusal) 1-2x2 Tbl.l/day p.o. Good and resilient positive effects can be achieved with UV therapy (UVB and PUVA). Other therapy options are anticonvulsants (e.g. pregabalin, naltrexone, paroxetine).

The best effects can be achieved after failure of antihistamine therapy with pregabalin (® 50-100 mg).

In the case of aquadynia, ibuprofen is recommended 30 minutes before water contact.

Literature
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  1. Cao T et al (2015) Idiopathic aquagenic pruritus: pathogenesis and effective treatment with atenolol. Dermatol Ther 28:118-121
  2. Goodkin R et al (2002) Repeated PUVA treatment of aquagenic pruritus. Clin Exp Dermatol 27: 164-165
  3. Heutkämper T et al (2010) Aquagener Pruritus: Associated underlying diseases and clinical pruritus characteristics. JDDG 10: 797-805
  4. Holme SA et al (2001) Aquagenic pruritus responding to intermittent photochemotherapy. Clin Exp Dermatol 26: 40-41
  5. Lubach D (1984) Aquagener Pruritus sine materia. dermatologist 34: 600-601
  6. McGrath JA et al (1990) Aquagenic pruritus and the myelodysplastic syndrome. Br J Dermatol 123: 414-415
  7. Newron JA et al (1990) Aquagenic pruritus associated with the idiopathic hypereosinophilic syndrome. Br J Dermatol 122: 103-106
  8. Nosbaum A et al (2011) Treatment with propranolol of 6 patients with idiopathic aquagenic pruritus. J Allergy Clin Immunol 128:1113
  9. Shelley W (1970) Post-wetness (aquagenic) pruritus. JAMA 212: 1385
  10. Siegel FP et al (2013) Aquagenic pruritus in polycythemia vera: characteristics and influence on quality of life in 441 patients. At J Hematol 88:665-669
  11. Treudler R et al (2002) Familial aquagenic urticaria associated with familial lactose intolerance. J Am Acad Dermatol 47:611-613

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020