Which histologic findings are characteristic of erythromelalgia?

Updated: Sep 17, 2018
  • Author: Antoine N Saliba, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

In patients with thrombocythemia, skin biopsy results have shown arteriolar endothelial cell swelling, with sparing of venules, capillaries, and nerves. Thickening of the vessel wall and narrowing of the lumen occur as a consequence of smooth muscle cell proliferation. Thickened arterioles may contain occlusive thrombi and ultimately may become fibrotic.

Biopsy specimens in patients with primary erythromelalgia show mild mononuclear perivascular infiltrates with edema, thickened vascular basement membranes, and moderate endothelial swelling; the intimal thickening and thrombi seen in secondary erythromelalgia are lacking.


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