|Year : 2016 | Volume
| Issue : 2 | Page : 195-198
Srinivas Kandula1, Atul A Bajoria1, Sangamesh N Chinnannavar1, Ashok Lingappa2
1 Department of Oral Medicine and Radiology, Kalinga Institute of Dental Sciences, Bhubaneswar, Odisha, India
2 Department of Oral Medicine and Radiology, Bapuji Dental College, Devangere, Karnataka, India
|Date of Submission||12-May-2016|
|Date of Acceptance||13-Aug-2018|
|Date of Web Publication||02-Dec-2016|
Atul A Bajoria
Department of Oral Medicine and Radiology, Kalinga Institute of Dental Sciences, Bhubaneswar, Odisha
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Orofacial granulomatosis (OFG) is a rare disease, usually presenting as a persistent swelling of the soft tissues in the orofacial region, predominantly lips (cheilitis granulomatosa). The treatment of OFG can be challenging as frequent recurrences are very common. Here, we present a case of an Asian female patient with persistent swelling of the upper lip from the past 6 months. After establishing the diagnosis, intralesional triamcinolone injection (10 mg on a weekly schedule) was planned for a period of 6 weeks. Complete resolution of the lesion was observed. This article presents a unique case of cheilitis granulomatosa (CG) with a brief highlight on the various treatment modalities and a structured algorithm discussing the various differential diagnoses, aiding in formulating an accurate diagnosis and effective treatment.
Keywords: Cheilitis ganulomatosa, lip swelling, triamcinolone
|How to cite this article:|
Kandula S, Bajoria AA, Chinnannavar SN, Lingappa A. Orofacial granulomatosis. J Indian Acad Oral Med Radiol 2016;28:195-8
| Introduction|| |
Orofacial granulomatosis (OFG) is a nonspecific granulomatous inflammation of unknown etiology, occurring in any part of the oral cavity. Most commonly it presents as a persistent or recurrent lip swelling, hence the term cheilitis granulomatosa (CG). It was introduced by Miescher  in 1945 and is considered as a monosymptomatic form of Melkersson-Rosenthal syndrome (MRS), which comprises CG, facial nerve palsy, and fissured tongue. CG usually presents in females in the second decade of life. , Many diseases such as Crohn's disease, tuberculosis, sarcoidosis, and lupus erythematosus may present as a persistent lip swelling.
| Case Report|| |
A female patient aged 37 years presented with an asymptomatic swelling of the upper lip from the past 6 months. The swelling was persistent and had increased to the present size [Figure 1]. Medical history of the patient was noncontributory. Palpatory findings included diffuse, firm, and nontender swelling of the upper lip. Dental infection was excluded using vitality tests and radiography. Complete blood count with serum ferritin, B12, and folic acid levels were performed, which were within normal limits. A colonoscopic examination was performed which revealed no abnormalities. Considering the abovementioned findings, clinical features, and other investigations, a working diagnosis of idiopathic granulomatous cheilitis was given. Incisional biopsy from the upper lip was performed which revealed fibrocollagenous and fibromuscular tissue with ill-defined granulomas formed by epitheloid cells, macrophages, and lymphocytes. No giant cells or areas of necrosis were seen. Ziehl-Neelson stain was negative for acid fast bacilli. Histopathology report was suggestive of CG. Intralesional injections using corticosteroid was planned for the patient. One mL of triamcinolone acetonide (TA) (10 mg/mL) along with 0.05 mL of 2% lignocaine per injection was given [Figure 2]. The total dose of TA was 10 mg per sitting, once a week for a period of 6 weeks. The patient was recalled once every month for a period of 6 months. Complete resolution of the lesion was observed after a period of 6 months [Figure 1].
| Discussion|| |
CG presents as a persistent painless swelling of the lip. It is considered as a manifestation of OFG or MRS, which is characterized as a triad of facial palsy and plicated tongue in the absence of any systemic disease.  CG has no racial, gender, and age predilection, and the incidence has been estimated to be 0.08% in the general population. The upper lip, lower lip, or both lips can be involved.  In the present case, only the upper lip was involved, which was in accordance with the case series presented by El-Hakim et al. 
