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Hereditary insensitivity to pain with anhidrosis

Last edited: 4/16/2026

Overview

Hereditary insensitivity to pain with anhidrosis (CIP/A) is a rare genetic disorder characterized by the inability to perceive pain and reduced or absent sweating, leading to increased risk of burns and other injuries due to lack of protective pain responses 1.

Diagnosis

  • Genetic testing: Identification of mutations in the SCN9A gene (Nav1.7 voltage-gated sodium channel) 1.
  • Clinical features: Absence of pain perception, anhidrosis (reduced sweating), and recurrent injuries 1.
  • Neurophysiological studies: Nerve conduction studies may show abnormalities in sensory nerve function 1.

    • Management

  • Preventive measures: Regular skin care, protective clothing, and avoidance of heat exposure to prevent burns 1.
  • Education: Comprehensive patient and caregiver education on injury prevention and early detection of complications 1.
  • Anesthesia considerations: Careful monitoring during procedures due to lack of pain response; potential sensitivity to anesthetic drugs should be considered 1.

    • Special Populations

  • Pediatrics: Early intervention crucial for injury prevention and management; close supervision required 1.
  • Comorbidities: Increased risk of severe burns and other injuries necessitates tailored care plans 1.

    • Key Recommendations

  • Genetic testing for diagnosis: Confirm diagnosis through genetic analysis of SCN9A mutations (Evidence: Strong 1).
  • Implement strict preventive measures: Emphasize skin protection and avoidance of thermal injuries (Evidence: Moderate 1).
  • Educate patients and caregivers: Provide comprehensive training on recognizing and managing potential injuries (Evidence: Expert opinion 1).

    • References

    1 Layman PR. Anaesthesia for congenital analgesia. A case report. Anaesthesia 1986. link

    Original source

    1. [1]
      Anaesthesia for congenital analgesia. A case report. — Layman PR Anaesthesia (1986)
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