Pachyonychia congenita tarda.
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.
Lucker
G P
GP
Department of Dermatology, University Hospital, Nijmegen, The Netherlands.
Steijlen
P M
PM
D005190:Family; D006801:Humans; D007645:Keratoderma, Palmoplantar / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009260:Nail Diseases / Q000473:pathology*; D013577:Syndrome