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Q1: What is the differential diagnosis for this lesion?
A possibility of bacterial, fungal, and viral infections should be borne in mind when establishing a differential diagnosis.1 Tuberculosis, syphilis, histoplasmosis, and actinomycosis are some of the infections which may produce a sarcoidal type of tissue response or granulomas. Other possible entities are foreign body granuloma and orofacial granulomatosis such as oral Crohn’s disease, granulomatous cheilitis, and Melkersson’s syndrome.2 In this patient our clinical diagnosis was minor salivary gland tumour given the site of occurrence, and hard nodular swelling with overlying normal mucosa.
Q2: How will you establish a definitive diagnosis and suggest the investigations necessary for the same?
Clinical features along with histological evidence of non-caseating epithelioid granuloma from tissue biopsy can be supplemented by chest radiography, the presence of tuberculin anergy, a positive Kviem-Siltzbach skin test, a raised serum angiotensin converting enzyme, and by an increased 24 hour urine calcium level.3 The characteristic histological picture of sarcoidosis in all involved tissues is the non-caseating granuloma composed of aggregates of epithelioid cells, scattered multinucleated giant cells, lymphocytes, plasma cells, and fibroblasts. Two other features often seen in the granuloma are laminated concretions composed of calcium and proteins, known as “Schaumann bodies” and satellite inclusions known as “asteroid bodies”. Although these two features are characteristic, they are not pathognomic.
Q3: Discuss the prognosis and treatment of this lesion?
Overall, the prognosis of sarcoidosis is good. The drugs of choice are glucocorticoids as they suppress the activated T helper-inducer cell processes occurring at the sites of the disease.1 Some advocate only surgical excision of the lesion,4 some medical treatment, while others combine the two modalities. Radiation also has been used as a mode of treatment,5 while spontaneous healing was documented in a few cases.6 Our patient responded well to the surgical treatment.
Sarcoidosis is a disease that has highest occurrence in the third and fourth decades of life and exhibits a slight female predominance (1.5:1).3 In a young adult with constitutional complaints, respiratory symptoms, erythema nodosum, blurred vision, and presence of bilateral hilar lymphadenopathy the diagnosis is almost always sarcoidosis.1 None of these features were seen in the present case. The most common cervicofacial manifestation, excluding ocular and lacrimal gland involvement, appears to be asymptomatic swelling of the parotid gland or cervical nodes.7 Intraoral presentation is uncommon, and in most cases systemic sarcoidosis has been diagnosed before the oral manifestation becomes apparent.8 One study reported the presence of non-caseating granulomas in 38%–58% of biopsies of normal appearing oral mucosa of patients with known sarcoidosis.9 Very few cases of tongue involvement have been reported.2,8,10,11 Bilateral hilar adenopathy is the hallmark of this disease and is also seen in lymphoma, tuberculosis, coccidiomycosis, brucellosis, and bronchogenic carcinoma.1 The presence of skin anergy is typical but not diagnostic.1 The Kviem-Siltzbach skin test yields sarcoidosis-like lesions in 70%–80% of patients, with fewer than 5% false positive results.1 Angiotensin converting enzyme is raised in the serum in approximately two thirds of patients but is also seen in asbestosis, silicosis, berylliosis, fungal infection, granulomatous hepatitis, hypersensitivity, pneumonitis, leprosy, lymphoma, and tuberculosis.1 A raised 24 hour urine calcium level is consistent with the diagnosis but is again not specific.1
Primary intraoral sarcoidosis is of rare occurrence.
Wide excision is diagnostic as well as therapeutic.
In patients presenting with smooth submucosal swelling of tongue a possibility of granulomatous lesions and infections should always be considered.
Primary intraoral sarcoidosis.