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CASE REPORT
Year : 2012  |  Volume : 30  |  Issue : 3  |  Page : 262-266
 

A rare case report of orofacial granulomatosis in a pediatric patient


1 Department of Pedodontics and Preventive Dentistry, HP Government Dental College, Shimla, Himachal Pradesh, India
2 Department of Pedodontics and Preventive Dentistry, VS Dental College and Hospital, Bangalore, Karnataka, India
3 Department of Oral Pathology and Microbiology, HP Government Dental College, Shimla, Himachal Pradesh, India

Date of Web Publication21-Dec-2012

Correspondence Address:
P Singhal
Department of Pedodontics and Preventive Dentistry, HP Government Dental College, Shimla, Himachal Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-4388.105022

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   Abstract 

Orofacial granulomatosis comprises a group of diseases characterized by noncaseating granulomatous inflammation affecting the soft tissues of the oral and maxillofacial region. The most common clinical presentation is persistent swelling of one or both lips. It is important to establish the diagnosis accurately because this condition is sometimes a manifestation of Crohn's disease or sarcoidosis. This article describes a case of orofacial granulomatosis.


Keywords: Granulomatosis, mouth diseases, orofacial


How to cite this article:
Singhal P, Chandan G D, Das U M, Singhal A. A rare case report of orofacial granulomatosis in a pediatric patient. J Indian Soc Pedod Prev Dent 2012;30:262-6

How to cite this URL:
Singhal P, Chandan G D, Das U M, Singhal A. A rare case report of orofacial granulomatosis in a pediatric patient. J Indian Soc Pedod Prev Dent [serial online] 2012 [cited 2019 Oct 23];30:262-6. Available from: http://www.jisppd.com/text.asp?2012/30/3/262/105022



   Introduction Top


Orofacial granulomatosis (OFG) is a rare and heterogeneous clinical condition that presents with chronic swelling of the oral or facial tissues due to granulomatous inflammation. [1] This term, introduced by Wiesenfeld in 1985, encompasses  Melkersson-Rosenthal syndrome More Details (MRS) and cheilitis granulomatosa (CG) of Miescher. [2] MRS is the term used when cheilitis occurs with facial palsy and plicated tongue. MRS is occasionally a manifestation of Crohn's disease or OFG. [3],[4] CG of Miescher is characterized by swelling restricted to the lips. [5] Miescher cheilitis is generally regarded as a monosymptomatic form of the MRS, although the possibility remains that these may be two separate diseases. According to Neville et al., [6] these two entities should not be considered distinct diseases and should both be included in the spectrum of OFG. In granulomatous cheilitis, normal lip architecture is eventually altered by the presence of lymphoedema and noncaseating granulomas in the lamina propria. The frequency is unknown; the condition is rare. Morbidity depends on whether underlying organic disease, such as Crohn's disease or sarcoidosis, is present. No racial predilection is recognized. No sexual predilection is known. Onset usually occurs in young adult life.

The precise cause of OFG is unknown. [7] Several theories have been suggested, including infection, genetic predisposition, and allergy. [8],[9],[10],[11],[12] More recently, researchers have identified a monoclonal lymphocytic expansion in OFG lesions and have suggested it could be secondary to chronic antigenic stimulation. [13] It appears that cytokine production by the lymphocytic clone could be responsible for the formation of granulomas in these lesions. [14] The classic presentation of OFG is a nontender recurrent labial swelling that eventually becomes persistent. [15] This swelling may affect one or both lips, causing lip hypertrophy (macrocheilia). [16] The swelling is initially soft but becomes firmer with time as fibrosis ensues. However, the clinical presentation can be highly variable, making the diagnosis difficult to establish. For example, the recurrent facial swelling may affect the chin, cheeks, periorbital region and eyelids, [17] and, in rare cases, it may not be associated with lip hypertrophy. Intraoral involvement may take the form of hypertrophy, erythema or nonspecific erosions involving the gingiva, oral mucosa, or tongue. [16],[17] The diagnostic dilemma may be further complicated by the fact that OFG may be the oral manifestation of a systemic condition, such as Crohn's disease, sarcoidosis, or, more rarely, Wegener's granulomatosis. [18] In addition, several conditions, including tuberculosis, leprosy, systemic fungal infections, and foreign body reactions may show granulomatous inflammation on histologic examination. [7] Therefore, these conditions must be distinguished. The diagnosis of OFG is made by histopathologic identification of noncaseating granulomas. Local and systemic conditions characterized by granulomatous inflammation must be excluded by appropriate clinical and laboratory investigations. [7],[18] This article presents a case of OFG. The article outlines the diagnostic approach used to investigate a patient presenting with macrocheilia (lip hypertrophy) and discusses various therapeutic modalities used in treating OFG.


