Orofacial manifestations of congenital insensitivity to pain with anhidrosis: a report of 24 cases.
OBJECTIVE
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.
Bodner
Lipa
L
Department of Oral and Maxillofacial Surgery, Soroka Medical Center, Ben Gurion University of the Negev, Beer-Sheva, Israel.
Woldenberg
Yitzhak
Y
Pinsk
Vered
V
Levy
Jacov
J
D018912:Arabs; D001734:Bites, Human / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005383:Finger Injuries / Q000209:etiology; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000150:complications*; D006801:Humans; D007223:Infant; D007557:Israel; D008046:Lip / Q000293:injuries; D008047:Lip Diseases / Q000209:etiology; D008297:Male; D008336:Mandibular Diseases / Q000209:etiology; D009059:Mouth Diseases / Q000209:etiology*; D009061:Mouth Mucosa / Q000293:injuries; D019226:Oral Ulcer / Q000209:etiology; D010019:Osteomyelitis / Q000209:etiology; D012652:Self Mutilation / Q000209:etiology; D014059:Tongue / Q000293:injuries; D014060:Tongue Diseases / Q000209:etiology; D014084:Tooth Avulsion / Q000209:etiology; D014076:Tooth Diseases / Q000209:etiology*; D016388:Tooth Loss / Q000209:etiology