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Pachyonychia congenita tarda
Author(s) -
LUCKER G.P.H.,
STEIJLEN P.M.
Publication year - 1995
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1111/j.1365-2230.1995.tb01307.x
Subject(s) - acitretin , medicine , dermatology , genodermatosis , palmoplantar keratoderma , nail (fastener) , nail disease , keratoderma , genetics , hyperkeratosis , biology , psoriasis , materials science , metallurgy , gene
Summary Pachyonychia congenita is a distinct hereditary disorder of keratinization, in which dystrophy of all nails is associated with palmoplantar keratoderma and other hyperkeratoses. Recently a late‐onset type has been reported. We report a second family with late‐onset pachyonychia congenita, showing a remarkable clinical heterogeneity. Furthermore, one patient demonstrated a number of associated hyperkeratoses not previously recognized. Acitretin proved useful in the treatment of this late‐onset form of pachyonychia congenita.