   Case Report Top


A 9-year-old female patient was referred for treatment of persistent swelling of the upper lip and gingiva in the anterior region of mouth. These symptoms had been present for approximately one and half year and were not associated with any change in oral hygiene products or cosmetics. There was no history of any treatment rendered. Patient did not present with any intestinal complaints. There was no history of facial paralysis, visual disturbance, or headache.

Extraoral examination revealed no lymphadenopathy or signs of dysphasia. A prominent upper lip swelling with Fissuring was noted [Figure 1]. Intraoral examination revealed gingiva in the area of the lower anterior teeth erythematous and swollen, with a slightly granular surface [Figure 2]. There were no appreciable changes on the dorsal surface of the tongue. The rest of the intraoral examination was unremarkable. The differential diagnosis included Crohn's disease, sarcoidosis, tuberculosis, an allergic reaction, and OFG. MRS was considered less likely in this case.
Figure 1: Prominent swelling and fissuring involving the upper lip

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Figure 2: Erythematous and swollen Gingiva

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Chest radiography and a series of blood tests, including assessment of serum levels of angiotensin converting enzyme, and C reactive protein test were requested. An in-depth gastrointestinal investigation did not appear justified in this case, since there were no sign of anemia or symptoms suggestive of Crohn's disease but still chronic inflammatory bowel disease profile (immunofluorescence) was done to rule out this disease. Monteux test was done to rule out tuberculosis. A biopsy sample of the upper lip and gingiva was obtained for histopathologic evaluation.

Microscopic examination of the biopsy sample revealed several noncaseating epithelioid granulomas with multiple giant cells, which were identified. A perivascular lymphocytic infiltrate was also noted [Figure 3] and [Figure 4]. The histopathologic diagnosis of OFG was made. The results of patch tests, done (with both regular and dental series) to exclude an allergic cause, were negative. Therefore, a final diagnosis of idiopathic OFG was made.
Figure 3: Photomicrograph of the lip biopsy sample, showing diffuse edema of the connective tissue and mild fibrosis of the lamina propria. Several granulomas are visible at this magnification, along with a perivascular inflammatory infiltrate. (Hematoxylin and eosin; original magnification 4)

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Figure 4: Photomicrograph of the gingiva biopsy sample, showing giant cells, along with a perivascular inflammatory infiltrate

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Once the diagnosis was established, the following treatment was planned for the patient-Triamcinolone (Aristocort) 10 mg/ml solutions; intralesional; per day for 10 days, oral prophylaxis followed by gingivectomy. Two weeks later, the patient reported with normal appearance of her lip and gingivae. There was slight recurrence 6 months postoperative, which was less severe than the condition in which the patient reported first to the clinic. If necessary, reduction cheiloplasty can be done in future.


