SUCCESSFUL ETRETINATE THERAPY OF OLMSTED SYNDROME

A 36-year-old North American patient has been suffering from Olmsted s yndrome since early childhood. This rare inborn error of keratinizatio n consists in pronounced, mutilating keratoderma of palms and soles an d associated periorificial keratoses. The patient has now been treated with etretinate for more than 6 years, resulting in a marked reductio n of hyperkeratoses. Electron microscopic investigation of skin biopsi es taken before and during therapy have confirmed the significant clin ical improvement. Even though this retinoic acid derivative, a powerfu l regulating agent with a de-differentiating effect, cannot cure the b asic defect, the marked hyperproliferation of the epidermis is reduced , so that terminal differentiation is delayed and thereby normalized. At the same time, the synthesis of keratinization proteins, such as ke ratins and keratohyalin, which is suppressed before therapy, increases . The familial connective tissue disorder existing independently of Ol msted syndrome in this patient (perhaps a mild form of Ehlers-Danlos s yndrome) is not influenced by the retinoid therapy.