A 9-year-old Caucasian female presented to the West Virginia University otolaryngology clinic with the chief complaint of gradually increasing swelling of her upper and lower lips which started 4 months prior. The lip swelling was reported to be very distressing to the child and her parents. Prior to presentation at the otolaryngology clinic, the patient was seen by a pediatric dentist, periodontist, and allergist. The patient had a history of excessive thumb sucking, wearing nail polish, and playing with homemade slime containing glue, saline, shaving cream, and glitter. The patient had a history of mild eczematous skin lesions and vague rhinitis including nasal congestion, occasional postnasal drainage, varying complaints of headaches, and occasional conjunctival pruritus. Seasonal symptoms were worse in spring time, with the pollens of trees, grasses, and weeds possibly playing a role. There was no contributory family history, medication history, or history of allergy to food, drugs, or cosmetics. The patient did not have any contributory past medical or surgical history.
Physical examination revealed a symmetrically enlarged, erythematous upper lip with erythematous and edematous maxillary anterior gingiva, particularly on the right side (Figures 1 and 2). The allergist also noted mild fissures in the lip area, as well as evidence of cheilitis. The patient was prescribed mupirocin ointment for the perioral areas where the cheilitis was present in order to clear any superficial infection. This treatment likely resolved these issues prior to patient presentation at the otolaryngology clinic.
The suggested differential diagnosis was Crohn's disease, sarcoidosis, tuberculosis, angioedema, and orofacial granulomatosis. Routine laboratory tests (basic metabolic panel, complete blood count with differential, erythrocyte sedimentation rate, hepatic function panel, HAV antibody, HCV antibody, HBV envelope antibody and antigen, C-reactive protein, immunoglobulin panel, C4 complement, C3 complement, glomerular basement membrane antibodies, and cytoplasmic neutrophil antibodies) were performed and were reported to be within normal limits. No clinical signs or symptoms of gastrointestinal disease were present.
A right upper lip incisional biopsy was performed. Submucosal lymphohistiocytic infiltrate with loose perivascular granulomas were found. The underlying salivary gland tissue was prominent with lymphocytic infiltrate with occasional germinal center formation. Acid-fast bacilli were not seen on a concentrated smear. The patient's history, physical examination, blood and serum tests, and pathology results led to a diagnosis of OFG of the upper and lower lip with maxillary gingival hyperplasia.
The patient was treated with azithromycin pulses for one month. This treatment consisted of 500 milligrams once daily for 3 days, followed by 4 days of no medication. This regimen was followed for a total of 4 weeks as previously described by De et al. in an effort to avoid corticosteroids given the parents' concern for corticosteroid side effects. The patient saw improvement of symptoms following this treatment. However, after using a lip gloss containing glitter, she experienced an exacerbation of her oral swelling and returned to the clinic. Given the perceived treatment failure, the patient was subsequently treated with 40 milligrams once daily of prednisone for 2 weeks. The patient reported marked improvement following oral steroid administration. Once again, however, the patient experienced an exacerbation of her OFG following swimming. She was treated again with the same regimen of oral steroids. In addition, she avoided foods containing benzoates as well as products containing glitter, both of which seemed to be triggers for her OFG. The patient experienced noticeable improvement in her gingival hyperplasia and lip swelling following the treatment with oral steroids and behavioral modifications.