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Most small-fiber neuropathies cause distal dysesthesias. In contrast, this new report describes 23 patients with mostly symmetric, non–length-dependent pain of previously unknown etiology. The patients, who were seen at four neuromuscular centers, underwent comprehensive studies, including skin biopsy for evaluation of epidermal nerve fibers and testing of sympathetic skin responses and parasympathetic function. Neuropathic pain developed acutely in eight (35%). Two thirds had involvement of the tongue, scalp, or both, and the trunk was affected in another two thirds. Limb involvement often began in the hands or face before the legs. Autonomic symptoms were uncommon.
Clinical features and epidermal nerve fiber analysis confirmed small-fiber …