Journal of Interdisciplinary Dentistry

CASE REPORT
Year
: 2021  |  Volume : 11  |  Issue : 1  |  Page : 27--31

Interdisciplinary approach in the diagnosis and treatment of orofacial granulomatosis


Chandulal Dhalkari, Sayali Chandrakant Patil, Maya Indurkar 
 Department of Periodontology and Implantology, Maharashtra University of Health Sciences, Nashik; Government Dental College and Hospital, Aurangabad, Maharashtra, India

Correspondence Address:
Dr. Sayali Chandrakant Patil
Government Dental College and Hospital, Aurangabad, Maharashtra - 431001
India

Abstract

Orofacial granulomatosis (OFG) is an uncommon disease and represents an idiopathic, nonnecrotizing granulomatous lesion of soft tissue in the orofacial region. Medical history is very important in the diagnosis of OFG as medical condition such as Crohn's disease can also present an extraintestinal manifestation of OFG. Exclusion of other entities such as sarcoidosis, myocardial infections, foreign body reactions, and biopsy helps establish a correct diagnosis. The term idiopathic OFG is used in cases with unknown etiology. This case report describes idiopathic OFG in a 35-year-old female patient with persistent upper lip swelling and gingival enlargement. Management with intralesional acetonide 20 mg/ml, twice week for 3 weeks and gingivectomy, resulted in significant remission without recurrence after a 6-month follow-up.



How to cite this article:
Dhalkari C, Patil SC, Indurkar M. Interdisciplinary approach in the diagnosis and treatment of orofacial granulomatosis.J Interdiscip Dentistry 2021;11:27-31


How to cite this URL:
Dhalkari C, Patil SC, Indurkar M. Interdisciplinary approach in the diagnosis and treatment of orofacial granulomatosis. J Interdiscip Dentistry [serial online] 2021 [cited 2021 Sep 17 ];11:27-31
Available from: https://www.jidonline.com/text.asp?2021/11/1/27/314179


Full Text



 Clinical Relevance to Interdisciplinary Dentistry



Rare gingival finding (gingival enlargement) associated with orofacial granulomatosis.

Interdisciplinary approach is the key to successful management of such cases. In this case the periodontist and the dermatologist formed a liaison to the effective treatment.

 Introduction



Wiesenfeld in 1985 introduced orofacial granulomatosis (OFG), which comprises a group of disease characterized by nonnecrotizing, noncaseating granulomatous inflammation of soft tissues in the orofacial region.[1] OFG is a nonspecific granulomatous inflammation presenting as facial or lip swelling, cheilitis, ulcerations, gingival enlargement, mucosal tags, and sometimes regional lymphadenopathy. Frequent expression of human leukocyte antigens among patients with OFG suggests autosomal dominant transmission with incomplete penetration and translocation at chromosome 9p11, thus signifying a genetic predisposition.[2] The involvement of microbial agents in the etiology of OFG has been suggested on the basis of implication of microorganism, especially bacteria, in similar granulomatous conditions such as Crohn's disease, sarcoidosis, and tuberculosis. However, a concrete causative microbial relationship has not been associated with OFG.[3] Although various theories have been associated with OFG, including genetic predisposition, infection, and allergy, the exact etiopathogenesis is still unknown.

The classic presentation of OFG is recurrent, persistent labial swellings, and the most common site of involvement is the lips, followed by the tongue, gingival, and buccal mucosa.[4] Initially, on palpation, the swelling appears soft, nontender, and nonpitting and later becomes rubbery and firm in nature. Other oral manifestations are oral ulcers, mucosal tags, submucosal swellings, fissured tongue, angular cheilitis, gingival enlargement, facial erythema and swelling, facial nerve palsy, and lymphadenopathy.[4]

 Case Report



A female patient, aged 35 years, reported to the Department of Periodontology and Oral Implantology, Government Dental College and Hospital, Aurangabad, Maharashtra, India, in April 2017, with the complaint of gingival swelling in the maxillary anterior region for the past 6 months and lip swelling for the past 2 years [Figure 1] and [Figure 2]. There were no associated symptoms, but a sense of disfigurement persisted. The patient had lip swelling for the past 2 years and consulted a dermatologist, who prescribed cetirizine. Lip swelling persisted and was cosmetic problem for the patient.{Figure 1}{Figure 2}

After obtaining written informed consent from patient, we started with further examination and treatment.

