Congenital Sensory Neuropathy With Anhidrosis

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.