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Primary or idiopathic erythermalgia is characterized by recurrent, red, warm, and painful lower extremities. It arises at young age and persists throughout life because no treatment is available. We report the cutaneous pathology of affected skin lesions of three patients with primary erythermalgia. Biopsy specimens showed a mild perivascular mononuclear infiltrate, thickened blood vessel basement membranes, abundant perivascular edema, and moderate endothelial swelling. The thickened basal membrane of the blood vessels showed a laminar structure, and abundant perivascular edema and moderate endothelial cell swelling were evident. These histopathologic findings in primary erythermalgia appear to be nonspecific but allow diagnostic differentiation from erythromelalgia in which fibromuscular intimal proliferation and occlusive thrombi in the endarteriolar capillaries are apparent and from erythermalgia secondary to vasculitis. Histopathologic examination of affected skin lesions in patients with red, congested, warm, and painful burning extremities is a valuable tool in the diagnostic process. Erythromelalgia and erythermalgia are defined as two independent and completely different disease entities (1-3). Clinically, there are three different types of recurrent red, warm, and burning pain in the extremities. Erythromelalgia is closely associated with thrombocythemia (>=350 × 10/L) in its primary form or is associated with various chronic myeloproliferative disorders (4,5). Symptoms are caused by platelet activation and aggregation in the end-arteriolar circulation, leading to ischemia and symptoms. The long-lasting relief of burning distress for 2-4 days afforded by a single low dose of aspirin is the valuable diagnostic test for erythromelalgia (6). The histopathology of erythromelalgia comprises arteriolar changes with narrowing of the lumen by (a) swelling of the endothelial cell lining, (b) proliferation of smooth muscle cells (SMC) with vacuolization and swelling of the cytoplasm, and (c) with or without thrombi (7-9). Primary or idiopathic erythermalgia is rare and arises at young age as a bilateral symmetric burning and redness of mainly the lower extremities in the absence of detectable disease (1-3). The diagnosis is made only according to a list of clinical criteria. Its pathogenesis remains elusive, and symptoms persist throughout life and are refractory to any treatment method. Secondary erythermalgia usually occurs at adult age in association with various underlying disorders (1,10-14). Diagnostic differentiation between primary erythermalgia and erythromelalgia is very important since treatment and, consequently, prognosis differ. Article Tools Complete Reference Abstract Reference Print Preview Email Jumpstart Email PDF Jumpstart Email Article Text