Oral Sub Lingual Sarcoidosis: Report of a Rare Case and Review of Literature
Heera Raveendran, Beena Valappil Thannikunnath, Kanaram Choudhary, Rency Simon
The oral cavity is frequently involved in conditions that affect the skin or other multiorgan diseases. Sarcoidosis is a multisystem non-caseating, granulomatous condition of unknown etiology, most commonly presenting in young adults. Pulmonary and lymph node involvement are common and although oral lesions can occur, these are rare. This is a case report of a female aged 26 who presented with a sublingual swelling. Oral lesions may constitute the first sign of systemic sarcoidosis and for this reason the oral mucous membrane needs to be explored when there is a suspicion of sarcoidosis, and systemic sarcoidosis must be included in the differential diagnosis of oral granulomatous lesions. We emphasize the role of angiotensin converting enzyme level, special bacterial stain and proper systemic evaluation in diagnosis of this rare presentation.
Key words: Heerfordt Syndrome;Oral Sublingual Sarcoidosis;Orofacial Granulomatosis.
Heera Raveendran, Beena Valappil Thannikunnath, Kanaram Choudhary, Rency Simon. Oral sub lingual sarcoidosis: Report of a rare case and review of literature. International Journal of Oral & Maxillofacial Pathology; 2011:2(1):29-32. ©International Journal of Oral and Maxillofacial Pathology. Published by Publishing Division, Celesta Software Private Limited. All Rights Reserved.
Received on: 25/12/2010 Accepted on: 11/02/2011
Sarcoidosis is an idiopathic systemic non-caseating granulomatous disease characterized by bilateral hilar lymphadenopathy and non-caseating granulomas in the lungs. It was first reported in the 19th century, by Jonathan Hutchinson (1875), an English surgeon-dermatologist, but the term sarcoidosis was introduced later by Boeck (1899), which in Greek means ‘fleshlike condition’.1 Sarcoidosis may affect nearly any organ system; organs involved include the lung, liver, heart, spleen, eyes, kidneys, skin and lymph system. Pulmonary manifestations are the most common and include dyspnea on exertion, nonproductive cough, chest pain, wheezing, nasal congestion, and hemoptysis. There is a worldwide difference in incidence rates of sarcoidosis. The reported incidence rate is 10 to 12 cases/1,000 new registrations annually at the Respiratory Unit at Kolkata and 61.2/100,000 new cases seen at the Vallabhbhai Patel Chest Institute, Delhi, India2.
The incidence rate reported in Sweden and the UK was respectively 64 and 20 cases per 100, 0003. In the USA, there is a wide racial variation in incidence rate, with 35.5–64 cases per 100, 000 for African-Americans and 10-14 cases per 100, 000 for Caucasians4. The disease exhibits a slight female predilection and a bimodal age distribution. The first peak is between the ages of 25 and 35 years and the second peak is between 45 and 65 years5. The multiple organs with progressive pulmonary involvement indicate a poor prognosis. Overall, the prognosis of sarcoidosis is good. As in about 15 to 20 % of patients, the disease remains active or recurs intermittently. Death is attributable directly to the disease in about 10% of all those affected6. Although infrequent, oral lesions may constitute the first sign of systemic sarcoidosis. For this reason the oral mucous membrane needs to be explored when there is a suspicion of sarcoidosis, and systemic sarcoidosis must be included in the differential diagnosis of oral granulomatous lesions. We hereby report a rare case of a female aged 26 who presented with a sublingual swelling. There is also emphasis on the role of angiotensin converting enzyme (ACE) level, special bacterial stain and proper systemic evaluation in diagnosis of this rare presentation.
A 26 year old female patient reported to the Department of Oral medicine and Radiology, Government Dental College, Trivandrum, Kerala, South India, with a chief complaint of swelling on the floor of mouth with 1 month duration. There was no history of trauma or infection or allergy. Intra oral examination showed a well demarcated round, soft, non-tender, non-ulcerated bluish swelling in right side of floor of mouth measuring 2 x 2 cm in diameter (Fig 1). Oral hygiene status was not good but no carious lesions were identified.
Figure1: Clinical photograph showing sublingual salivary gland swelling
There was no associated lymphadenopathy, fever, chronic illness, breathlessness or xerostomia. A clinical diagnosis of ranula was made. Excision of the lesion with removal of right sublingual gland was performed. Gross specimen were of two bits, large bit was of size 2.5×1×0.8cms, grayish brown in color and soft to firm in consistency and small bit was of size 1x0.3x0.3cms, translucent brown in color and soft in consistency.
Microscopy with hematoxylin and eosin stain revealed tissue composed of salivary gland acini replaced by non-caseating granulomatous tissue. The non-caseating granulomatous tissue was made up of large number of epitheliod cells in the center, surrounded by multinucleated giant cells and lymphocytes peripherally. Some of the multinucleated giant cells appeared like Langhan’s type of giant cells with horse shoe shaped nuclear arrangement. Surrounding stromal tissue was infiltrated by dense diffuse collection of chronic inflammatory cells chiefly lymphocytes (Fig 2).
Figure 2: Non- caseating granuloma with central Langhan’s giant cell surrounded by epithelioid cells (x40)
The other bit showed dilated salivary gland duct filled with mucin and also spilled mucin in the stromal connective tissue. (Fig 3)
Figure 3: Areas showing ductal proliferation and mucin spillage (x 10)
The unusual presentations of microscopy lead us to other investigations such as differential leucocytes count, erythrocyte sedimentation rate and chest radiograph which were all within normal limits. (Fig 4) Mantoux test was performed to rule out tuberculosis, which was negative. Acid fast staining of the section was also done to rule out tuberculosis and was negative. (Fig 5) The clinical features along with the microscopic features when correlated with the investigations done, prompted us to make the diagnosis of sublingual sarcoidosis.
