OBSERVATION Gingival Manifestations of Orofacial Granulomatosis Sı´lvia V. Lourenc¸o, DDS, PhD; Alice Z. C. Lobo, MD; Paula Boggio, MD; Fernando Fezzi, DDS; Alexandre Sebastia˜o, DDS; Marcello Menta S. Nico, MD Background: Orofacial granulomatosis is a clinical en- gival biopsy. Histologic examination of all the gingival tity presenting with swelling of the facial and/or oral tis- biopsy specimens showed noncaseating granulomas, sues in association with histologic evidence of noncase- edema of the superficial lamina propria, and a chronic ating granulomatous inflammation. Labial swelling is the inflammatory infiltrate consisting predominantly of lym- most common finding. Compromise of the gingival and phocytes and multinucleated giant cells. Treatment op- periodontal tissues may occur but has rarely been de- tions included anti-inflammatory therapy associated with scribed in the literature. Our objective was to character- periodontal care. ize granulomatous gingivitis in patients with orofacial granulomatosis. Conclusion: Gingival tumefaction with histologic evi- dence of granulomatous inflammation may occur in oro- Observations: The study included 29 cases of orofa- facial granulomatosis and might be more common than cial granulomatosis seen in our clinic between January reported in the literature. 1, 1989, and December 31, 2006. Of these 29, 5 had clini- cal evidence of gingival tumefaction and underwent gin- Arch Dermatol. 2008;144(12):1627-1630 HE TERM OROFACIAL GRANU- ogy clinic and to present a detailed de- lomatosis describes a clini- scription of clinical and histopathologi- cal entity presenting with cal features of this particular presentation. swelling of the facial and/or oral tissues in association METHODS Twith histologic evidence of noncaseating granulomatous inflammation. This clas- sification includes idiopathic disorders The study included 29 patients with orofacial such as Melkersson-Rosenthal syndrome granulomatosis (14 females and 15 males), ex- amined between January 1, 1989, and Decem- and Miescher chronic granulomatous chei- ber 31, 2006, in the Oral Diseases Clinic of the litis, as well as the localized orofacial pre- Department of Dermatology, University of Sa˜o sentations of Crohn disease and sarcoid- Paulo. Patients included in the study had no osis.1-4 sign of disseminated granulomatous disease. All This condition has no sex or racial pre- patients underwent biopsy and, in those pre- dilection and the age at onset is highly vari- senting with clinical gingival changes, a gin- able, being more frequent in the second gival biopsy was also performed. Biopsy speci- and third decades of life. Orofacial swell- mens were fixed in 10% buffered formalin and ing may be intermittent initially but tends embedded in paraffin. Sections were stained to become chronic with fluctuations in se- with hematoxylin-eosin for histologic exami- nation and with the histochemical techniques verity. It may also affect various sites or of periodic acid–Schiff, acid-fast bacilli, and be oligosymptomatic. Labial swelling is the Grocott for detection of infectious microor- most common finding, and it may in- ganisms. volve one or both lips, often in an asym- metrical manner.2,5,6 Specific gingival mani- festations (granulomatous gingivitis) have RESULTS Author Affiliations: rarely been described in the literature but Department of General may occur in 21% to 26% of patients.1,5,7-9 Gingival-specific manifestations were Pathology, Dental School Detailed descriptions of these manifesta- detected in 5 of the 29 patients with oro- (Dr Lourenc¸o), and Department tions are scarce, with few publications deal- facial granulomatosis examined. Their of Dermatology, Medical School 8 (Drs Lourenc¸o, Lobo, Boggio, ing with this issue. clinical characteristics and outcome are Fezzi, Sebastia˜o, and Nico), The objective of this study was to de- summarized in the Table. The Figure University of Sa˜o Paulo, Sa˜o tect gingival manifestations in patients with illustrates the clinical and histologic Paulo, Brazil. orofacial granulomatosis in a dermatol- findings. (REPRINTED) ARCH DERMATOL/ VOL 144 (NO. 12), DEC 2008 WWW.ARCHDERMATOL.COM 1627 ©2008 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Table. Patients With Orofacial Granulomatosis Manifesting Granulomatous Gingivitis Patient No. Characteristic 1 (Figure, A and B) 2 (Figure, C and D) 3 (Figure, E and F) 4 (Figure, G and H) 5 (Figure, I and J) Patient age, y/sex 28/F 18/F 52/M 24/F 13/M Duration of symptoms, y 33423 Specific clinical Upper lip edema Upper lip edema Upper and lower lip Upper and lower lip Upper lip edema manifestation edema edema Gingival manifestation Gingival hyperplasia Marked hyperplasia of Marked hyperplasia of Marked hyperplasia