Congenial Insensitivity to Pain with Anhydroses (CIPA): A Case Report

Autonomic neuropathy is a rare disorder inherited as insensitivity to pain and temperature. It has been divided into five groups, the fourth of which named congenital insensitivity to pain with anhydrosis, (CIPA) is the most common type of this disorder. CIPA is a recessive inherited condition with courses of idiopathic fever, reduction of sweat glands secretion despite having normal glands, insensitivity to pain and temperature changes, and mild mental retardation. Oral symptoms due to their severe abuse in these patients are considerable. Gas shock tongue, lips and other oral mucosa have been reported.Case Report: Examination of a 15 month old boy revealed severe oral ulcers and burns in the fingers of both hands due to contact with the heater. Pathological studies and special attention to specific aspects of the patient revelead that he was insensitive to pain and thermal stimulation and was diagnosed as a possible case of CIPA. CIPA is a autosomal recessive disorder which is very rare in childhood, as insensitivity to pain or the incidence of abuse and lack of objective response to pain in people with peripheral and central nervous system are normal. Tyrosine gene defect related to gene code provided membrane tyrosine has been identified in these patients. Mutation in this gene results in insensitivity to pain, anhydrosis, self abuse, and mental retardation and is associated with cancer. More than half of the patients die before the age of three. Oral manifestations of this syndrome are the characteristics of the disorder and can help to make an early diagnosis.