Treatment of hereditary palmoplantar keratoderma: a review by analysis of the literature

摘要

Background No specific or curative therapy exists for hereditary palmoplantar keratoderma (hPPK), which can profoundly alter patient quality of life, leading sometimes to severe functional impairment and pain. The rarity and the aetiological diversity of this group of disorders can explain the difficulty in comparing the efficacy of available treatments. Objectives To review the different treatments tried in patients withhPPKsince 2008, their efficacy and safety, with an evaluation of the various therapeutic modalities that can be used to treathPPK. Methods We undertook a comprehensive review of the literature data published since 2008. Results Only a few case series and individual case reports were identified. Topical (emollients, keratolytics, retinoids, steroids) and systemic treatments (mostly different retinoids), often combined, are used to relieve symptoms. Oral retinoids appear to be the most efficient treatment, but not in allPPKforms, and with variable tolerance. New targeted treatments, according to the specific mechanisms ofhPPK, appear promising for the future. Conclusions More studies using robust methodology and involving larger cohorts of well-characterized patients (phenotype-genotype) are necessary and should be prioritized by structured networks, such as the European Network for Rare Skin Diseases (ERN-Skin), with the aim of better management of patients with rare skin diseases.