A case of congenital insensitivity to pain with anhidrosis.

Congenital insensitivity to pain with anhidrosis (CIPA) syndrome is a rare autosomal recessive disease which is also known as hereditary sensory and autonomic neuropathy type 4 (1). The prominent characteristics of the disease include fever due to anhidrosis, absence of sense of pain, painless ulcers in the structures inside the mouth and extremities, self-harm behavior, mild to severe mental retardation, myelination defect in the sural nerve biopsy and loss of small myelinized fibers. Infection in the fingers and toes, lips and tongue and scarring is observed commonly. Keratoderma palmoplantaris is a typical finding in the advanced period (1, 2). Other problems include chronic bone and joint infections.