Treatment of hereditary palmoplantar keratoderma : a review by analysis of the literature
© 2020 British Association of Dermatologists..
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 with hPPK since 2008, their efficacy and safety, with an evaluation of the various therapeutic modalities that can be used to treat hPPK.
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 all PPK forms, and with variable tolerance. New targeted treatments, according to the specific mechanisms of hPPK, 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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2021 |
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| Erschienen: |
2021 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:184 |
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| Enthalten in: |
The British journal of dermatology - 184(2021), 3 vom: 19. März, Seite 393-400 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Bodemer, C [VerfasserIn] |
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| Links: |
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| Themen: |
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| Anmerkungen: |
Date Completed 02.06.2021 Date Revised 02.06.2021 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1111/bjd.19144 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM308924673 |
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| 245 | 1 | 0 | |a Treatment of hereditary palmoplantar keratoderma |b a review by analysis of the literature |
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| 500 | |a Date Revised 02.06.2021 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2020 British Association of Dermatologists. | ||
| 520 | |a 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 | ||
| 520 | |a OBJECTIVES: 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 | ||
| 520 | |a METHODS: We undertook a comprehensive review of the literature data published since 2008 | ||
| 520 | |a 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 all PPK forms, and with variable tolerance. New targeted treatments, according to the specific mechanisms of hPPK, appear promising for the future | ||
| 520 | |a 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 | ||
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| 650 | 4 | |a Review | |
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| 650 | 7 | |a Retinoids |2 NLM | |
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| 700 | 1 | |a O'Toole, E A |e verfasserin |4 aut | |
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