The detailed dental, medical, family history and history for allergy were noncontributory. A general physical examination of the patients was normal with no evidence of systemic disease. The patient was moderately built and well-nourished.

On extraoral examination, no lymphadenopathy was found, and there was no sign of dysphasia. The upper lip swelling was distinctly edematous, everted, and reddish in color. On palpation, it was soft, edematous, and nontender. Intraoral examination revealed diffuse gingival enlargement of the maxillary facial aspect involving interdental papilla extending up to the coronal third of the crown (grade 2).[5] Gingival consistency was soft and edematous with generalized loss of scalloping. No substantial changes were found on the dorsum of the tongue. An OPG was taken to rule out any bony involvement.

A provisional diagnosis of OFG was established based on history and clinical features. Differential diagnosis of inflammatory enlargement, angioneurotic edema, Melkersson–Rosenthal syndrome, cheilitis granulomatosa, sarcoidosis, tuberculosis, and Crohn's disease was considered. A complete hemogram including erythrocyte sedimentation rate (ESR), sputum analysis, C1 esterase inhibitor assay, serum level of angiotensin-converting enzyme, and a chest radiograph was done.

C1 esterase inhibitor assay obtained was 0.27 g/L within normal limits (0.21–0.39 g/l) ruling out angioneurotic edema. Melkersson–Rosenthal syndrome, is characterized by the triad of persistent lip or facial swelling, fissured tongue and recurrent facial paralysis. Clinically normal dorsal surface of tongue and absence of facial paralysis ruled out Melkersson–Rosenthal syndrome, The chest radiographs did not showed hilar lymphadenopathy and negative sputum analysis Based on clinical, microbiological and radiographic interpretations, tuberculosis was ruled out. The results obtained from the angiotensin converting assay revealed a value of 39U/L (normal range: 8–52U/L). Thus, sarcoidosis was ruled out. Useful evaluations for differentiation of OFG and Crohn's disease include erythrocyte sedimentation rate (ESR), complete blood count (CBC), serum folic acid, iron, vitamin B12 and gastrointestinal evaluation including endoscopy and biopsy. In the present case on medical examination there were no abdominal symptoms and both the ESR and C-reactive protein levels were within normal limits. Biopsy of the terminal ileum was also done to rule out Crohn's disease.

An incisional biopsy was done on the maxillary labial mucosa. Histopathology picture showed keratinized stratified squamous epithelium with connective tissue picture of perivascular and paravascular pool of inflammatory cells and focal noncaseating granulomas in a patchy pattern.

Final diagnosis of idiopathic OFG was made, after correlating both the clinical and histopathologic findings.

Case management

Treatment of the case consisted of,

Emergency phase: Not requitedPhase 1 therapy: Scaling and root planing, and oral hygiene instructions. (The patient was advised to rinse with 10 ml of 0.2% chlorhexidine mouthwash, 1:1 dilution, twice daily for 2 weeks) [Figure 3],On re-evaluation after 1 month, it was found that the clinical appearance and consistency of the gingival enlargements had not changed and had not reduced in size or severityPhase 2 therapy: After obtaining written informed consent, a surgical excisional procedure (gingivectomy) of the right and left maxillary labial arch and labial biopsy was carried out in the same visit [Figure 4] and [Figure 5]Phase 3 therapy: Not requiredMaintenance therapy: Gingival enlargement of the maxillary labial segment was reduced [Figure 5].{Figure 3}{Figure 4}{Figure 5}

The patient was advised to avoid common allergic food substances.

