Oral manifestations of hereditary sensory and autonomic neuropathy type IV. Congenital insensitivity to pain with anhidrosis.

OBJECTIVE: Hereditary sensory and autonomic neuropathy type IV (congenital insensitivity to pain with anhidrosis) is a rare disorder. In this study, we investigated the oral and dental manifestations associated with hereditary sensory and autonomic neuropathy type IV.

STUDY DESIGN: Eighteen patients with hereditary sensory and autonomic neuropathy type IV whose ages ranged from 1 year 0 months to 22 years 3 months were examined for oral signs and symptoms of tooth abnormalities, malocclusions, soft tissue disorders, tongue papilla atrophy, and morphologic 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 resultant 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 ventral surface of the tongue, resulting from trauma of the incisal edge of erupting mandibular primary incisors during sucking or nursing. These ulcers led to several local and systemic problems, such as tongue bleeding, infection, malnutrition, and halitosis. A large number of missing teeth and a high incidence of dental caries were additional characteristic findings. Such oral self-mutilations were found to decrease with age and with the intellectual, social, and/or emotional development of the patients. However, not all of the mutilations were completely eliminated. Two patients had partial dentures to replace missing teeth.

CONCLUSIONS: Our study suggests that early diagnosis and specific 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.