(48) Cheilitis Granulomatosa: A Case Review

Abstract: Abstract: Cheilitis granulomatosa (CG) is a rare granulomatous condition characterized by chronic edema of the lips. It may present in isolation (granulomatosa of Miescher) or in a triad with the latter associations being a fissured tongue and facial nerve paralysis (Melkersson-Rosenthal syndrome). An 11-year-old white male presented for evaluation of an isolated recalcitrant edematous upper lip. Swelling began in June of 2017. Orofacial examination demonstrated nontender, globally edematous upper lip with associated cheilitis. Multiple case reports discussing treatment of CG include the use of systemic corticosteroids, topical clobetasol or triamcinolone, as well as the use of dapsone.1 Treatment with 4 courses of local intralesional corticosteroid therapy (Kenalog 10mg/mL) resulted in remission after 3 months. The patient’s condition remains in remission today. This case report highlights an 11-year-old white male who presented with unexplained chronic swelling of the upper lip. The patient did not recall any inciting incident. An allergic reaction and inflammatory bowel disease were excluded by hypersensitivity testing and colonoscopy, respectively. Laboratory testing including autoantibody testing and inflammatory markers were unremarkable. Apart from cracking of the lips the patient had no additional complaints or associated symptoms. At the time of evaluation by oral and maxillofacial surgery, the edematous upper lip was refractory to systemic and topical corticosteroids, as well as non-steroidal anti-inflammatory medications (NSAIDs). Orofacial examination demonstrated nontender, globally edematous upper lip with associated cheilitis. No other notable oral or physical exam findings were discovered. A clinical diagnosis of cheilitis granulomatosa was made. Cheilitis granulomatosa, first described in 1945 by Meischer is a poorly understood disorder consisting, clinically, of chronically edematous lip(s), and a granulomatous infiltrate on histology.2,3,4 When CG manifests as part of a triad, including a fissured tongue and peripheral nerve paralysis it is referred to as Melkersson-Rosenthal syndrome (MRS). CG is the most common subset of MRS. The incidence in the general population is estimated at 0.8%5. The etiology of CG is unknown. Proposed mechanisms include microbial, allergic, and genetic. There have been no clinical trials investigating the treatment of CG. The successes and failures described in case reports and case series guide treatment. Medications including glucocorticoids (systemic, topical, intralesional), immunomodulators (clofazimine, hydroxychloroquine, fumaric acid esters), antibiotics (macrolides, tetracyclines, metronidazole), monoclonal antibodies, immunosuppressants (methotrexate, azathioprine), biologics (anti-tumor necrosis factor alpha [TNF-α]), and laser therapy have been documented in the treatment of GC6. The authors found success with a series of 4 intralesional corticosteroid injections (Kenalog 10mg/mL, 20 units per visit) resulted in remission after 3 months.References1. Sobjanek M, Michajłowski I, Żelazny I, Mędrzycka-Dąbrowska W, Włodarkiewicz A. What is the most effective treatment of cheilitis granulomatosa in Melkersson-Rosenthal syndrome? Journal of the European Academy of Dermatology and Venereology. 2010;24(3):364-365. doi:10.1111/j.1468-3083.2009.03540.x 2. Critchlow WA, Chang D. Cheilitis Granulomatosa: A Review. Head & neck pathology (Totowa, NJ). 2014;8(2):209-213. doi:10.1007/s12105-013-0488-23. Ratzinger G, Sepp N, Vogetseder W, Tilg H. Cheilitis granulomatosa and Melkersson-Rosenthal syndrome: evaluation of gastrointestinal involvement and therapeutic regimens in a series of 14 patients. Journal of the European Academy of Dermatology and Venereology. 2007;21(8):1065-1070. doi:10.1111/j.1468-3083.2007.02194.x4. Brown R, Farquharson A, Cherry-Peppers G, Lawrence L, Grant-Mills D. A case of cheilitis granulomatosa/orofacial granulomatosis. Clinical, cosmetic and investigational dentistry. 2020;12:219-224. doi:10.2147/CCIDE.S2548995. El-Hakim M, Chauvin P. Orofacial granulomatosis presenting as persistent lip swelling: review of 6 new cases. J Oral Maxillofac Surg. 2004;62(9):1114–1117. doi: 10.1016/j.joms.2003.11.013.6. Yashdeep Singh Pathania, Current treatment modalities in granulomatous cheilitis, International Journal of Dermatology, 10.1111/ijd.16126, 61, 6, (755-759), (2022).

Cheilitis Granulomatosa at Presentation
Cheilitis Granulomatosa Mid-treatment
Cheilitis Granulomatosa at Completion of Therapy