Aquagenic (Pseudo)keratoderma

Aquagenic (palmoplantar) keratoderma is an uncommon hereditary or sporadic condition, whose pathogenesis is largely uncovered, but characterized by a peculiar transitory palmar and more rarely plantar skin wrinkling after brief water exposure. Association with cystic fibrosis is frequent and related to mutations on the same cystic fibrosis transmembrane conductance regulator gene, usually the ΔF508 mutation, either homozygous or heterozygous. However, aquagenic keratoderma is frequently idiopathic or secondary to other internal organ dysfunctions, as well as drug-induced. Diagnosis is mainly clinical and easily confirmed with an immersion test, if one is aware of this entity. Histopathology is rarely performed, but highly informative of the transient alterations of the stratum corneum and eccrine glands. Sporadic form may resolve spontaneously after a few years or have periods of remission during the life course. For very disturbing conditions, topical aluminum hydroxide and botulinum toxin injections are the most used treatments.