Orofacial Granulomatosis in an Orthodontic Patient: a Case Report

Case report

A 14-year-old male was transferred to the Orthodontic department at Addenbrooke's Hospital, Cambridge, from another hospital unit due to his family relocating. He was already undergoing fixed appliance orthodontic therapy to camouflage his Class II division 1 malocclusion (Figures 1a–f). He had extractions of upper first premolars and lower second premolars to aid with overjet reduction, relief of crowding, correction of molar relationship and dental centrelines.

Four months into his treatment he developed persistent swelling of his upper lip (Figure 2a, b). There was also some crusting and scab formation on the upper lip. This continued without improvement and an incisional biopsy was performed. The histology showed the presence of chronic inflammation, a finding that could indicate a number of disease processes, including OFG. The possibility of a delayed hypersensitivity reaction to nickel was considered as the scabbed area was in contact with the upper appliance and so nickel-titanium archwires were removed and replaced with stainless steel archwires. There was no improvement and, with continued discomfort and swelling of the upper lip, the appliance was debonded (Figures 3a–c). Although symptoms improved slightly after removal of the appliance, there was no resolution of the lesion. A referral was made to an Oral Medicine clinic for further investigation.

The diagnosis from the Oral Medicine clinic was infective exfoliative cheilitis superimposed on OFG. The management included a swab of the scabbed lesion which demonstrated infection with candida and coliform organisms. Antifungal and antibiotic agents were prescribed to treat the superimposed infection. A cinnamon-free and benzoate-free diet, and elimination of chocolate, was undertaken by the patient under the guidance of a dietitian. This continued for 12 weeks and when the patient attended for a review at the joint Oral Medicine–Gastroenterology clinic the upper lip swelling had resolved (Figures 4a, b). He has remained symptom free and continues to avoid the causative dietary agents. The patient has decided to delay any further orthodontic treatment, and his treatment will be reconsidered at a later stage.

Discussion

Orofacial granulomatosis is mainly present in young adults; a cohort that is likely to be seen by orthodontists. These patients may present with numerous oral symptoms, thus making it important for clinicians to understand the nature of the condition. Orthodontists may be the first to see the signs and symptoms of the condition and hence they will be required to make an appropriate onward referral to a physician. OFG may be indicative of progression to, or exist concurrently with, Crohn's Disease (CD). Furthermore, some authors suggest that specifically the childhood onset of OFG may be predictive of progression to CD,^8,9 although this has been contradicted in another group.² Other conditions, such as sarcoidosis and tuberculosis, have also been associated with OFG. A thorough family and medical history is required in order to ascertain or eliminate any suggestion that there may be underlying systemic disease linked to the condition.

Orthodontic patients who have had OFG identified in their medical history before the onset of treatment will need careful assessment of whether they will be able to tolerate fixed appliances (Figure 5). A sectional appliance may be used to test the tolerance of the appliance and, if successful, this can progress to a complete fixed appliance. As expected, each case should be treated on an individual basis.

Alternative treatment modalities may also be considered for those patients who are unable to tolerate conventional labial fixed appliances. The use of aligners for mild malocclusions may be considered, if appropriate. For patients who require growth modification, the wireless ‘Newport’ Twin Block appliance may be the appliance of choice (Figures 6a–c). The absence of any components that are likely to traumatize already irritable sites may allow these types of appliances to be better tolerated. Functional appliances can also be modified in order to prevent excessive irritation of the lips and affected mucosa, and due care should be given to components around the affected mucosa when designing the appliance.

Difficulties with wearing fixed appliances may also arise for orthodontic patients developing OFG during treatment. Symptoms of OFG may be similar to a Type IV delayed hypersensitivity reaction to nickel, although this is likely to present within weeks of commencing orthodontic treatment.¹⁰ A Type IV hypersensitivity reaction should be included in the differential diagnosis of upper lip swelling, but there are differences in the epidemiology and presentation of the two conditions (Table 1). In this case, nickel-titanium archwires were replaced with stainless steel ones. Although stainless steel contains nickel, it is thought that there is less nickel released from this alloy.¹¹ Ideally, titanium molybdenum alloy would be the preferred choice if nickel allergy was suspected.

Patch testing may be carried out by a dermatologist if sensitivity to a dental material is suspected. The British Contact Dermatitis Society recommended standard series would include nickel testing, however it would not rule out other common dental materials.¹² Patch testing for food additives may not accurately predict dietary outcome.⁵ Elimination of cinnamon and benzoates in the diet led to a complete resolution of OFG in this case. However, any attempt to do so should be done with consultation from a physician or dietitian.

Conclusion

Orofacial granulomatosis is an uncommon condition that often presents as a persistent lip swelling and may be seen in young patients.

A thorough medical history and appropriate referral is required in order to ensure correct diagnosis, and to rule out any systemic disease that may be associated with this condition.

Patients with OFG may find it difficult to tolerate orthodontic appliances and careful assessment and management is required.