Aquagene palmoplantar keratoderma

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Aquagenes acrokeratoderm; Aquagenes pseudokeratoderm; Aquagenic palmar keratoderma; Aquagenic (pseudo) keratoderma; aquagenic syringeal acrokeratoderma; Localized aquagenic syringeal acrokeratoderma; Transient reactive papulotranslucent acrokeratoderm; washerwoman's hands; Watersport Hands

History
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English and McCollough, 1996

Definition
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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.

Occurrence/Epidemiology
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Incidence figures are not available. The literature is limited to individual casuistry. It is mainly women who are affected.

Etiopathogenesis
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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 - gray 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. Osmotically relevant changes of the produced sweat are held responsible for this.

Furthermore, the triggering by drugs is also discussed (aspirin, indomethacin, salazopyrin, COX-2 inhibitors).

Manifestation
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First manifestation in adolescence

Localization
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Palms, the thenar and hypothenar region is preferred.

Clinical features
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Mostly bilateral (almost only occurring on the palms of the hands), more rarely unilateral localized skin changes after contact with water or after increased sweating of the hands. These show themselves in the form of approximately 2cm large, island-like but also large-area, itchy, burning or even aching, white, wrinkled, edematous plaques. The symptoms always disappear within a few minutes or a few hours after the hands have dried. In patients without lesions, the "hand-in-the-bucket" sign, the immersion of the hands in water with triggering of the symptoms, can be diagnostically groundbreaking.

Differential diagnosis
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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.

Therapy
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Literature
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  1. Coelho-Macias V et al.(2013) Aquagenic keratoderma associated with a mutation of the cystic fibrosis gene. Rev Port Pneumol 19:125-128.
  2. English JC 3rd et al. (1996) Transient reactive papulotranslucent acrokeratoderma.
    J Am Acad Dermatol 34:686-687.
  3. Ertürk-Özdemir E et al. (2015) Acquired aquagenic syringeal acrokeratoderma: A case series of 10 patients. Australas J Dermatol 56:e43-5.
  4. Errichetti E et al (2015) Aquagenic keratoderma treated with tap water iontophoresis. Indian J Dermatol 60: 212

  5. Houle MC et al(2010) Unilateral aquagenic keratoderma treated with botulinum toxin A. J Dermatol Case Rep 4:1-5.
  6. Kent JB et al (2014) Watersport hands. Sports Health 6:360-362.

  7. Mengi G (2016) Aquagene palmoplantar keratoderma. Nude Dermatol 42: 467-482

  8. 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.
  9. Rongioletti F et al(2012) Aquagenic (pseudo) keratoderma: a clinical series with new pathological insights. Br J Dermatol 167:575-582.
  10. Sezer E et al (2015) Permanent treatment of aquagenic syringeal acrokeratoderma with endoscopic thoracic sympathectomy. Indian J Dermatol Venereol Leprol 81:648-650.

  11. Uyar B (2014) Aquagenic syringeal acrokeratoderma. Indian J Dermatol 59:632.

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020