HEREDITARY INSENSITIVITY TO PAIN WITH ANHIDROSIS

Hereditary sensory neuropathy type IV is an autosomal-recessive disord er characterized by congenital insensitivity to pain and anhidrosis an d resulting in recurrent hyperpyrexia, self-mutilation, recurrent infe ctions, chronic osteomyelitis, bone and joint deformities, and limb am putations. Described is a child with signs as well as skin and nerve b iopsy results compatible with this disease, emphasizing the importance of early diagnosis and appropriate medical and educational care to pr event complications. (C) 1998 by Elsevier Science Inc. All rights rese rved.