Mutilating palmoplantar keratoderma with periorificial keratotic plaques (Olmsted's syndrome).
We report the case of a boy, now aged 4 years, with a progressive, painful and disabling palmoplantar keratoderma, associated with well-defined hyperkeratotic plaques around the mouth and nostrils. His mother has an identical palmoplantar keratoderma. The palmoplantar keratoderma failed to improve with etretinate, but considerable benefit resulted from full-thickness excision of the skin of both palms, followed by skin grafting. We believe that he has a distinctive but extremely rare form of hereditary palmoplantar keratoderma, first described by Olmsted.
Atherton
D J
DJ
Department of Dermatology, Hospital for Sick Children, London, U.K.
Sutton
C
C
Jones
B M
BM
D002675:Child, Preschool; D005148:Facial Dermatoses / Q000473:pathology*; D006801:Humans; D007645:Keratoderma, Palmoplantar / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D008297:Male; D016038:Skin Transplantation; D013577:Syndrome