HistoryThis section has been translated automatically.
English and McCollough, 1996
DefinitionThis section has been translated automatically.
Rare palmar skin disease triggered by contact with water and sweat, which only manifests itself in a moist skin condition. Dry skin shows no clinical changes, including palmar keratosis.
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Occurrence/EpidemiologyThis section has been translated automatically.
Incidence figures are not available. The literature is limited to individual casuistry. It is mainly women who are affected.
EtiopathogenesisThis section has been translated automatically.
In one third of the cases an association to a mutation of the "Cystic Fibrosis Transmembrane Conductance Regulator gene (CFTR gene) on chromosome 7q31.2 could be established (6 mutation classes with about 2,000 mutations of the CFTR gene are known). The consequence of this mutation is a defective CFTR protein. Clinically, such mutations lead to autosomal recessive cystic fibrosis (E 84.9).
Defective CFTR proteins represent defective chloride channels which cause functional disorders in all exocrine glands, including the sweat glands of the skin (chloride content of sweat > mmol/l -grau range 30-60 mmol/l). Pathogenetically, an increased fluid absorption via the palmoplantar skin barrier with the consequence of swelling of eccrine sweat glands is discussed. The osmotically relevant changes in the sweat produced are held responsible for this.
Furthermore, the triggering by drugs is discussed (aspirin, indomethacin, salazopyrin, COX-2 inhibitors).
ManifestationThis section has been translated automatically.
First manifestation in adolescence
LocalizationThis section has been translated automatically.
Palms, the thenar and hypothenar region is preferred.
Clinical featuresThis section has been translated automatically.
Mostly on both sides (almost only on the palms), more rarely on one side localized skin changes after contact with water or after increased sweating of the hands. These appear in the form of approximately 2cm large, island-shaped but also large, itchy, burning or also painful, white, shrivelled, oedematous plaques. The symptoms always resolve within a few minutes or a few hours after drying of the hands. In patients without lesions, the "hand-in-the-bucket" sign, the immersion of the hands in water with triggering of the symptoms, may be diagnostic.
Differential diagnosisThis section has been translated automatically.
The clinical picture is to be distinguished from palmoplantar keratosis, aquagenic, diffuse, non-epidermolytic (type Bothnian) in which a mutation of the AQP5 gene aquaporin is present.
TherapyThis section has been translated automatically.
LiteratureThis section has been translated automatically.
- Coelho-Macias V et al.(2013) Aquagenic keratoderma associated with a mutation of the cystic fibrosis gene. Rev Port Pneumol 19:125-128.
- English JC 3rd et al (1996) Transient reactive papulotranslucent acrokeratoderma. J Am Acad Dermatol 34:686-687.
- Ertürk-Özdemir E et al (2015) Acquired aquagenic syringeal acrokeratoderma: A case series of 10 patients. Australas J Dermatol 56:e43-5.
- Errichetti E et al (2015) Aquagenic keratoderma treated with tap water iontophoresis. Indian J Dermatol 60: 212
- Houle MC et al.(2010) Unilateral aquagenic keratoderma treated with botulinum toxin A. J Dermatol Case Rep 4:1-5.
- Kent JB et al (2014) Watersport hands. Sports Health 6:360-362.
- Mengi G (2016) Aquagenic palmoplantar keratoderma. Act Dermatol 42: 467-482
- Nadal M eg al (2015) Aquagenic palmar keratoderma in a patient heterozygous for the mutation c.3197G>C in the CFTR gene. Ann Dermatol Venereol 142:201-205.
- Rongioletti F et al.(2012) Aquagenic (pseudo) keratoderma: a clinical series with new pathological insights. Br J Dermatol 167:575-582.
- Sezer E et al. (2015) Permanent treatment of aquagenic syringeal acrokeratoderma with endoscopic thoracic sympathectomy. Indian J Dermatol Venereol Leprol 81:648-650.
- Uyar B (2014) Aquagenic syringeal acrokeratoderma. Indian J Dermatol 59:632.
Outgoing links (4)Aluminium chloride hexahydrate solution, 15/20% 2-propanol (nrf 11.1.); Botulinum toxin a; Iontophoresis; Palmoplantar keratosis aquagene diffuse non-epidermolytic (type bothnian);
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