Feras Ghazal, DDS Mohammed Ahmad, MD Hussein Elrawy, DDS Tamer Said, MD Department of Oral Health (Drs. Ghazal and Elrawy) and Department of Family Medicine/Geriatrics (Drs. Ahmad and Said), MetroHealth Medical Center, Cleveland, Ohio tsaid@metrohealth.org
The authors reported no potential conflict of interest relevant to this article.
Persistent idiopathic facial pain (PIFP), previously known as atypical facial pain, is a persistent facial pain that does not have the classical characteristics of cranial neuralgias and for which there is no obvious cause.2,10,23 PIFP is not triggered by any of the factors that typically precipitate neuralgias.2 The onset may be spontaneous or associated with dental intervention or facial injury, but it usually does not have a demonstrable local cause.24,25
Diagnosis of trigeminal neuralgia can be tricky; more than half of patients experience less severe pain after the main sharp attack.
Neuropathic mechanisms that might be at work in PIFP include nociceptor sensitization, phenotypic changes and ectopic activity from the nociceptors, central sensitization possibly maintained by ongoing activity from initially damaged peripheral tissues, sympathetic abnormal activity, alteration of segmental inhibitory control, or hyperactivity or hypoactivity of descending controls.2
PIFP is most frequently reported in women in their 40s and 50s.25 The history of a patient with PIFP often include mood disorders, chronic pain, or poor coping skills.14 Patients complain of a steady, unilateral, poorly localized pain that is deep, constant, aching, pulling, or crushing. It is usually present all day, every day. The constancy of the pain is its distinguishing feature. In the beginning, this pain may be in a limited area on one side of the face, usually the nasolabial folds or the angle of the mandible. Later, it may affect both sides of the face and extend to the neck and upper limbs.23,24 Most patients with PIFP report other symptoms, including headache, neck and backache, dermatitis, pruritus, irritable bowel, and dysfunctional uterine bleeding.26
Making the diagnosis. A targeted history and accurate clinical examination are essential.2,10 Although there are no formal diagnostic criteria, a patient can be assumed to have PIFP if:2,10 • There is pain in the face for most of the day or all day, every day. • Initially, the pain may be confined to a portion of the face, but it is poorly localized and deep. • The pain is not associated with other physical signs or loss of sensation. • Imaging does not reveal an obvious anatomic or structural cause.
Treatment. Treatment of PIFP can be difficult and unsatisfactory.23 Counseling to educate patients about the chronic and nonmalignant nature of the illness is the mainstay of treatment, followed by pharmacotherapy.23 TCAs have shown a moderate effect in several trials. Gabapentin, topiramate, carbamazepine, and pregabalin also have shown limited to modest benefit in some patients. Surgical therapies appear to be of little or no use.23 Experimental treatments such as pulsed radiofrequency, low-energy level diode laser have shown success in small studies.10,23
Vascular pain
Giant cell arteritis (GCA) is a systemic, chronic vasculitis involving the large and medium-sized vessels, mainly the extracranial branches of the carotid artery.6,11 It predominantly affects people older than age 50 and is more common among women and those of Scandinavian ethnicity.27
A distinguishing feature of persistent idiopathic facial pain is that the pain is present all day, every day.
The cause of GCA is unclear. Genetic predisposition linked to humoral and cellmediated immunity is believed to play a role.28 Familial aggregation and predominance of the HLA-DR4 allele has been reported in patients with GCA.6
What you’ll see. The most common signs and symptoms of GCA are temporal headache (seen in two-thirds of patients), jaw claudication and tenderness, and swelling of the temporal artery.6,11 The headache of GCA usually is unilateral, severe, boring or lancinating, and localized to the temporal or occipital regions of the scalp.6 Other orofacial manifestations include trismus, throat pain that develops while chewing, changes in tongue sensation and tongue claudication, tooth pain, dysphagia, dysarthria, submandibular mass, lip and chin numbness, macroglossia, glossitis, lip and tongue necrosis, and facial swelling.11
Visual symptoms include diplopia, ptosis, and possibly blindness if treatment is not instituted at first suspicion. Ocular symptoms result from anterior ischemic optic neuropathy, posterior ischemic optic neuropathy, or central retinal or cilioretinal artery occlusion.6,28 Patients have also reported low-grade fever, asthenia, anorexia, weight loss, and generalized aches.11,28
Making the diagnosis. Arterial biopsy is the gold standard for diagnosis of GCA. It is usually performed on the temporal artery and is positive in 80% to 95% of people with the condition.28 Other useful lab tests include erythrocyte sedimentation rate (ESR; elevated), white blood cell count (mildly elevated), and C-reactive protein (elevated).
Treatment. Prednisone is used to treat GCA, in initial doses ranging from 30 to 80 mg. A maintenance dose may be required for up to 2 years, with close follow-up and periodic ESR measurements.28