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A 6-year-old girl had congenital sensory neuropathy with anhidrosis (CSNA), one of the five variants of a group of very rare genetic disorders of the peripheral nervous system--hereditary sensory neuropathies (HSN). Clinical, laboratory, and physiopathologic aspects are discussed. Dermatologic findings of anhidrosis and self-mutilation suggest the diagnosis.
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Mariano et al. (1994) studied this question.
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