Three forms of histopathological findings may be seen in OFG, two of which include noncaseating granulomas and one includes the presence of nonspecific inflammation. Dilated lymphatics in the superficial lamina propria and perivascular aggregation of histiocytes and plasma cells may also be present. In the present case, ill-defined granulomas formed by epitheloid cells, macrophages, and lymphocytes were seen, which was consistent with the findings of Perez-Calderon et al.,  and Bartell et al.,  depicting "diffuse lymphocytic infiltrate in submucosa, with perivascular, poorly formed granulomas."
The treatment of OFG can be challenging as frequent recurrences are very common. The treatment aspect could be divided into surgical mode of treatment, medical management, or a combination of the abovementioned treatments. Surgical management may include esthetic surgery and cheiloplasty (lip reduction) whereas medical management includes administration of antihistamines, nonsteroidal anti-inflammatory drugs, antibiotics, antituberculous drugs, antilepromatous agents (clofazimine), sulfa drugs (sulfasalazine), antimalarials (hydroxycholoroquine), thalidomide, and steroids (systemic or intralesional). , Surgical mode of treatment is usually carried out in resistant cases when long duration chronic inflammation has caused fibrous tissue proliferation in the lip.  Hence, early diagnosis plays a pivotal role in better treatment outcome.
However, use of chemotherapeutic, antituberculous, and sulfa drugs have various hematologic and systemic adverse effects. Based on the previously published literature, we suggested repeated weekly injections of TA (10 mg/ml) until the desired response was achieved. Low dose of intralesional corticosteroids have minimal or no adverse effects when compared to other chemotherapeutic drugs, and hence should be considered as the first line of treatment for CG.
In the present case we followed the intralesional injection technique described by Mignogna et al.  0.5 mL of TA (10 mg/mL) was injected into each of the 2 equidistant points on the border between the upper lip vermillion and mucosa. In addition to TA, we added 0.05 mL of 2% lignocaine solution (with adrenaline) per injection point. The needle was inserted vertically toward the top and was directed slightly inwards [Figure 2]. The potential side effects of this technique could be ischemia, hypopigmentation, and soft tissue atrophy which may lead to cosmetic hitches. Thus, the needle insertion line was inclined orally to omit these adverse effects. 
Persisting lip swelling can be a puzzling presentation and common etiologies such as trauma and infection due to dental origin must first be ruled out before any invasive tests. After a detailed and thorough history of the patient, the clinician should proceed to the clinical examination, which should also include cranial nerve examination. The diagnosis of CG is based on exclusion of other disease entities. Thus, we proposed an expedient algorithm, which will aid the clinician in diagnosing a case of lip swelling [Table 1] and [Figure 3]. ,
| Conclusion|| |
CG should be diagnosed early, because during the later stages, the fibrous tissues predominates, thus narrowing the treatment options. The present case demonstrates a successful and predictable treatment outcome achieved by small doses of triamcinolone acetonide (10 mg/ml) as intralesional injections, which should be considered as the first-line treatment.
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There are no conflicts of interest.
| References|| |
Miescher G. über essentielle Granulomatose makrocheilie [Cheilitis granulomatosa]. Dermatologica 1945;91:57-85.
Melkersson E. Ett fall av recidiverande facialispares i samband med ett angioneurotiskt ödem. Hygiea 1928;90:737-41.
Rosenthal C. Klinisch-erbbiologischer Beitrag zur Konstitutionspathologie. Gemeinsames Auftreten von Facialislähmung, angio-neurotischem Gesichtsödem und Lingua plicata in Arthritismus-Familien. Z Gesamte Neurol Psych 1931;131:475-501.
El-Hakim M, Chauvin P. Orofacial granulomatosis presenting as persistent lip swelling: Review of 6 new cases. J Oral Maxillofac Surg 2004;62:1114-7.
Perez-Calderon R, Gonzalo-Garijo MA, Chaves A, de Argila D. Cheilitis granulomatosa of Melkersson-Rosenthal syndrome: Treatment with intralesional corticosteroid injections. Allergol Immunopathol 2004;32:36-8.
Bartell HL, Harting M, Eldin KW, Hollier LH, Metry DW. Chronic, progressive enlargement of the lower lip in a healthy girl. Dermatol Online J 2007;13:20.
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.
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.
Glickman LT, Gruss JS, Birt BD, Kohli-Dang N. The surgical management of Melkersson-Rosenthal syndrome. Plast Reconstr Surg 1992;89:815-21.
Mignona M, 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.
[Figure 1], [Figure 2], [Figure 3]