Hereditary insensitivity to pain with anhidrosis.

Abstract

Hereditary sensory neuropathy type IV is an autosomal-recessive disorder characterized by congenital insensitivity to pain and anhidrosis and resulting in recurrent hyperpyrexia, self-mutilation, recurrent infections, chronic osteomyelitis, bone and joint deformities, and limb amputations. Described is a child with signs as well as skin and nerve biopsy results compatible with this disease, emphasizing the importance of early diagnosis and appropriate medical and educational care to prevent complications.

• Authors: M Berkovitch, L Copeliovitch, T Tauber, Z Vaknin, E Lahat
• Journal: Pediatric neurology (Pediatr Neurol) Vol. 19 Issue 3 Pg. 227-9 (Sep 1998) ISSN: 0887-8994 [Print] United States
• PMID: 9806143 (Publication Type: Case Reports, Journal Article)

Topics

• Biopsy
• Child, Preschool
• Consanguinity
• Female
• Hereditary Sensory and Autonomic Neuropathies (complications, diagnosis, genetics)
• Humans
• Hypohidrosis (etiology)
• Intellectual Disability (etiology)
• Mental Disorders (etiology)
• Neurologic Examination
• Osteomyelitis (etiology)
• Pain Insensitivity, Congenital (etiology)
• Sural Nerve (pathology)
• Sweat Glands (pathology)