On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.84 no.5 Rio de Janeiro Sept./Oct. 2009
Maria Bandeira de Melo Paiva SeizeI; Mayra IanhezII; Patrícia Karla de SouzaIII; Osmar RottaIV; Silmara da Costa Pereira CestariV
IDermatologist certified by the SBD,
specialization course student in Pediatric Dermatology at the UNIFESP-EPM, masters'degree
student in Dermatology at the UNIFESP Sao Paulo (SP), Brazil
IIDermatologist certified by the SBD, specialization course student in Pediatric Dermatology at the UNIFESP-EPM, master's degree student in Dermatology at the UNIFESP Sao Paulo (SP), Brazil
IIIDermatologist certified by the SBD, masters'degree in Dermatology from the UNIFESP, collaborating physician of the Department of Dermatology - UNIFESP, in charge of the Outpatient's Clinic of Dermatology/Urticaria at the UNIFESP Sao Paulo (SP), Brazil
IVDermatologist certified by the SBD, masters'degree in Dermatology from the Faculdade de Medicina da Universidade de São Paulo, PhD in Dermatology from the UNIFESP-EPM, associate professor, Department of Dermatology - UNIFESP, extension course coordinator, deputy supervisor of residency and head of the Department of Dermatology, UNIFESP Sao Paulo (SP), Brazil
VEspecialista em dermatologia pela SBD, mestre e doutora em dermatologia pela Universidade Federal de São Paulo-Escola Paulista de Medicina (UNIFESP-EPM), professora adjunta do Departamento de Dermatologia da Unifesp São Paulo (SP), Brasil
Aquagenic urticaria is a rare form of physical urticaria, characterized by pruritic wheals that appear following contact with water, independently of its temperature. There are few reports of cases of aquagenic urticaria, and only five include the familial form. We present the first case of familial aquagenic urticaria in Brazil (mother and daughter). Both patients presented wheals following contact with water, especially when showering, regardless of its temperature. The mother reported onset of urticaria four years before and the daughter presented wheals since birth. For diagnostic purposes, they were submitted to a challenge test with water, and both subjects presented wheals, as well as to tests using ice cubes in plastic bag with dermographometer and challenge tests for cholinergic urticaria, with no appearance of lesions, excluding other forms of physical urticaria.
Keywords: Histamine H1 antagonists; Pruritus; Urticaria; Water
Aquagenic urticaria was first described in 1964 by Shelley and Rawnsley.1 It is a rare form of physical urticaria characterized by perifollicular urticaria-like lesions following contact with water at any temperature. The lesions measure 2-3 mm (diameter), are located mostly on the thorax and upper portions of the limbs, and may last from 10 to 50 minutes.1-2
This condition affects women more often than men, and generally starts during puberty.1 It occurs rarely in children;3-7family cases are also uncommon, and most patients are isolated cases.2-4
Aquagenic urticaria may be associated with other types of physical urticaria, such as cholinergic or cold urticaria, and dermographism. 1,3 To make a correct diagnosis of aquagenic urticaria it is essential to exclude other forms of physical urticaria; the water challenge test should cause urticaria-like lesions.8
We present two cases of family aquagenic urticaria in mother and daughter. These appear to be the first cases of family aquagenic urticaria published in Brazil; only a single case aquagenic urticaria in a Brazilian adult patient with no family history was published before.9 The younger of these two cases is one of few cases of aquagenic urticaria starting before puberty;2,3,5,6 it is probably the first case in which the onset of this condition is soon after birth.
CASE 1 A 28-year old female white patient reported urticaria starting about ten minutes after contact with water, for the past four years; this occurred mainly after a shower with water at any temperature. Lesions are generally spots on the neck and thorax, lasting from five to 20 minutes. There were no lesions in the extremities. Contact with higher or lower temperatures without water did not cause the lesions. There was no personal history of atopy. Her father, brother and sister had a similar clinical response to contact with water. The patient had not used antihistaminic drugs or other medication previously. No vertigo, palpitations or headaches occurred during the crises. The physical examination did not reveal any lesions.
Challenge tests on the dorsum yielded the following results:
Dermographism (with a dermographometer = 4.0 X 10 5 Pa) negative.
Cold (applying an ice cube in plastic during 20 minutes) negative.
Cholinergic urticaria test (warmth and physical exercise) negative.
Aquagenic challenge test (applying a water-drenched compress at bodily temperature during 20 minutes) positive, showing urticaria-like lesions (Figure 1).
Following these tests, a diagnosis of aquagenic urticaria was made; hydroxyzine was the recommended therapy, which the patient refused. CASE 2 A white female aged one year and seven months, a daughter of the patient described as Case 1, manifested urticaria since birth, which appeared about ten minutes after contact with water of any temperature. Lesions arose mainly after showers or baths; similar to Case 1, the most frequently affected regions were the thorax and neck. Limbs were not involved; lesions lasted from five to 20 minutes. The grandfather, an uncle and an aunt on the mothers side had similar lesions. No lesions were seen upon physical examination.
