Osteosclerosis of the phalanges in Werner syndrome.

ABSTRACT

Werner syndrome is an autosomal-recessive disease characterized by premature aging, shortness of stature, scleroderma-like skin changes, endocrine abnormalities, and cataracts. Although radiographic findings have been well documented, the presence of distinctive osteosclerotic changes in the phalanges of the hands and feet has not been emphasized in previous publications. The authors' review of radiographs of both hands in nine patients and of both feet in six patients with Werner syndrome documented the frequent occurrence of phalangeal sclerosis related predominantly to endosteal thickening. In the hand, sclerosis was present in every patient, was generally symmetric in distribution, predominated in the distal phalanges, and demonstrated an ulnar predilection. Similar changes in the phalanges of the feet were demonstrated in only two patients. The presence of osteosclerosis in the phalanges of the hand alone or both the hand and foot, when combined with osteoporosis and periarticular calcification, suggests the diagnosis of Werner syndrome.