   Discussion Top


OFG is an uncommon disease, normally seen in the second decade of life and has a female predilection. [19] The differential diagnosis of a persistent labial swelling includes angioedema (idiopathic or hereditary), sarcoidosis, Crohn's disease, OFG, CG, and some specific infections (tuberculosis, leprosy, and deep fungal infections). [20],[21] Amyloidosis, certain soft-tissue tumors, minor salivary gland tumor, and Ascher's syndrome may also be included in the differential diagnosis. [15] All of these conditions must be taken into account during the investigation of a patient with persistent lip swelling. The medical history and the results of the clinical examination help to direct the investigation. The biopsy represents an important step in establishing the correct diagnosis, especially if angioedema is not a favored possibility. Upon microscopic identification of granulomatous inflammation, special stains are used to rule out deep fungal infections (periodic acid schiff stain (PAS) with diastase, Grocott) or specific bacterial infections (Ziehl-Neelsen, Gram). Polarized light microscopy is used to identify foreign bodies in the tissues. Ancillary tests are ordered to assess whether a systemic disease is responsible for the granulomatous inflammation. Such tests might include chest radiography and assessment of serum levels of angiotensin-converting enzyme for sarcoidosis; complete blood count, erythrocyte sedimentation rate and serum levels of folic acid, iron and vitamin B12 for Crohn's disease; and tuberculin skin test and chest radiography for tuberculosis. Gastrointestinal assessment is essential, especially in the presence of signs of anemia and intestinal malabsorption and symptoms suggestive of Crohn's disease. If the initial investigation does not confirm the diagnosis, a second assessment should be carried out, especially if the gastrointestinal signs and symptoms persist. The diagnosis of OFG is therefore a diagnosis of exclusion and is based on appropriate clinical and pathologic correlation.

The treatment of OFG is difficult, particularly in the absence of an etiologic factor. Treatment objectives are to improve the patient's clinical appearance and comfort. Although rare, spontaneous remission is possible. [22] The elimination of odontogenic infections may reduce the swelling in certain patients. [23] First-line treatment involves the use of local or systemic corticosteroids or both. Intralesional injections of triamcinolone 10 mg/ml is often used in the treatment of OFG. [24] Recently, higher concentrations of the drug (40 mg/ml) have been suggested. The higher concentration offers the advantages of reducing the volume of fluid injected, the administration of a higher dose, and the maintenance of remission. [21] The side effects of local treatment are limited to skin atrophy and hypopigmentation. [21] The use of systemic corticosteroid therapy [22],[25] in treating OFG is limited because of the chronic, recurrent nature of the disease, and the side effects associated with long-term use of these drugs. Results are often immediate with either local or systemic corticosteroid therapy. However, relapses are common, and long-term treatment may be required. [15] Other therapeutic measures have been reported in the literature, including hydroxychloroquine, [15],[25] methotrexate, clofazimine, [25] metronidazole, minocycline [26] alone or in combination with oral prednisone, thalidomide, [27],[28] dapsone, and danazol. Cheiloplasty is used by some clinicians, especially in cases complicated by major lip deformation or inadequate response to local corticosteroid therapy.

Surgery may be necessary to manage the complications of the disease and is also used in cases that do not respond to medical treatment. The literature on OFG, MRS, and CG shows an important problem in the classification of these entities. This is probably related to a lack of understanding of etiologic and pathogenic mechanisms. For example, some authors consider CG as an oligosymptomatic or monosymptomatic variant of MRS, [15],[16],[23] whereas others suggest that these conditions are distinct entities. [5],[25] Some claim that CG is a manifestation of sarcoidosis or Crohn's disease. Yet others consider OFG, MRS, CG, Crohn's disease, and sarcoidosis to represent different manifestations of the same disease process. [22],[29] Use of the term "OFG" in cases of noncaseating granulomatous inflammation has the advantage of describing a clinicopathologic situation without linking it to a specific disease entity. It is essential then to specify whether the condition is caused by a systemic disease or a local condition or if it is essentially idiopathic. Therefore, terms like "OFG in the context of sarcoidosis or in the context of Crohn's disease" and "OFG secondary to a chronic dental infection or to contact hypersensitivity" are recommended. A diagnosis of idiopathic OFG is made on the basis of negative results of a thorough investigation.


