Findings: Plain films of the left hand reveal resorption of the distal phalanges. There is also absence of cortical bone along the middle phalanx of the 4th digit. Narrowing of the carpometacarpophalangeal joints is visualized, as well as several lesions at the heads of the 2nd, and 5th phalanges.

Diagnosis: Scleroderma with acroosteolysis.

Discussion: Scleroderma (progressive system sclerosis) is a collagen vascular disease which commonly involves the hands. Manifestations of PSS include Raynaud's phenomenon (pain, cyanosis, coolness of the distal extremities), ulcerations, flexion contractures and telangiectasias. These changes are thought to be due to intimal layer hypertrophy and inflammation; collagen disposition and fibrosis occur resulting in subsequent vessel lumen occlusion. Such vascular alterations have been described in the digital arteries, superficial palmar arch and ulnar artery. Angiographic studies have demonstrated little or no collateral circulation distal to the fibrotic vessels. In addition to bony involvement, visceral organs are often affected. Bilateral basilar lung fibrosis, pulmonary vascular hypertension, esophageal and small bowel dysmotility are not infrequent. Mortality is usually secondary to disease of the heart (cardiomyopathy, arrhythmias) and kidney (angiotension-related vasoconstriction, systemic hypertension and renal failure).

Classic radiographic findings in the hands include soft tissue calcifications, atrophy of the finger tips (tapering), "autoamputation" of the terminal tufts (penciling) and intercarpal joint space narrowing. The differential diagnosis is as follows: psoriasis, diabetes, hyperparathyroid, frostbite, sarcoidosis, leprosy, occupational exposures (polyvinyl chloride), post-traumatic and congenital (syndrome of Hajdu and Cheney).

References:

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3. Rad Clin N Amer 1988 Nov; 26(6):1195-1212.
4. Diag Imag Clin Med 1986;55(4-5):262-265