Management of recurrent lip swelling protocol began with intralesional injections 0.1 ml of triamcinolone acetonide (20 mg/ml) given at three equidistant points on the upper lip on the vermillion border. Care was taken to inject triamcinolone acetonide deep into the musculature to avoid lip skin ischemia. The dose was given at an interval of 3 days for 3 weeks, resulting in significant resolution of the condition.

The patient is being reviewed regularly, and no recurrent swelling has appeared [Figure 6].{Figure 6}

 Discussion



OFG is nonspecific granulomatous inflammation presenting as facial or lip swelling, cheilitis, ulcerations, gingival enlargement, mucosal tags, and sometimes lymphadenopathy. It is so named because during microscopic examination of affected swollen tissue, small collections of inflammatory cells, called granulomas, are often found [Figure 7].{Figure 7}

Although several theories have been advanced regarding the etiology of OFG, the exact cause remains widely debated and largely unknown.[2],[3] The diagnostic dilemma may be further complicated by the fact that OFG may be the oral manifestation of a systemic condition, such as sarcoidosis, Crohn's disease, Wegener granulomatosis, intraoral tuberculosis, or a food allergy.[3] In most of these conditions, there are very few distinguishing local signs and symptoms, so they create a diagnostic as well as a therapeutic problem for the physician. These local and systemic conditions, which are characterized by granulomatous inflammation, may be differentiated by appropriate history, clinical examination, and laboratory investigations. Crohn's disease is an intestinal inflammatory disease characterized by granulomatous inflammation of the GI tract.[6] There is apthous like lesions, small mucosal pustules and cobblestone like mucosal overgrowth. Useful evaluations for differentiation of OFG and Crohn's disease include ESR, complete blood count, serum folic acid, iron, Vitamin B12, and GI evaluation, including an endoscopy and biopsy.[7] In the present case, on medical examination, there were no abdominal symptoms, and both the ESR and C-reactive protein levels were within normal limits. Biopsy of the terminal ileum was also carried out to rule out Crohn's disease. In sarcoidosis, there is classic swelling in the cervical lymph nodes affecting young to middle-aged adults.[8] In the oral cavity, the most affected sites are salivary glands. Since no lymphadenopathy was seen, sarcoidosis was ruled out. Melkersson–Rosenthal syndrome is characterized by the triad of persistent lip or facial swelling, fissured tongue, and recurrent facial paralysis.[9] A clinically normal dorsal surface of the tongue and an absence of facial paralysis ruled out Melkersson–Rosenthal syndrome. The present case described no elevation of ESR, and there was no evidence of upper respiratory tract involvement, which occurs in about 90% of cases of Wegener granulomatosis.[10] Intraoral tuberculosis is rare and constantly accompanied by a primary lesion in the lung or elsewhere.[11] The chest radiographs did not show hilar lymphadenopathy, and the serum level of angiotensin converting enzyme was not elevated. Based on clinical and radiographic interpretations, tuberculosis was ruled out. On the basis of negative results of a thorough investigation, a final diagnosis of OFG was made.

The management of OFG is difficult with frequent recurrences despite different treatment modalities applied. Medical treatment includes administration of antimalarial (hydroxychloroquine), antitubercular, and antilepromatous agents (clofazimine); tumor necrosis factor-alpha; infliximab; steroids; and thalidomide.[12] However, corticosteroids are effective in alleviating facial swelling and are thus considered the drug of choice for the treatment. Due to the relapsing nature of OFG, use of systemic steroids has limitations because of side effects. An intralesional corticosteroid injection of triamcinolone acetonide (20 mg/mL) has been proven to be effective and beneficial.[12] In major cases, where severe lip deformation is present, cheiloplasty can be done.

