Congenital insensitivity to pain with anhidrosis : A case report

Congenital insensitivity to pain with anhidrosis (CIPA) is a very rare gene tic disorder of the peripheral nervous system characterized by recurrent ep isodes of unexplained fever, generalized anhidrosis, insensitivity to pain and temperature, and accompanied by self-mutilating behavior and mental ret ardation, We report on a 16 month-old boy with CIPA who exhibited these cha racteristic clinical features. A sural nerve biopsy revealed markedly reduc ed numbers of unmyelinated and small myelinated fibers, consistent with the characteristic features of CIPA.