2019 Volume 40 Issue 1 Pages 27-32
Introduction：Congenital insensitivity to pain with anhidrosis (CIPA), a rare disorder classified as hereditary sensory and autonomic neuropathy type IV, is considered to show autosomal recessive inheritance with many uncertainties in the pathology. In the present article, we report a 9-month-old girl diagnosed with this disorder on the basis of abnormal ulceration of the tongue.
Case and course：Patient：A 9-month-old girl. First visit：March, 2015. Chief complaint：Ulceration of the tongue and feeding difficulty. Family history：Not applicable. Medical history：In November 2014 when she was 5 months old, she was presented at our pediatrics clinic with aggravation of erosion and swelling on the skin of the bilateral thumbs and left second finger due to finger sucking. History of present illness：On March, 2015, she was admitted to our pediatrics clinic for dehydration due to feeding difficulty and referred to us for close examination of ulceration and bleeding of the tongue. Intraoral findings：The lingual body was rather swelled, and a large ulceration with surrounding crust was observed near the lingual apex. General condition：Dehydration was observed. Right second and third finger nails were peeled, and bites were observed on the bilateral hand fingers. Palpation revealed hand skin dryness. Clinical course after admission：Fluid replacement was started. Her body temperature was 38°C when she was presented due to bleeding of the tongue. Then we started oral care and intervention for ulceration, such as applying dexamethasone ointment and grinding sharp edges of teeth. This patient was referred to pediatrics for close examination of CIPA, suspecting self-injurious behavior leading to abnormal ulceration. The body temperature was reduced after cooling. Dehydration was corrected with fluid replacement. Ulceration was improved, gradually allowing better feeding；therefore, she was discharged on March. Later, the disorder was diagnosed in pediatrics based on the sweating test and clinical symptoms. Now a follow-up is being conducted among pediatrics, orthopedics, and us. A developmental test was performed in pediatrics, indicating mild developmental disorder. She has presented to the orthopedics clinic with a fracture of the lower extremity. Our treatment was discontinued because of a stable intraoral condition, and then regular oral maintenance is performed at a primary dental clinic.
Conclusion：CIPA could be diagnosed on the basis of abnormal ulceration of the tongue. Early detection of and intervention for this disorder should be done even by dentists for improving QOL of a patient and his/her family members.
