Urticaria aquagene L50.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 07.07.2022

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Synonym(s)

Aquagene Urticaria; Aquagenic urticaria; Urticaria aquagene; Water Contact Urticaria; Water Urticaria

History
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Rawnsley and Shelley, 1964

Definition
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Rare (about 100 cases have been documented so far, a significantly higher number can be assumed) subtype of physical urticaria, in which the appearance of pinhead-sized, reddened, follicular papules in the contact area after water contact is typical. Often occurs only after prolonged contact with water (within 2-30 minutes after contact), see also aquagenic pruritus.

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Etiopathogenesis
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Not clear. Formation of a histamine liberator by contact of water and sebum is discussed. To what extent an associated lactose intolerance is etiopathogenetically significant is still unclear.

An association between familial aquagenic urticaria and the Bernard-Soulier Syndrome (BSS) has been described sporadically. This is a rare autosomal recessive coagulation disorder characterized by a mutation of the GPIb-IX-V complex genes located on chromosomes 17p12, 22q11.2, 3q21, which code for a "platelet receptor" for von Willebrand factor (vWF) (Pitarch G et al. 2006; see below platelet). The connection is not clear.

Manifestation
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Mostly teenagers and young adults are affected.

Localization
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Preferably the upper body is affected, rarely the face and lower extremities. Never occurring on Palmae and Plantae (absence of sebaceous glands). Only skin areas that have had contact with water can develop skin changes.

Histology
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Superficial perivascular dermatitis with eosinophils, neutrophils, lymphocytes and some mast cells. Severe dermal edema.

Diagnosis
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Without contact with water the tests remain negative.

Test by placing wet, body temperature controlled cloths on the skin for 20 minutes. The occurrence of itchy hives is considered a positive test result.

Differential diagnosis
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Cholinergic urticaria; cold urticaria.

General therapy
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Prophylaxis is at the same time therapy, i.e. avoiding long-term water contact (e.g. bathing, rinsing). Alternative: The attempt of hardening by daily showering is possible, see also Pruritus, aquagener.

External therapy
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Intensive body care with highly greasing and refatting external substances such as linola fat, ashes base ointment or ungt. In cases of pronounced pruritus, mild glucocorticoid-containing ointments or fatty ointments, e.g. 0.25% prednicarbate (e.g. Dermatop ointment/fatty ointment), 0.1% methylprednisolone (e.g. Advantan ointment), are permitted for a short time.

Radiation therapy
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Attempts to combine PUVA therapy and water bath treatments are described.

Internal therapy
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Poor response to antihistamines. If necessary, try levocetirizine (e.g. Xusal) 1-2 tbl/day or desloratadine (e.g. Aerius) 1-2 tbl/day. Therapy trials with H2-blockers, acetylsalicylic acid, cholestyramine and iron substitution are described.

Literature
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Bayle P et al (2003) Localized aquagenic urticaria: efficacy of a barrier cream. Contact dermatitis 49: 160-161
Hedge E (1981) Water contact urticaria. dermatologist 32: 532-534
Parker RK et al (1992) Aquagenic urticaria. Cutis 50: 283-284
Pitarch G et al (2006) Familial aquagenic urticaria and bernard-soulier syndrome. Dermatology 212:96-97.
Savoia A et al (2014) Spectrum of the mutations in Bernard-Soulier syndrome. Hum mutate 35:1033-1045
Shelley WB, Rawnsley HM (1964) Aquagenic urticaria. Contact sensitivity reaction to water. JAMA 189: 895-898
Treudler R et al (2002) Familial aquagenic urticaria associated with familial lactose intolerance. J Am Acad Dermatol 47: 611-613
Wang F et al (2017) Aquagenic cutaneous disorders. J Dtsch Dermatol Ges 15:602-608.