Bifid tongue in hereditary sensory and autonomic neuropathy type IV

Abstract

Hereditary sensory and autonomic neuropathy type IV or Congenital insensitivity to pain with anhydrosis is a rare autosomal recessive disorder. It is characterized by absence of small myelinated and unmyelinated sympathetic nerves resulting in pain and temperature insensitivity. We report a case of a 15 year old boy with hereditary sensory and autonomic neuropathy type IV associated with bifid tongue which is an extremely rare oral presentation and not often reported in literature. Keywords: Hereditary sensory and autonomic neuropathy type IV, Bifid tongue, Self mutilation.