Olmsted syndrome

Background. The Olmsted syndrome is a rare congenital disorder with palmopl antar keratoderma. and hyperkeratotic periorifical plaques. Only 19 cases w ere reported in the literature. Other cutaneous and organic findings have b een reported with different frequencies. Case report. A 17-year-old girl presented with genital, perigenital and per ianal hyperkeratotic plaques extending to the thighs, palmoplantar keratode rma and nail dystrophy. A small erythematokeratotic plaque was localized be low the lower lip. Improvement of the palmoplantar and genital lesions was noted after one month of treatment with 25 mg acitretine daily. Further sub stantial improvement of the palmoplantar and ungueal lesions was observed d uring the next 7 months. However, discrete erythematous perianal and perige nital lesions persisted in these areas 2 years later. Discussion. Efficacy of retinoids in the Olmsted syndrome is controversiall y discussed in the literature. However, acitretin seems to be the treatment of choice of this syndrome in adults.