Figure 4: Chest radiograph showing no detected anomalies
Oral involvement of sarcoidosis is quite uncommon. It may be solitary or part of a generalized sarcoidosis. Indeed, in the review of literature, oral involvement usually appeared in patients with chronic multisystem sarcoidosis. However, it was the first manifestation of the disease in 35.2% of the cases1,7. Poe reported the first confirmed case
Figure 5: Acid fast staining of the Paraffin section (x10)
of sarcoidosis affecting the oral cavity in the mandible in 1943. According to literature, since then there had only been 73 well-documented cases of oral sarcoidosis1,8.
The cause of sarcoidosis is unknown. A putative genetic pathogenesis has been suggested due to the presence of familial clusters in sarcoidosis. Various infectious and noninfectious agents have been implicated, but no direct cause and effect relationship with any specific agent is established. However, all available evidence is consistent with the concept that the disease results from an exaggerated cellular immune response (acquired, inherited, or both) to a limited class of persistent antigens or self-antigens1,6.
The jaw bones were one of the common sites of manifestations in the oral cavity, occurring in 29.6% of patients; clinical signs were mainly due to lytic and / or expansile lesions in the bone. Other oral sites have further been described in patients with sarcoidosis, involving the buccal mucosa (18.3%), the gingiva, in form gingivitis and gingival hyperplasia (16.9%), the lips (8.5%), the floor of the mouth (8.5%), the tongue (7%), the palate (4.2%) and the submandibular glands (2.8%). Multiple site involvement in oral cavity was seen in 4.2% of the patients. Such sarcoidosis related oral lesions may present as painless swelling, submucosal nodules or ulcerations9. Our case report confirms these data as the patient exhibited a painless swelling in the floor of the mouth of 1 month duration.
Parotid gland involvement occurs in 6% of patients with sarcoidosis. The gland involvement is usually bilateral and is slightly more common in women10. Submandibular and sublingual glands involvement is less common than parotid gland involvement11,12. Clinical presentation of sarcoidosis in major salivary glands is usually as painless firm swellings, which do not show variation in the size during meal time. When palatal or labial salivary glands from clinically normal mucosa in patients with sarcoidosis were biopsied, the rate of involvement ranged from 19% to 58%11,13. The exact number of minor salivary gland involvement is not known, but it is possible that if it is extensive, it may contribute to xerostomia12. The association of sarcoidosis with Heerfordt-Waldenstrom and Lofgren’s syndrome is well established6.
Sarcoidosis with extreme variation in site and tissue involvement has been reported in literature. Five cases of coexistence of Sjogren’s syndrome and sarcoidosis,14 neurosarcoidosis involving trigeminal nerve,15 sarcoidosis with oronasal fistula,8 sinonasal sarcoidosis and a rare association of thymoma, myasthenia gravis and sarcoidosis were also reported in literature16.
The differential diagnosis for oral sarcoidosis found on biopsy in the oral cavity is orofacial granulomatosis which encompasses a group of related conditions affecting the oral and maxillofacial region, characterized by the presence of non-specific granulomatous inflammation. Orofacial granulomatosis includes bacterial infections (tuberculosis, syphilis, cat-scratch disease and leprosy), fungal infections (histoplasmosis, coccidiodomycosis), foreign body granulomas and Crohn’s disease1,6.
Diagnosis is based upon the combination of the clinical features, bilateral hilar lymphadenopathy in chest radiograph and a histological diagnosis of noncaseating granulomas of no identifiable cause6. Cells within the granulomas synthesize angiotensin converting enzyme8. Hence ACE levels will be increased in most patients with sarcoidosis. Thus, serum ACE levels can be a useful tool in monitoring disease activity and the response to treatment in addition to aiding in diagnosis6,17. In the case reported, ACE level was 43 µ/l, the normal value being 8-52 µ/l. We are assuming that the ACE level in the presented case is slightly less than the upper limit of 52µ/l. This could be due to the fact that the blood sample was collected 15 days after excision of the lesion.
In the reported case, we arrived at the diagnosis of sarcoidosis involving sublingual salivary gland after exclusion of above mentioned differential diagnosis and proper systemic medical evaluation. Review of reported cases of solitary involvement of
salivary gland by sarcoidosis was also helpful for a conclusive diagnosis of sublingual sarcoidosis.
The treatment most frequently carried out is surgical excision, as in our case, which is the treatment of choice for singular nodular lesions that are uncomfortable1. The oral administration of corticosteroid should be considered only for painful or progressive lesions that interfere with the functionality of oral cavity, making oral exercises difficult1.
In conclusion, sarcoidosis is a relatively common disease but its oral manifestations are fairly uncommon. Oral lesions may be the initial manifestation of the disease. Clinical history, adjuvant haematological and biochemical tests in combination with specific histochemical stains will help in a precise diagnosis. Suspected cases of oral sarcoidosis should be biopsied and also referred to a physician to rule out other systemic involvement.
Author Affiliations: 1. Dr. Heera Raveendran, Associate Professor, 2. Dr. Beena Valappil Thannikunnath, Professor and Head, 3. Dr. Kanaram Choudhary, Post Graduate Student, 4. Dr. Rency Simon, Post Graduate Student, Department of Oral and Maxillofacial Pathology, Govt. Dental College, Trivandrum, Kerala.
Acknowledgement: All the Staff members in the Department of Oral and Maxillofacial Pathology, Govt. Dental College, Trivandrum, Kerala.
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Dr. Heera Raveendran,
Dept of Oral and Maxillofacial Pathology,
Govt. Dental College,
E mail. firstname.lastname@example.org
Source of Support: Nil, Conflict of Interest: None Declared
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