Granulomatous cheilitis

Published on 18/03/2015 by admin

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Granulomatous cheilitis

Charlene Lam and Bryan E. Anderson

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

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Granulomatous cheilitis is the painless swelling of one or both lips, with histologic evidence of non-caseating granulomatous inflammation. Granulomatous cheilitis exists along the spectrum of orofacial granulomatosis which encompasses localized disease (granulomatous cheilitis, Miescher cheilitis) to multisystem disease (Melkersson–Rosenthal syndrome). Melkersson–Rosenthal syndrome describes the triad of recurrent orofacial edema, recurrent facial nerve palsy, and lingua plicata (fissured tongue). Granulomatous cheilitis and Melkersson–Rosenthal syndrome may represent separate diseases, but many investigators consider isolated granulomatous cheilitis to be a monosymptomatic form of Melkersson–Rosenthal syndrome.

Management strategy

The treatment of granulomatous cheilitis is very challenging. As the etiology of Melkersson-Rosenthal syndrome/granulomatous cheilitis 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 waxing-and-waning nature of the condition, treatment outcomes are difficult to assess. Rarely, spontaneous remissions of granulomatous cheilitis may occur, 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 for granulomatous cheilitis is directed at preventing permanent labial deformity. Conservative measures for acute granulomatous cheilitis involve symptomatic relief including cold compresses and oral antihistamines for reducing 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. Owing to the need for increased volumes injected at lower concentrations, nerve blocks may be necessary to reduce patient discomfort. Although short courses of prednisone 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 be used. Patients must be monitored for the development of peripheral neuropathy and warned of its teratogenicity. Monotherapy with metronidazole, tetracyclines, penicillin, erythromycin, ketotifen, 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.

The newest successful therapies include infliximab and adalimumab. It has been suggested that infliximab is more effective than adalimumab, but too few cases have been reported to render definitive conclusions.

Granulomatous cheilitis may become persistent. Patients who suffer from permanent aesthetic 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’s disease, sarcoidosis, infections (tuberculosis, leprosy, deep fungal, HIV, and syphilis), acquired or hereditary angioedema, cheilitis glandularis, and leukemic infiltrates. Orofacial granulomatosis is associated with Crohn’s disease especially in children and may precede gastrointestinal symptoms. The signs of symptoms of Crohn’s disease may be minimal, necessitating a complete physical examination and continued observation and surveillance of the patient. Furthermore, it is also important to rule out any provocative cause such as odontogenic infections or allergenic sensitizers.

Specific investigations

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.

Standard patch testing, as well as metal, bakery, dental, and other patch series may be required.

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