Familial Aquagenic Urticaria and Bernard-Soulier Syndrome

  title={Familial Aquagenic Urticaria and Bernard-Soulier Syndrome},
  author={Gerard Pitarch and Arantxa Torrijos and Teresa Mart{\'i}nez-Mench{\'o}n and Jose S{\'a}nchez-Carazo and Jos{\'e} Miguel Fortea},
  pages={96 - 97}
Department of Dermatology, Valencia University General Hospital, Valencia , Spain of age and subsequently gradually worsened. The two younger siblings had Bernard-Soulier syndrome. A challenge test could be performed in the 26-year-old sibling, proving positive for tap water at room temperature, and for hot and cold water – though the lesions were less numerous than in the case of the older patient. A challenge test could not be performed with the 24-year-old sibling. The mother of the 3 women… 

Aquagenic Urticaria: A Report of Two Cases

Treatment of the 19-year-old man with 180 mg fexofenadine daily was successful to prevent the wheals and erythema, and treatment with 5 ml ketotifen syrup bid per day resulted in improvement of symptoms in the 4- year-old boy.

Localized salt-dependent aquagenic urticaria, a rare subtype of urticaria: a case report.

The case of a 25-year-old atopic woman with a 4-year history of recurrent urticarial rashes is described, which supports the existence of a distinct salt-dependent subtype of aquagenic urticaria (SDAU), which seems to be triggered mostly by sea bathing, affects young women and has a characteristic localization on the inferior facial contours and neck.

[Familial aquagenic urticaria: report of two cases and literature review].

The first case of familial aquagenic urticaria in Brazil (mother and daughter) is presented, with both patients presented wheals following contact with water, especially when showering, regardless of its temperature.

Aquagenic urticaria: diagnostic and management challenges

A therapeutic ladder for the treatment of AU is described, including an oral administration of nonsedating, second-generation H1 antihistamines, but many patients may require further interventions to have adequate symptomatic control.

Aquagenic Urticaria: A Review of Literature and Case Reports

The available theranostics are summarized to provide guidance for the management of urticaria, the common malady worldwide, but such vulnerability to water is a rare and a distressing phenomenon requiring considerable lifestyle modifications.

Aquagenic urticaria in twins

To the knowledge, this is the first report of aquagenic urticaria in monozygotic twins, further supporting a genetic component to this disease.

The Role of Platelets in Chronic Urticaria

Overall, in CU patients, conflicting results have been obtained from the assessment of platelet indices, such as mean platelet volume, platelet count and distribution width, as well as markers of platelets aggregation and activation, which suggest a link between inflammation, coagulation and histamine release in the pathophysiological network of CU.

The definition, diagnostic testing, and management of chronic inducible urticarias – The EAACI/GA2LEN/EDF/UNEV consensus recommendations 2016 update and revision

These recommendations for the definition, diagnosis and management of chronic inducible urticaria (CIndU) extend, revise and update the previous consensus report on physical urticarias and cholinergic uricaria (Allergy, 2009), and acknowledge the latest changes in understanding of CIndU.

Aquatic Skin Diseases from Chemical and Physical Causes

An occupational chronic traumatic scleroedema of the hands, of various etiologies, is frequently observed in fishermen; the complaint can persist for many years after abandonment of the work activity.



Familial aquagenic urticaria associated with familial lactose intolerance.

The familial occurrence of aquagenic urticaria over 3 generations is reported here in association with familial lactose intolerance, a condition in which the enzyme lactase encoded on chromosome 2, is deficient.

Familial polymorphous light eruption with aquagenic urticaria: successful treatment with PUVA.

The skin symptoms in the authors' patient were initially urticarial and later mainly papular and vesicular and also appeared after 400 and 500 nm and window glass offered little protection.

[Bernard -Soulier syndrome].

People with Bernard-Soulier syndrome tend to bruise easily and have an increased risk of nosebleeds (epistaxis).

Familial aquagenic urticaria.

2 cases with the same eruption due to water are reported. This occurs in the same family. 1 case presents a positive patch test with water, and responds well to antihistamines.

Aquagenic urticaria.

Aquagenic urticaria: report of a case and review of the literature.

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