Author(s): Sriram P, lamaran V, Tejal Risbud, Srinivasa Raghavan, Sreejit R
Hereditary sensory and autonomic neuropathy type IV or Congenital insensitivity to pain with anhydrosis is a rare disorder. It is an autosomal recessive disorder characterized by absence of small myelinated and unmyelinated sympathetic nerves resulting in pain and temperature insensitivity. There is also anhydrosis due to lack of innervations of sweat glands resulting in recurrent episodes of hyperpyrexia. Two cousins with history of insensitivity to pain and temperature with signs of self mutilation, recurrent episodes of febrile seizures, and corneal anaesthesia are reported.