Introduction Orofacial granulomatosis (OFG) is a rare chronic inflammatory disease of unknown aetiology sharing histological features with gut Crohn's disease (CD). A notes review of 207 patients with OFG aimed to define the common presentation of this condition and establish differentiating features between those with and without concurrent diagnosis of CD.
Methods Data were extracted for age of onset, gender, clinical features, blood parameters, concurrent CD and treatments used. Diagnosis of CD was established by standard criteria.
Results Ninety-seven out of 207 patients (47%) were female. Median age of disease onset was 23 years (range 2–73 years). Referrals were mainly sourced from maxillo-facial surgeons (31%) and gastroenterologists (19%). The buccal mucosa (74%) and lower lip (68%) were the most common sites involved followed by gingivae (63.5%) and upper lip (61%). Forty-six (22%) had CD. Ulcers (46% vs 15%, p<0.001) and mucosal scarring (20% vs 5%, p<0.001) were more common in patients with CD than in those without as was a raised C reactive protein (73% vs 49%, p=0.016), abnormal full blood counts (46% vs 23%) and low haemoglobin (31% vs 11%). The sulcus (27% vs 13%, p=0.019) and fauces (4% vs 0%, p=0.008) were significantly more likely to be affected in those with CD.
Of the patients with concurrent CD, half were diagnosed with this prior to onset of OFG symptoms. Conversely 42.5% had OFG symptoms prior to diagnosis of CD. The remaining patients (7.5%) presented with symptoms and were diagnosed with CD within the same year. The predominant treatment used (86%) was the cinnamon and benzoate free diet. Topical treatments including antifungal and steroidal creams, ointments, mouthwashes and intralesional steroid injections were used in 64% of cases. Azathioprine was used in 39% of patients and anti TNF α therapy in 7.5% of patients. Only 3% of patients required cheiloplasty.
Conclusion OFG most commonly presents with buccal and lower lip involvement. Abnormalities in inflammatory markers, haematinic deficiencies and oral presentation of ulceration and scarring are all factors which can increase the likelihood of concurrent CD. Initial presentation of OFG is not necessarily predictive of further development of CD.
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