   Conclusions Top


One case of idiopathic OFG has been described. The differential diagnosis, investigation, and treatment plan for the case have been discussed. The use of standardized terminology when reporting such cases to identify epidemiologic, etiologic, and therapeutic data is recommended. Use of standard terms should eventually lead to improvements in both therapeutic decision-making and patients' prognosis.

 
   References Top

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2.Wiesenfeld 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.  Back to cited text no. 2
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3.Greene RM, Rogers RS 3 rd . Melkersson-Rosenthal syndrome: A review of 36 patients. J Am Acad Dermatol 1989;21:1263-70.  Back to cited text no. 3
    
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9.Pachor ML, Urbani G, Cortina P, Lunardi C, Nicolis F, Peroli P, et al. Is the Melkersson-Rosenthal syndrome related to the exposure to food additives? A case report. Oral Surg Oral Med Oral Pathol 1989;67:393-5.  Back to cited text no. 9
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13.Lim SH, Stephens P, Cao QX, Coleman S, Thomas DW. Molecular analysis of T cell receptor beta variability in a patient with orofacial granulomatosis. Gut 1997;40:683-6.  Back to cited text no. 13
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14.De Quatrebarbes J, Cordel N, Bravard P, Lenormand B, Joly P. Miescher's cheilitis and lymphocytic clonal expansion: 2 cases. Ann Dermatol Venereol 2004;131:55-7.  Back to cited text no. 14
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15.Allen 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.  Back to cited text no. 15
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16.Zimmer WM, Rogers RS 3 rd , Reeve CM, Sheridan PJ. Orofacial manifestations of Melkersson-Rosenthal syndrome. A study of 42 patients and review of 220 cases from the literature. Oral Surg Oral Med Oral Pathol 1992;74:610-9.  Back to cited text no. 16
    
17.Mignogna MD, Fedele S, Lo Russo L, Lo Muzio L. The multiform and variable patterns of onset of orofacial granulomatosis. J Oral Pathol Med 2003;32:200-5.  Back to cited text no. 17
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18.Girlich C, Bogenrieder T, Palitzsch KD, Scholmerich J, Lock G. Orofacial granulomatosis as initial manifestation of Crohn's disease: A report of two cases. Eur J Gastroenterol Hepatol 2002;14:873-6.  Back to cited text no. 18
    
19.Reddy S, Rakesh N, Ramadoss T, Jatti D. Orofacial granulomatosis-A rare case report with review of literature. J Clin Exp Dent 2010;2:e138-41.  Back to cited text no. 19
    
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21.Mignogna MD, Fedele S, Lo Russo L, Adamo D, Satriano RA. Effectiveness of small-volume, intralesional, delayed-release triamcinolone injections in Orofacial granulomatosis: A pilot study. J Am Acad Dermatol 2004;51:265-8.  Back to cited text no. 21
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22.Sciubba JJ, Said-Al-Naief N. Orofacial granulomatosis: Presentation, pathology and management of 13 cases. J Oral Pathol Med 2003;32:576-85.  Back to cited text no. 22
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23.Worsaae N, Christensen KC, Schiødt M, Reibel J. Melkersson-Rosenthal syndrome and cheilitis granulomatosa. A clinicopathological study of thirty-three patients with special reference to their oral lesions. Oral Surg Oral Med Oral Pathol 1982;54:404-13.  Back to cited text no. 23
    
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25.van der Waal RI, Schulten EA, van der Meij EH, van de Scheur MR, Starink TM, van der Waal I. Cheilitis granulomatosa: Overview of 13 patients with long-term follow-up-results of management. Int J Dermatol 2002;41:225-9.  Back to cited text no. 25
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28.Hegarty A, Hodgson T, Porter S. Thalidomide for the treatment of recalcitrant oral Crohn's disease and orofacial granulomatosis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;95:576-85.  Back to cited text no. 28
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29.Lloyd DA, Payton KB, Guenther L, Frydman W. Melkersson-Rosenthal syndrome and Crohn's disease: One disease or two? Report of a case and discussion of the literature. J Clin Gastroenterol 1994;18:213-7.  Back to cited text no. 29
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