Pruritus aquagenerL29.8

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.

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).

• 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.

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.

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11. Treudler R et al (2002) Familial aquagenic urticaria associated with familial lactose intolerance. J Am Acad Dermatol 47:611-613