Successful treatment of pachyonychia congenita with simvastatin

Abstract

Pachyonychia congenita (PC) is a rare autosomal-dominant genodermatosis. It is an ectodermal, dysplastic disorder of keratinization that typically arises at birth or early infancy due to keratin-specific gene mutations, including mutations in KRT6A, KRT6B, KRT6C, KRT16 and KRT17 has a myriad of presentations, the most notable features of which includes nail dystrophy and dyskeratosis of the skin (palmoplantar hyperkeratosis) and mucous membrane changes (leukokeratosis). The latter clinical features were better identified by Jadassohn and Lewandowsky in 1906 as an addition to Muller's findings in 1904.