Orofacial manifestations of congenital insensitivity to pain with anhidrosis: a report of 24 cases.

Purpose: To report the incidence and severity of the oral and dental manifestations associated with congenital insensitivity to pain with anhidrosis (CIPA).

Methods: Young children with CIPA underwent orofacial examination. The tongue, lips, and buccal mucosa were examined for soft tissue disorder. Missing and luxated teeth were recorded.

Results: Twenty four patients (14 males and 10 females, mean age 60 months, range 9-144 months) with CIPA showed moderate to severe self-mutilation. Oral self-mutilation, such as biting injuries and scarring of soft tissues (tongue, lip, and buccal mucosa) were found in all patients. Fingertip biting was also found in most patients. Among infant patients, the mutilation was typically characterized by decubital ulcers of the tongue. Many edentulous areas due to previously extracted teeth were also found.

Conclusions: Early diagnosis and specific dental care for patients with CIPA can be helpful in prevention of the fingertip biting and orofacial manifestations seen in this disorder.