J Korean Acad Pediatr Dent.  2019 Nov;46(4):416-421. 10.5933/JKAPD.2019.46.4.416.

Dental Management in a Patient with Congenital Insensitivity to Pain with Anhidrosis : A Case Report

• Bae C
• Lee D
• Kim J
• Yang Y

• KMID: 2467078
• DOI: http://doi.org/10.5933/JKAPD.2019.46.4.416

Abstract

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare, autosomal recessive disorder; affected patients are characterized by inability to feel pain and to sweat over the entire body, as well as by mental retardation. Because, in the oral examination, no specific findings on soft or hard tissue may be found except possible lesions due to self-mutilation, early recognition and diagnosis are essential for these patients. Pediatric dentists must be aware of the clinical manifestations and treatment considerations related to uncontrolled body temperature, tactile hyperesthesia and lack of pain reflex. In this case report, dental management of CIPA was suggested by presenting a 6-year follow-up of young patient.

Keyword

CIPA; Pain insensitivity; Anhidrosis; Self-mutilation

MeSH Terms

Body Temperature
Dentists
Diagnosis
Diagnosis, Oral
Follow-Up Studies
Hereditary Sensory and Autonomic Neuropathies*
Humans
Hyperesthesia
Hypohidrosis
Intellectual Disability
Pain Insensitivity, Congenital*
Reflex
Sweat