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 pa thology of affected skin lesions of three patients with pri mary erythermalgia. Biopsy specimens showed a mild perivascular mononuclear infiltrate, thickened blood ves sel basement membranes, abundant perivascular edema, and moderate endothelial swelling, The thickened basal membrane of the blood vessels showed a laminar struc ture, and abundant perivascular edema and moderate en dothelial cell swelling were evident. These histopatholog ic findings in primary erythermalgia appear to be nonspe cific b u t allo w d ia g n o stic d iffe re n tia tio n from erythromelalgia in which fibromuscular intimal prolifera tion and occlusive thrombi in the endarteriolar capillaries are apparent and from erythermalgia secondary to vascu litis. Histopathologic examination of affected skin lesions in patients with red, congested, warm, and painful burn ing extremities is a valuable tool in the diagnostic pro cess.
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