Pachyonychia congenita (PC) is an autosomal dominant genodermatosis characterized by a triad of chronic severe plantar pain, focal palmoplantar keratoderma and hypertrophic nail dystrophy. Plantar pain can be debilitating and have a profound impact on quality of life. Current therapeutic options for pain in PC are limited to lifestyle adjustment and mechanical techniques, with a small subgroup of patients benefiting from oral retinoids. This review investigates the pathogenesis of pain in PC and provides a summary of the current and future therapeutic options.
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http://dx.doi.org/10.1093/ced/llae199 | DOI Listing |
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