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.
Ishii
N
N
Department of Dermatology, Yokohama City University School of Medicine, Japan.
Kawaguchi
H
H
Miyakawa
K
K
Nakajima
H
H
D005260:Female; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D007007:Hypohidrosis / Q000150:complications*; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000150:complications; D012652:Self Mutilation / Q000209:etiology; D013577:Syndrome