Partial improvement of Olmsted syndrome with etretinate

An 11-year-old Japanese boy with Olmsted syndrome was seen at our clinic. He had a sharply marginated, painful keratoderma with a red border on his palms and soles. Flexion contractures of the fingers were also observed. Hyperkeratotic plaques were present below the lower lip, on the elbows and knees, and in the sacral area. Localized alopecia, leukokeratosis on the tongue, shortness of stature, and laxity of the large joints corresponded to the clinical features of Olmsted syndrome. Treatment with etretinate was effective for the palms and fingers, but resulted in no improvement of the keratoderma of the soles. Because of periosteal thickening of the tibia, presumably caused by etretinate, therapy was terminated one year after it began.