Oral and maxillofacial manifestations of hereditary sensory neuropathy

Hereditary sensory neuropathies are a rare group of neurological disorders manifested from early childhood by diminished or absent sensibility to pain, touch and temperature. A Kashmiri family with four members affected by congenital sensory neuropathy and its oral manifestations is described. Pain and temperature sensation was lost in various parts of the body including the orofacial region resulting in mutilating acropathy, particularly of the limbs and face. Orofacial motor function was normal. Three of the four members had corneal opacilication due to scarring from keratitis. To prevent any further mutilation, any corrective surgery is best delayed until the patient is old enough. A discussion of the oral manifestations of this condition with a review of the literature is presented.