A Case of Congenital Insensitivity to Pain with Anhidrosis.
- Author:
Munhyang LEE
1
;
Nam Seon BECK
;
Dongkyu JIN
;
Yeon Lim SUH
Author Information
1. Department of Pediatrics, Sung Kyun Kwan University, College of Medicine, Samsung Medical Center, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Congenital insensitivity to pain with Anhidrosis (CIPA)
- MeSH:
Female;
Fever;
Hereditary Sensory and Autonomic Neuropathies*;
Humans;
Hypohidrosis;
Infant;
Intellectual Disability;
Natural History;
Pain Insensitivity, Congenital*;
Tongue
- From:Journal of the Korean Pediatric Society
1997;40(11):1621-1621
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Congenital insensitivity to pain with anhidrosies (CIPA) is one of the exceedingly rare hereditary sensory autonomic neuropathies (HSAN). There are five types of HSAN according to hereditary transmission, natural history, clinical manifestations, and pathologic findings. The CIPA corresponds to type IV and is characterized by episodes of recurrent fever with generalized anhidrosis, insensitivity to pain and temperature with self-multilation, and mental retardation. We report a 14 month old girl with the clinical symptoms and neuropathological findings of CIPA without family history. She presented with episodes of recurrent fever and self-mutilation of her tongue. Clinical, laboratory and pathologic aspects of this patient are discussed along with review of the literature.
