Orofacial granulomatosis successfully treated with mycophenolate mofetil
Authors: Albert S. Antonyan, Venessa Pena-Robichaux, Chauncey A. McHargue
Journal: Journal of the American Academy of Dermatology (2014)

To the Editor: Orofacial granulomatosis (OFG) is a chronic, relapsing, potentially disfiguring inflammatory disorder of unknown etiology.1Mignogna M.D. Pollio A. Leuci S. Ruoppo E. Fortuna G. Clinical behavior and long-term therapeutic response in orofacial granulomatosis patients treated with intralesional triamcinolone acetonide injections alone or in combination with topical pimecrolimus 1%.J Oral Pathol Med. 2013; 42: 73-81Crossref PubMed Scopus (15) Google Scholar It is characterized by persistent enlargement of the orofacial tissues and noncaseating granulomatous inflammation on tissue biopsy in the absence of diagnosable Crohn disease or sarcoidosis.1Mignogna M.D. Pollio A. Leuci S. Ruoppo E. Fortuna G. Clinical behavior and long-term therapeutic response in orofacial granulomatosis patients treated with intralesional triamcinolone acetonide injections alone or in combination with topical pimecrolimus 1%.J Oral Pathol Med. 2013; 42: 73-81Crossref PubMed Scopus (15) Google Scholar, 2Grave B. McCullough M. Wiesenfeld D. Orofacial granulomatosis – a 20-year review.Oral Dis. 2009; 15: 46-51Crossref PubMed Scopus (137) Google Scholar, 3Al Johani K.A. Moles D.R. Hodgson T.A. Porter S.R. Fedele S. Orofacial granulomatosis: clinical features and long-term outcome of therapy.J Am Acad Dermatol. 2010; 62: 611-620Abstract Full Text Full Text PDF PubMed Scopus (75) Google Scholar It is thought that OFG represents a spectrum of disease ranging from localized granulomatous inflammation of the lips (cheilitis granulomatosa, Miescher cheilitis) to persistent lip or facial swelling with mucosal ulceration, recurrent facial paralysis, and lingual fissuring (Melkersson-Rosenthal syndrome).2Grave B. McCullough M. Wiesenfeld D. Orofacial granulomatosis – a 20-year review.Oral Dis. 2009; 15: 46-51Crossref PubMed Scopus (137) Google Scholar, 3Al Johani K.A. Moles D.R. Hodgson T.A. Porter S.R. Fedele S. Orofacial granulomatosis: clinical features and long-term outcome of therapy.J Am Acad Dermatol. 2010; 62: 611-620Abstract Full Text Full Text PDF PubMed Scopus (75) Google Scholar Due to its unknown etiology and unpredictable clinical course, no definitive pharmacologic therapy is yet available.1Mignogna M.D. Pollio A. Leuci S. Ruoppo E. Fortuna G. Clinical behavior and long-term therapeutic response in orofacial granulomatosis patients treated with intralesional triamcinolone acetonide injections alone or in combination with topical pimecrolimus 1%.J Oral Pathol Med. 2013; 42: 73-81Crossref PubMed Scopus (15) Google Scholar, 2Grave B. McCullough M. Wiesenfeld D. Orofacial granulomatosis – a 20-year review.Oral Dis. 2009; 15: 46-51Crossref PubMed Scopus (137) Google Scholar We present a case of OFG successfully treated with mycophenolate mofetil (MMF), suggesting this treatment may be of benefit in these patients. A 59-year-old Caucasian woman with a history of systemic lupus erythematosus, hepatitis C, and recurrent herpes labialis presented with a 4-month history of rash and swelling of her upper lip. The lesion started as a “cold sore” with occasional burning and mild pruritus. She was treated with oral steroids and amoxicillin by her primary care physician with temporary relief and was referred for further evaluation by a dermatologist. Her condition was persistent with erythema, mild scaling, and diffuse swelling of the upper lip extending to the right oral commissure and lower lip. No other oral mucosal or lingual lesions were noted and she denied any constitutional, respiratory, or gastrointestinal symptoms. A biopsy of her upper lip revealed chronic dermal inflammation with focal granuloma formation (Fig 1). Gram, Fite, and periodic acid–Schiff stains were negative. Complete blood count, serum angiotensin-converting enzyme, C-reactive protein, tuberculin skin test, chest radiographs, funduscopic examination, and prior colonoscopy were all unremarkable. Based on these findings, OFG was diagnosed. High-potency topical steroids, valacyclovir, and doxycycline yielded no improvement. Systemic steroids were discussed as a treatment option but were contraindicated due to history of avascular necrosis of the hip, and she refused intralesional steroid injections. She was started on MMF 500 mg twice daily with significant improvement after 1 month and near complete return to baseline orofacial structural anatomy after 6 months of therapy (Fig 2). At 1 year of MMF monotherapy, she continues to be disease-free with no clinical or laboratory adverse effects of treatment. MMF is a potent immunosuppressant widely described in the dermatology literature for its attractive safety profile and effectiveness in a variety of inflammatory skin conditions.4Zwerner J. Fiorentino D. Mycophenolate mofetil.Dermatol Ther. 2007; 20: 229-238Crossref PubMed Scopus (75) Google Scholar Two patients with OFG treated with MMF in combination with topical therapy (corticosteroids and/or tacrolimus) have been reported, although with unclear protocols and outcomes.3Al Johani K.A. Moles D.R. Hodgson T.A. Porter S.R. Fedele S. Orofacial granulomatosis: clinical features and long-term outcome of therapy.J Am Acad Dermatol. 2010; 62: 611-620Abstract Full Text Full Text PDF PubMed Scopus (75) Google Scholar A search of PubMed and Google Scholar using the terms “mycophenolate mofetil” and “orofacial granulomatosis,” “cheilitis granulomatosa,” “Miescher cheilitis,” or “Melkersson Rosenthal syndrome” showed no previous reports of MMF used as a single-therapy agent in the successful treatment of OFG. MMF may be a beneficial treatment option for these patients, particularly in those who cannot tolerate systemic or intralesional corticosteroid therapy.