Olmsted syndrome

Olmsted syndrome is an uncommon inherited disorder of keratinization that p resents mutilating palmoplantar keratoderma, perioral hyperkeratosis, leuko keratosis and alopecia. We report a case of this rare syndrome diagnosed in a 48-year-old woman and confirms the existence of a generalized abnormalit y in keratin expression. Immunoreactivity in our case suggests an abnormal expression of keratins 5 and 14 similar to the observed in other hyperproli ferative disorders.