The Olmsted syndrome

A 35-year-old man had a hyperkeratotic disorder of the palmoplantar skin since the age of 6 months. The palmoplantar keratoderma progressed to thick and warty hyperkeratotic plaques, which enlarged and formed verrucous lesions and deep fissures. The acral keratoderma gradually involved the dorsal surface of the hands and feet with flexion contractures of the fingers and toes (Fig. 1). Since the age of 2 years, the patient also showed universal alopecia and small sharply marginated hyperkeratotic plaques around the nose and mouth, in the groin, and in the intergluteal area (Fig. 2). All of these keratotic lesions were strikingly symmetric. Examination of the oral mucosa revealed a white plaque over the left lateral border of the tongue; crusted white verrucous plaques were found at the labial commissures and underneath the lower lip. There was complete absence of the nails and the roentgenographic examination of the hand showed pronounced osteolysis of the distal phalanx of each of the fingers. The palmoplantar keratoderma has been complicated by the development of constricting bands of keratin around the bases of the fingers, leading to their strangulation and autoamputation (Fig. 1). The mental and physical appearance were normal. A family history of similar verrucous lesions was lacking. Histopathologic examination of the palmar skin showed massive parakeratotic hyperkeratosis and considerable acanthosis. In the thickened horny layer, many cells showed perinuclear edema. The patient was treated with topical retinoic acid cream, 0.1%, at night. This produced an improvement in the plaques around the nose and mouth.