CONGENITAL INSENSITIVITY TO PAIN WITH ANHIDROSIS : CASE REPORT

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Journal of the Korean Academy of Pediatric Dentistry 1999;26(1):139-145.
Published online February 28, 1999.

CONGENITAL INSENSITIVITY TO PAIN WITH ANHIDROSIS : CASE REPORT

Congenital Insensitivity to Pain with Anhidrosis 환아의 치험례

김태근, 최남기, 양규호

전남대학교 치과대학 소아치과학교실

Abstract

Congenital Insensivity to Pain with Anhidrosis(CIPA) is rare autosomal recessive disorder which is known to be hereditary sensory and autonomic neuropathies(HSAN) type IV. It is predilection for male and observed in early infant. Its clinical features include congenital analgesia, which leads to self-mutilation; inability to sweat, which leads to defective thermoregulation; and mild to moderate mental retardation. Its dental features include self-mutilation by nail peeling, which leads to gingival ulcer, tongue ulcer due to tongue biting, and enamel hypoplasia. Partial anodontia is often observed as well. Seventeen-months-old boy with CIPA was reported. This is the case in which the dental characteristics are described and the dental treatment of patient is discussed.

Key Words: Congenital Insensitivity to Pain with Anhidrosis, self-multilation