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

Authors
  • Bodemer, C1
  • Steijlen, P2
  • Mazereeuw-Hautier, J3
  • O'Toole, E A4
  • 1 Department of Dermatology, Reference Centre for Genodermatoses, MAGEC Necker Enfants Malades, Paris-centre University, APHP5, ERN-Skin, France. , (France)
  • 2 Department of Dermatology, Maastricht University Medical Centre and the GROW School for Oncology and Developmental Biology, Maastricht, ERN-Skin, the Netherlands. , (Netherlands)
  • 3 Department of Dermatology, Centre de référence des maladies rares de la peau, Larrey Hospital, Paul Sabatier University, Toulouse, ERN-Skin, France. , (France)
  • 4 Department of Dermatology, Royal London Hospital, Barts Health NHS Trust, London, ERN-Skin, UK.
Type
Published Article
Journal
British Journal of Dermatology
Publisher
Wiley (Blackwell Publishing)
Publication Date
Mar 01, 2021
Volume
184
Issue
3
Pages
393–400
Identifiers
DOI: 10.1111/bjd.19144
PMID: 32307694
Source
Medline
Language
English
License
Unknown

Abstract

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. To review the different treatments tried in patients with hPPK since 2008, their efficacy and safety, with an evaluation of the various therapeutic modalities that can be used to treat hPPK. We undertook a comprehensive review of the literature data published since 2008. 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 all PPK forms, and with variable tolerance. New targeted treatments, according to the specific mechanisms of hPPK, appear promising for the future. 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. © 2020 British Association of Dermatologists.

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