The same challenge tests performed in Case 1 were carried out in this patient; the child was also immersed in a warm bath. The water challenge test was positive, with urticaria-like lesions on the thorax after the test. Tests with the dermatographometer, with ice cubes and cholinergic urticaria challenge were negative.
The proposed therapy was hydroxyzine, which the mother refused based on the argument that the medication would not cure the condition.
Aquagenic urticaria manifests as urticaria-like lesions arising a few minutes after contact with water at any temperature.1 These lesions last about 30 minutes and for the most part have no associated manifestations. Some cases, however, may present dermographism and other extra-cutaneous signs, such as headaches, bronchospasm and angioedema.5,10
In both cases, clinical findings are similar among published cases of aquagenic urticaria.10
This type of urticaria generally presents as an isolated form. The family form, as described in the cases above, is rare. Based on our survey on the Pubmed, Lilacs, Medline, Scielo and Cochrane Library databases, we found only 30 cases of aquagenic urticaria published, of which fewer than ten are familial.10 The first family cases was reported in 1967 by Tromovitch, who described the condition in two siblings, a female child in which the onset was at age nine years, and a male child.2 Most patients in family-related cases are female,3,4,10 as were the cases above.
Our patients appear to be the first Brazilian cases of family aquagenic urticaria published; our survey of the databases mentioned above found a single case published by Medeiros, in 1996, of an adult patient with no family association.9
Aquagenic urticaria generally starts in puberty; this condition rarely starts in childhood, and there are few such cases in the literature. 2,4-7,10 According to the literature, both sexes are involved equally in the infantile form of aquagenic urticaria. There are three cases published of the family form in children, most of them in females as in Case 2 above. This patient is one of the youngest we found with this condition in the literature, and appears to be the first published case of aquagenic urticaria in Brazil.
The pathogenesis of this form of urticaria has not been fully clarified; many mechanisms have been proposed. In 1964, Shelley and Rawnsley suggested that water interacted with components of the corneal strata or sebaceous glands to form a toxic substance that would be absorbed by the skin, resulting in degranulation of perifollicular mast cells and histamine release, which would result in urticarial lesions.1 Czarnetzki et al.11 suggested that there was a water-soluble antigen on the epidermis that, in contact with water, would diffuse throughout the dermis, causing mast cells to release histamine, which appears to be the chemical mediator in the pathogenesis of aquagenic urticaria. Other mediators, such as acetylcholine, serotonin, and bradikynin may also be involved in the pathogenesis of aquagenic urticaria. 5,9,12
Two criteria are important for a correct diagnosis of this disease:7 excluding other causes of physical urticaria, and a positive water challenge test. This test is done by applying a water-drenched compress at 37 ºC to skin, preferably on the thorax, for 20 minutes. In patients with aquagenic urticaria, the test is positive when urticariform lesions appear, confirming the diagnosis.2,11,13
The differential diagnosis should be made with cholinergic urticaria and cold urticaria.13 Lesions form in cholinergic urticaria when bodily temperature increases.2 Lesions form in cold urticaria when the patient is exposed to low temperatures.1,2 The challenge test with ice is negative in aquagenic urticaria, which excludes cold urticaria. Lesions do not arise only with increased temperatures, as occurs in cholinergic urticaria.
The patients described above form urticarial lesions after having contact with water; the challenge tests were negative with ice, the dermographometer, and with cholinergic stimulation, all of which suggest the possibility of family aquagenic urticaria (mother and daughter). A diagnosis of aquagenic urticaria is not made as often as it should, possibly because of similarities with other forms of chronic urticaria. The clinical history and carefully undertaken skin challenge tests are important to make correct diagnosis.
The current medications of choice to treat aquagenic urticaria are antihistamine drugs, although some cases may not respond. Anticholinergic drugs, such as scopolamine, may also be used.14 Emollients cannot control urticaria efficiently, although there have been reports of improvement with barrier creams before exposure to water.14
The cases reported above are relevant because they are the first cases of family aquagenic urticaria described in Brazil. The patient in Case 2 is probably the youngest patient with this condition in the literature, and the only case with lesions soon after birth.2,4,7,10
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Maria Bandeira de Melo Paiva Seize
Alameda Joaquim Eugenio de Lima, 1.094 ap. 82
01403 002 São Paulo SP
Tel.:/Fax: 55 11 3887-2245 / 8315-1488 / 3088-9000
Conflict of interest: None
Financial funding: None
How to cite this article: Seize MBMP, Ianhez M, Souza PK, Rotta O, Cestari SCP. Urticária aquagênica familiar: relato de dois casos e revisão da literatura. An Bras Dermatol. 2009;84(5):530-3.