THE OLMSTED-SYNDROME

A 35-year-old man had a hyperkeratotic disorder of the palmoplantar sk in since the age of 6 months. The palmoplantar keratoderma progressed to thick and warty hyperkeratotic plaques, which enlarged and formed v errucous lesions and deep fissures. The acral keratoderma gradually in volved the dorsal surface of the hands and feet with flexion contractu res of the fingers and toes (Fig. 1). Since the age of 2 years, the pa tient also showed universal alopecia and small sharply marginated hype rkeratotic plaques around the nose and mouth, in the groin, and in the intergluteal area (Fig. 2). All of these keratotic lesions were strik ingly symmetric. Examination of the oral mucosa revealed a white plaqu e over the left lateral border of the tongue; crested 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 pha lanx of each of the fingers. The palmoplantar keratoderma has been com plicated by the development of constricting bands of keratin around th e bases of the fingers, leading to their strangulation and autoamputat ion (Fig. 1). The mental and physical appearance were normal, A family history of similar verrucous lesions was lacking. Histopathologic exa mination of the palmar skin showed massive parakeratotic hyperkeratosi s and considerable acantilosis. In the thickened horny layer, many cel ls showed perinuclear edema. The patient was treated with topical reti noic acid cream, 0.1%, at night. This produced an improvement iii the plaques around the nose and mouth.