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Cheilitis Granulomatosa

Definition

Cheilitis granulomatosa, or Miescher cheilitis, is an uncommon, chronic, noncaseating granulomatous inflammatory disorder.

Etiology

The etiology of cheilitis granulomatosa is unknown, however, a cell-mediated hypersensitivity to foods, food additives, and flavoring agents may play a role in the development of the disease.

Main Clinical Features

Cheilitis granulomatosa may occur as an isolated disorder or as part of other granulomatous diseases, e.g., Melkersson-Rosenthal syndrome. Crohn disease, and sarcoidosis.

•Painless, diffuse swelling of the upper or lower lip or both

•Small vesicles, erosions, and scaling may rarely develop

•Lesions may appear suddenly and have a chronic course with remissions and exacerbations

•Permanent enlargement of the lips may occur

Diagnosis

The clinical diagnosis should be confirmed by a biopsy and histopathologic examination.

Differential Diagnosis

•Cheilitis glandularis

•Melkersson-Rosenthal syndrome

•Crohn disease

•Sarcoidosis

•Orofacial granulomatosis

•Tuberculosis

•Angioedema

•Lymphedema

•Lymphangioma

•Foreign body reaction

Treatment

Basic Guidelines

•Before treatment systemic granulomatous diseases should be excluded.

•Food additives, flavoring agents, some foods, and foreign materials should be ruled out as causative agents.

Suggested Therapies

•Intralesional corticosteroids such as triamcinolone acetonide or betamethasone dipropionate and sodium phosphate retard are recommended as initial therapy. A course of 3-6 intralesional injections may be used.

•Systemic corticosteroids, e.g., prednisone 30-40 mg/day for about 2-3 weeks and then gradually tapered over 1-3 months, may significantly improve the condition.

•Minocycline 100-200 mg/day for 3-6 months in combination with systemic corticosteroids is the best therapeutic regimen.

•Clofazimine, thalidomide, hydroxychloroquine, sulfasalazine, and dapsone have also been used as second-line treatments.

•Plastic surgery reconstruction is indicated in advanced, chronic cases with lip disfiguration.

References

Arbiser JL, Moschella SL, Clofazimine: A review of its medical uses and mechanisms of action. J Am Acad Dermatol 1995;32:241–247.

Rees TD. Orofacial granulomatosis and related conditions. Periodontology 2000 1999;21:145–157.

Ridder GJ, Fradis M, Lohle E. Cheilitis granulomatosa Miescher: Treatment with clofazimine and review of the literature. Am Otol Rhinol Laryngol 2001;110:964–967.

Stein SL, Mancini AJ. Melkersson-Rosenthal syndrome in childhood: Successful management with combination steroid and minocycline therapy. J Am Acad Dermatol 1999;41:746–748.

Thomas P, Walchner M, Ghoreschi K, Rocken M. Successful treatment of granulomatous cheilitis with thalidomide. Arch Dermatol 2003;139:136–138.

Van der Waal RI, Suhulten EA, van der Meij EH, et al. Cheilitis granulomatosa: Overview of 13 patients with long-term follow-up results of management. Int J Dermatol 2002;41:225–229.

Veller FC, Catalano P, Peserico A. Minocycline in granulo-matous cheilitis: Experience with 6 cases [letter]. Dermatology 1992;185:220.