Congenital insensitivity to pain with anhidrosis( CIPA) is associated with systemic sweating and loss of pain and temperature senses; the clinical symptoms include mental retardation, fever of unknown origin, frequent trauma and burns, and self-injury. CIPA is a rare autosomal recessive disorder classified as hereditary sensory and autonomic neuropathy type IV. We report a case of CIPA in an infant in whom intractable sublingual ulcers developed at the time of mandibular primary central incisor eruption.
The patient was a 6-month-old boy with no abnormalities during pregnancy, labor, or after birth. Fever of unknown origin intermittently occurred since he was 1 month old. Because no signs of discomfort or crying were observed when securing peripheral blood vessels or inserting a needle for blood collection, and sweating had never been observed, the patient was suspected to have CIPA and thus underwent a sweat test at the pediatrics department of this hospital. There was no discoloration at the application site, which indicated anhidrosis. Because the patient had a poor response to pain stimuli, he was given a clinical diagnosis of CIPA. The patient was referred to the department of dentistry and oral surgery in this hospital because sublingual ulcer formation was observed at the time of mandibular primary central incisor eruption at 5 months of age. An approximately 10-mm shallow ulcer without bleeding was found on the sublingual mucosa. A corticosteroid ointment was topically applied to the affected area, and a mandibular protection device was provided. Although the ulcer appeared to be healing, self-injury might occur when teeth begin to erupt; therefore, close follow-up of the patient is required.
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