Unseen Pain: Understanding Congenital Insensitivity to Pain with Anhidrosis and Riga-Fede Disease

Congenital Insensitivity to Pain with Anhidrosis (CIPA) is a rare hereditary sensory and autonomic neuropathy type IV disorder characterized by an inability to feel pain and temperature, with impaired sweating results from mutations in the NTRK1 gene, affecting nerve development and function. Patients often suffer from repeated injuries, burns, and fractures due to their inability to perceive pain, leading to severe complications. Anhidrosis further predisposes individuals to life-threatening hyperthermia. CIPA is diagnosed through clinical evaluation and genetic testing. Currently, no cure exists, and management focuses on injury prevention and supportive care. A 4-month-old male infant was presented to the department with a traumatic ulcer on the ventral surface of the tongue involving the lingual frenum and the tip of the tongue. The early eruption of the lower central incisors was observed. The patient had recurrent fever for 1 month and was on medication. Initial diagnosis was made as Riga Fede Disease but later with subsequent symptoms diagnosed as CIPA. The treatment performed consisted of the extraction of the lower central incisors, debridement of the ulcerative lesion, continuation of systemic antibiotics, and regular follow-up. Complete wound healing with the regeneration of tongue was observed after 1 month follow up.