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Edward A. Edwards, M.D.
JAMA. 1956;161(16):1530-1534. doi:10.1001/jama.1956.02970160010003.
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• Pain in hands and feet, with areas of coldness and cyanosis, was observed in 20 patients. The group included both sexes, all ages from the fourth to the ninth decade of life, and both smokers and nonsmokers. Attacks were not limited to any one time of the year.

Material for microscopic study was obtained incident to amputation of toes in two cases and by biopsy in nine additional cases. Characteristic findings were perivascular inflammation, intimal proliferation in the arterioles of the skin, and occlusion of the capillaries of the skin by hyaline thrombi. Remissions occurred in all cases but only after an unpredictable intensity and duration of symptoms. Amputation of a toe became necessary in each of two cases. Lumbar sympathectomy was done, but with little effect, in one case.

The phrase remittent necrotizing acrocyanosis describes this condition objectively. Rest, sedation, and warmth are beneficial; cessation of smoking and use of vasodilator drugs are helpful. Functional vascular spasm and organic occlusion are both factors in this disease.


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