Granulomatous cheilitis

Management Strategy

The treatment of granulomatous cheilitis is very challenging. Because the etiology is unknown, a variety of therapeutic strategies with varying degrees of success have been attempted. No randomized clinical trials or comparative studies have been performed. Given the evanescent nature of the condition, treatment outcomes are difficult to assess. Spontaneous remission of granulomatous cheilitis rarely occurs, further confounding assessment of therapies. Furthermore, a recent retrospective study demonstrated long-term treatment with topical medications and/or combination therapy may be required.

The aim of treatment is to prevent permanent labial deformity. Conservative measures for acute granulomatous cheilitis involve symptomatic relief, including cold compresses and oral antihistamine s to reduce erythema and ointments to protect against fissuring of the lips. Initial therapy frequently includes corticosteroids, either topical, intralesional, or systemic. Initial topical therapy may include either triamcinolone or clobetasol (compounded into Orabase). Intralesional steroids may be used in doses ranging from 10 to 40 mg/kg. Nerve blocks may reduce patient discomfort in cases where large volumes at low concentrations are needed. Although short courses of prednisone (25–50 mg/day) will frequently improve tissue swelling, flares are often noted on cessation.

Clofazimine has been reported to treat granulomatous cheilitis successfully. Response to therapy has been variable. Possible side effects include transient orange-pink discoloration of the skin, nausea, and vomiting. Fatal enteropathy may occur but only at higher doses than those recommended for granulomatous cheilitis treatment . Thalidomide can also be considered, especially in refractory cases. Patients must be monitored for the development of peripheral neuropathy and warned of its teratogenicity. Monotherapy with metronidazole , tetracyclines , azithromycin , hydroxychloroquine , or sulfasalazine , although less well substantiated, may be attempted. These medications may be used with corticosteroids. The addition of minocycline 100 mg twice daily or tetracycline 500 mg daily may prevent rebound after prednisone discontinuation. The value of dapsone and topical tacrolimus in the treatment of granulomatous cheilitis is unclear. Biologics, including infliximab and adalimumab, have also been successful treatments.

Granulomatous cheilitis may become persistent. Patients who suffer from permanent esthetic deformity or functional impairment may benefit from cheiloplasty. Surgical intervention should be performed only when more conservative approaches have failed and when inflammation is quiescent. An important consideration for Melkersson–Rosenthal syndrome patients undergoing anesthesia for surgical intervention or any surgical procedures is the risk of urticarial reaction due to anesthetic triggers during the procedure. In the past, remission was maintained with the use of postoperative corticosteroid injections. More recent reports, however, have described long remissions after surgery, with no additional treatment needed.

Granulomatous cheilitis has characteristics that are associated with several conditions that may require evaluation, including Crohn disease, sarcoidosis, infections (tuberculosis, leprosy, deep fungal, human immunodeficiency virus (HIV), and syphilis), acquired or hereditary angioedema, cheilitis glandularis, and leukemic infiltrates. It is also important to rule out any provocative cause such as odontogenic infections or allergenic sensitizers. Orofacial granulomatosis is associated with Crohn disease, especially in children, and may precede even minimal gastrointestinal symptoms.

Initial biopsies of granulomatous cheilitis may reveal dilated lymphatic channels, non-specific inflammatory infiltrates, and edema. In the later stages, the classic non-caseating granulomas are found. Absence of granulomata on biopsy does not exclude the diagnosis of granulomatous cheilitis. Granulomatous cheilitis is primarily a clinical diagnosis.