The potential side effects of corticosteroid such as temporary ischemia, hypopigmentation, and soft tissue atrophy may cause cosmetic problems. Thus, path of needle insertion should be directed orally. In the present case, intralesional injections of 0.1 ml triamcinolone acetonide (20 mg/ml) were given into each of the three equidistant points on the border between upper lip vermillion and mucosa. The needle was inserted vertically, and in order to avoid lip ischemia, the same treatment modalities used for the idiopathic OFG with dose of intralesional triamcinolone acetonide 10 mg/ml and 40 mg/ml, respectively, by Chandna et al. and Rangdhol et al.[13],[14]

The procedure was repeated every 3 days for 2 weeks with 20 mg/ml and with tapering of dose 10 mg/ml for 2 weeks. An 6-month follow-up did not show recurrence.

 Conclusion



The diagnosis of idiopathic OFG is by exclusion supported by the histopathological evidence of chronic granulomatosis inflammation. Endoscopy, negative GI symptoms ruled out Crohn's disease. Intralesional corticosteroids are beneficial and a regular follow-up and review is essential. Intralesional corticosteroids are beneficial and a regular follow-up is essential for management of idiopathic OFG.

Acknowledgments

The authors gratefully thank Dr. Hardik Rana and Dr. Abinesh M. Sennerby for their advice and encouragement during the course of this case study.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Wiesenfeld D, Ferguson MM, Mitchell DN, MacDonald DG, Scully C, Cochran K, et al. Oro-facial granulomatosis – A clinical and pathological analysis. Q J Med 1985;54:101-13.
2Smeets E, Fryns JP, Van den Berghe H. Melkersson-Rosenthal syndrome and de novo autosomal t (9;21)(p11;p11) translocation. Clin Genet 1994;45:323-4.
3Riggio MP, Gibson J, Lennon A, Wray D, MacDonald DG. Search for Mycobacterium paratuberculosis DNA in orofacial granulomatosis and oral Crohn's disease tissue by polymerase chain reaction. Gut 1997;41:646-50.
4Allen CM, Camisa C, Hamzeh S, Stephens L. Cheilitis granulomatosa: Report of six cases and review of the literature. J Am Acad Dermatol 1990;23:444-50.
5Angelopoulos AP, Goaz PW. Incidence of diphenylhydantoin gingival hyperplasia. Oral Surg Oral Med Oral Pathol 1972;34:898-906.
6Matricon J, Barnich N, Ardid D. Immunopathogenesis of inflammatory bowel disease. Self Nonself 2010;1:299-309.
7Kalmar JR. Crohn's disease: Orofacial considerations and disease pathogenesis. Periodontol 2000 1994;6:101-15.
8Allen R, Mendelsohn FA, Csicsmann J, Weller RF, Hurley TH, Doyle AE. A clinical evaluation of serum angiotensin converting enzyme in sarcoidosis. Aust N Z J Med 1980;10:496-501.
9Rogers RS 3rd. Melkersson-Rosenthal syndrome and orofacial granulomatosis. Dermatol Clin 1996;14:371-9.
10Bachmeyer C, Petitjean B, Testart F, Richecoeur J, Ammouri W, Blum L. Lingual necrosis as the presenting sign of Wegener's granulomatosis. Clin Exp Dermatol 2006;31:321-2.
11James J, Ferguson MM. Orofacial granulomatosis presenting clinically as tuberculosis of cervical lymph nodes. Br Dent J 1986;161:17-9.
12Alajbeg I, Rogulj AA, Hutinec Z. Orofacial granulomatosis treated with intralesional triamcinolone. Acta Dermatovenerol Croat 2011;19:165-9.
13Chandna S, Mahendra A, Kaur R. Gingival manifestations of orofacial granulomatosis: A rare finding. Clin Adv Periodontics 2017;7:57-62.
14Rangdhol RV, Madhulika N, Dany A, Jeelani S, Asokan GS. Idiopathic orofacial granulomatosis – A diagnostic & treatment challenge. J Clin Diagn Res 2014;8:ZD07-10.