ORAL MANIFESTATIONS OF HEREDITARY SENSORY AND AUTONOMIC NEUROPATHY TYPE-IV - CONGENITAL INSENSITIVITY TO PAIN WITH ANHIDROSIS

Objective. Hereditary sensory and autonomic neuropathy type IV (congen ital insensitivity to pain with anhidrosis) is a rare disorder. In thi s study, we investigated the oral and dental manifestations associated with hereditary sensory and autonomic neuropathy type IV. Study desig n. Eighteen patients with hereditary sensory and autonomic neuropathy type IV whose ages ranged from 1 year 0 months to 22 years 3 months we re examined for oral signs and symptoms of tooth abnormalities, malocc lusions, soft tissue disorders, tongue papilla atrophy, and morphologi c abnormalities of hands and fingers. Results. All 18 patients showed congenital insensitivity to pain and anhidrosis. Oral self-mutilations , such as autoextraction of teeth and severe biting injuries (with res ultant scarring) of the finger tips and oral soft tissues (tongue, lip , and buccal mucosa), were found in most patients. In infant patients the condition was typically characterized by decubital ulcers on the v entral surface of the tongue, resulting from trauma of the incisal edg e of erupting mandibular primary incisors during sucking or nursing. T hese ulcers led to several local and systemic problems, such as tongue bleeding, infection, malnutrition, and halitosis. A large number of m issing teeth and a high incidence of dental caries were additional cha racteristic findings. Such oral self-mutilations were found to decreas e with age and with the intellectual, social, and/or emotional develop ment of the patients. However, not all of the mutilations were complet ely eliminated. Two patients had partial dentures to replace missing t eeth. Conclusions. Our study suggests that early diagnosis and specifi c dental management for patients with hereditary sensory and autonomic neuropathy type IV are important for prevention of the characteristic oral and dental problems accompanying this disorder.