OLMSTED SYNDROME - CASE-REPORT AND IDENTIFICATION OF A KERATIN ABNORMALITY

Background: Olmsted syndrome is a rare disorder characterized by a mut ilating palmoplantar keratoderma and periorificial keratotic plaques. It begins in early childhood and is complicated by the development of painful flexion contractures, constrictions, and autoamputations of th e digits. Only 11 cases of Olmsted syndrome have been reported to date . However, no biochemical abnormalities in the skin were reported in a ny of these cases. Observations: We report the 12th case of Olmsted sy ndrome. In addition, we describe a keratin abnormality found in a skin specimen obtained from our patient. The specimen showed a suprabasila r staining pattern with AE1, an antibody that shows only basilar stain ing in normal skin. Conclusion: We report the 12th case of Olmsted syn drome, review the literature, and describe a keratin abnormality that was found in our patient's skin specimen.