A case of oro-facial granulomatosis in a paediatric patient
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The present case has been published in the Journal of Pediatrics.

An 8-year-old previously healthy girl presented with acute swelling and scaling of the right side of the face overlying the mandible. She had a 2-year history of intermittent bilateral facial swelling, with prior episodes resolving without treatment in 4-6 weeks. These episodes had not responded to topical corticosteroid or antifungal therapy.

On examination, the patient was afebrile with age-appropriate vital signs. She had firm, nontender facial edema extending from the right oral commissure to the cheek, with overlying red-purple scaling plaques (Figure). The lower lip was swollen and scaling. Gingival hyperplasia, cobblestoning of the nonkeratinized mucosa of the lower lip, and a hyperplastic ridge along the right inferior gingival sulcus were noted in the oral cavity.

The remainder of her examination, including genital mucosa and rectal examinations, was normal. Initial laboratory testing including inflammatory markers was unremarkable and a head and neck ultrasound examination showed subcutaneous edema of the lateral right side of the mouth. The initial diagnosis was infectious cellulitis and the patient received clindamycin and vancomycin intravenously.

With no improvement by day 6, biopsies were obtained from the right buccal mucosa, revealing noncaseating granulomatous inflammation with chronic inflammatory infiltrate consistent with the diagnosis of orofacial granulomatosis. Antibiotics were discontinued, and the patient was discharged on oral prednisone. Upper and lower gastrointestinal endoscopy was macroscopically normal with no histologic findings suggestive of luminal inflammatory bowel disease.

Prednisone led to a slight improvement in swelling and was tapered after 1 month. The patient was started on monthly infliximab infusions, resulting in significant improvement at the 2-month follow-up.

Lessons learnt:-
- The differential diagnosis of facial swelling is broad and includes cellulitis, abscess, fungal infection, angioedema, Sjögren syndrome, atopic dermatitis, parotitis, and orofacial granulomatosis.

- Historical findings pointing toward a diagnosis of orofacial granulomatosis include the persistent nature of the disease and its lack of responsiveness to antibiotic, antifungal, and topical corticosteroid therapy.

- Gingival hyperplasia and cobblestoning of the buccal mucosa can be important clues. Skin biopsy showing granulomas is helpful in determining the etiology of the swelling.

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