Aquagene palmoplantar keratoderma

English and McCollough, 1996

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.

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

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).

First manifestation in adolescence

Palms, the thenar and hypothenar region is preferred.

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.

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.

Apply 15-20% aluminium chloride solutions 2x/day.

Alternative: Tap water iontophoresis.

Alternative: Botulinum toxin injections

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