Congenital sensory neuropathy with anhidrosis

Congenital sensory neuropathy with anhidrosis (CSNA) is a well-defined entity among a group of sensory deficiency syndromes. Children with this genetic disease are insensitive to pain and temperature, do not sweat, and suffer from mental retardation. Self-mutilation may be severe and may lead to insoluble orthopedic problems. We treated a girl with CSNA, who was born to nonconsanguineous Japanese parents. This disease is important in the dermatological field, since the degree of anhidrosis and self-mutilation influence the prognosis of patients with CSNA.