Abstract: Congenital pain insensitivity and anhidrosis is an extremely rare
disorder characterized by lack of pain sensation, painless injuries of the
arms, legs and oral structures, hyperthermia during hot weather because of
inability to sweat, mental retardation, infection and scarring of the tongue,
lips and gums, chronic infections of bones and joints, bone fractures, multiple
scars, osteomyelitis and joint deformities. We report this rare case in a
2-year-old girl who presented with recurrent episodes of high-grade fever with
anhidrosis, dry fissured skin, self-mutilation, tooth loss. She also had three
episodes of generalized tonic-clonic seizure during afebrile period. This case
report highlights the rare association of congenital insensitivity to pain and
anhidrosis with seizure disorder.