of Marked hyperplasia of upper anterior upper and lower upper anterior upper anterior gingivae including gingivae, gingivae including gingivae including interdental papillae abscesses, and interdental papillae interdental papillae fistulas; severe periodontal disease and tooth mobility Tongue manifestation No No Yes (plicated and Yes (geographic No geographic tongue) tongue) Facial palsy No No No No No Treatment Dapsone (100 mg/d), Dapsone (100 mg/d), Dapsone (100 mg/d), Dapsone (100 mg/d), Dapsone (100 mg/d), intralesional intralesional oral prednisone (40 intralesional intralesional corticosteroid corticosteroid mg/d), intralesional corticosteroid corticosteroid infiltration (lip), infiltration (lip), corticosteroid infiltration (lip) infiltration (lip), and gingivoplasty and gingivoplasty infiltration (lip), and gingivoplasty and gingivoplasty Outcome Mild improvement Good improvement Good improvement Good improvement Good improvement (lost to follow-up (2-y follow-up) (3-y follow-up) (2-y follow-up) (1-y follow-up) after 1 y) None of the patients had signs or symptoms of sys- orofacial swelling and histologically by noncaseating epi- temic disease. The histologic findings in the lip biopsy thelioid granulomas.1 The term orofacial granulomatosis specimens of the 29 patients examined included edema is nonspecific and is used to encompass clinical and his- and lymphocytic diffuse and perivascular inflammatory tologic features of more than 1 underlying etiology.1-3 infiltrate. In most specimens, loose and well-developed Melkersson-Rosenthal syndrome is the triad of recur- noncaseating granulomas were detected. The histopatho- rent or persistent orofacial swelling, peripheral facial nerve logical appearance of the 5 gingival biopsy specimens paralysis, and plicated tongue.5,7 However, few patients showed edema of the superficial lamina propria and an present with the complete syndrome. Hornstein2 re- infiltrate of chronic inflammatory cells consisting pre- ported that only 6 of 73 patients (8%) showed the com- dominantly of lymphocytes and multinucleate giant cells, plete triad. Cheilitis granulomatosa is the most frequent forming noncaseating granulomas (Figure, K and L). No finding and may occur as an isolated feature. Fissured birefringent foreign material was identified by polarized tongue (or plicated tongue) is not considered a pathog- light microscopy. Special histochemical stains detected nomonic sign of Melkersson-Rosenthal syndrome be- no fungi or acid-fast bacilli. cause it is common in the general population, and facial Complete blood cell count and results of kidney and nerve paralysis is usually of sudden onset and clinically liver function tests were normal in all patients. Chest ra- indistinguishable from Bell palsy.2,4 Several other symp- diographs were unremarkable. The diagnosis of orofa- toms, including intraoral, neurologic, ophthalmologic, cial granulomatosis with gingival involvement was es- and otologic manifestations, have been described in as- tablished on the basis of the clinical-histologic correlation sociation with orofacial granulomatosis/Melkersson- in the 5 cases presented. No patient was using any medi- Rosenthal syndrome.2,4,8 cations that could cause gingival hyperplasia; thus, the Among our initial cohort of 29 patients with orofa- possibility of drug-induced gingival hyperplasia was not cial granulomatosis, 5 (17%) presented with specific gin- considered. gival lesions. We found a wide range of gingival aspects Therapeutic modalities used included anti- in these cases. One patient (case 4) had mild gingival in- inflammatory drugs (intralesional and oral corticoste- volvement with edema of interdental papillae, and pa- roids and dapsone). Additional periodontal treatment was tients 2 and 5 had a diffuse infiltration. Two patients performed in all 5 cases of granulomatous gingivitis showed massive gingival enlargement (cases 1 and 3), (Table). Response to treatment was not uniform and associated with bleeding. In addition, patient 3 showed ranged from modest to almost complete improvement. periodontal fistulas and tooth mobility. All of these cases were confirmed to be due to specific infiltration of non- COMMENT caseating granulomas. Only a few studies describing gingival manifesta- Orofacial granulomatosis is a descriptive term used for a tions of orofacial granulomatosis/Melkersson- broad group of disorders affecting soft tissues of the face Rosenthal syndrome are available in the literature, espe- and oral cavity. It is characterized clinically by chronic cially regarding histologically confirmed granulomatous (REPRINTED) ARCH DERMATOL/ VOL 144 (NO. 12), DEC 2008 WWW.ARCHDERMATOL.COM 1628 ©2008 American Medical Association. All rights reserved.