JOURNAL CLUB PRESENTATIONPRESENTED BY: Dr. BIPIN R. UPADHYAY

GUIDED BY: Dr. RAJEEV GADGIL

Dr. AJAY BHOOSREDDY

Dr. CHETAN BHADAGE

Dr. PRASHANT JAJU

Dr. SHAILESH GONDIVKAR

Dr. AJAY NAYAK

ORAL SARCOIDOSIS WITH TONGUE INVOLVEMENT

- A. SERRAT SOTO ET.AL.OOOOE 1997;83:668-71

• Sarcoidosis is a multisystem granulomatous disease of unknown cause that may affect any organ.

• It manifests most frequently with bilateral hilar lymphadenopathy, pulmonary infiltration, and skin or eye lesions.

• Histologically, the hallmark of this disease is a noncaseating, epithelioid-cell granuloma.

• The onset of sarcoidosis is most commonly seen between 20 and 40 years of age.

• Blacks are more commonly affected than whites (10:1)

• Females more commonly affected than males.

• The diagnosis is established when clinical and radiographic findings are supported by histologic evidence of noncaseating epithelioid cell granulomas.

• A 56-year-old white woman was referred for evaluation and treatment of a lesion on the ventral surface of the tongue.

• Her medical history included a diagnosis of systemic sarcoidosis with pulmonary involvement of several years' duration.

CASE REPORT

• Intraoral examination showed a 2 x 3 cm erosive, papular lesion on the right ventral surface of the tongue.

• The lesion had been present for 5 months and was slowly enlarging.

• Additionally, she had a 1.5 cm submucosal nodule in the left commissure of the mouth that had become apparent about 19 months before the lesion on the tongue.

• Both lesions were asymptomatic. • The patient had no difficulty in eating

or talking, and the motility of the tongue was nor reduced.

• No cervical lymph nodes were palpable, and the salivary glands were not enlarged.

• The remainder of the head and neck examination was normal.

• Under local anesthesia, an incisional biopsy was taken from the lesion of the tongue.

• Histologic examination revealed numerous epithelioid cell granulomas, with a variable rim of histiocytes and lymphoid cells surrounding the granuloma, and Langerhans' giant cells.

• Central necroses were not found, nor was there microscopic evidence of acid-fast bacilli, fungi, or birefringent foreign material.

• We also performed a fine-needle aspiration biopsy from the buccal nodule.

• Microscopic examination of the specimen showed the presence of epithelioid cells mixed with multinucleate giant cells in a granulomatous pattern.

• The patient required systemic corticosteroids to control other aspects of her disease; drug therapy was initiated with prednisone, 40 mg daily.

• The prednisone dose was reduced weekly.

• At follow-up examinations, the patient showed a marked reduction in the intraoral lesions.

• The most common cervicofacial manifestation of sarcoidosis, except ocular and lacrimal gland involvement, appears to be asymptomatic swelling of the parotid glands or cervical nodes.

• Oral presentations of sarcoidosis, unrelated to lymph node or salivary gland disease, have been reported infrequently. Reported sites of oral involvement have included the hard and soft palate, lips, tongue, gingiva and tonsil but the disease may affect any site of the mouth.

DISCUSSION

• Oral lesions have typically been described as nontender, well-circumscribed, brownish red or violaceous nodules or papules that occasionally show superficial ulceration.

• Cutaneous sarcoidosis of the facial skin is generally recognizable as a nodule, maculopapules, plaques, or lupus pernio (violaceous infiltrated nodules and plaques that occur more or less symmetrically on the nose, cheeks, ears, etc.).

• Sarcoidosis lesions of the facial bones have been reported.

• Osteoporosis, cortical thinning, a well-defined cyst, and extensive bone destruction have been described.

• The facial nerve is frequently affected, with or without Heerfordt's disease (uveitis, parotid gland enlargement, fever, and facial nerve palsy).

• Sarcoid granulomas may be found on the nasal mucosa, larynx] and paranasal sinuses.

• Intraoral presentation of sarcoidosis is uncommon and, in most cases, sarcoidosis has been diagnosed before the oral manifestation became apparent.

• Oral involvement generally appears in patients with chronic multisystem sarcoidosis and very seldom occurs in the acute stage.

• The tongue is rarely involved in sarcoidosis.

• The SACE level has been studied extensively as a laboratory marker in sarcoidosis.

• Secretion of ACE by granuloma-forming epithelioid cells results in high serum levels in 80 to 90 percent of patients with sarcoidosis.

• However, because of the lack of specificity, an elevated SACE level is only suggestive of, rather than diagnostic for, sarcoidosis.

• Depending on the involvement of the lungs and the lymph nodes, sarcoidosis in the chest radiographs may be staged as follows (Johns and Michelle, 1999):

Stage 0: Normal chest radiograph.Stage I: Bilateral hilar lymphadenopathy without pulmonary infiltrates.Stage II: Bilateral hilar lymphadenopathy with pulmonary infiltrates.Stage III: Pulmonary infiltrates without hilar lymphadenopathy.Stage IV: End-stage fibrosis, cystic cavities, and honeycombing.

• Although the etiology of sarcoidosis is unknown, genetic, infectious and environmental factors have been postulated as possible causes.

• Infectious agents such as mycobacterium, propionobacteria, Epstein–Barr virus (EBV), and human herpes virus-8 (HHV-8) have been considered as possible etiological agents but so far the scientific results have been inconsistent and inconclusive.

•

ETIOLOGY

• Studies confirm a genetic predisposition for sarcoidosis and presents evidence for the allelic variation at the HLA-DRB1 locus as a major contributor.

• Environmental factors (wood dust, pollen, clay, mold, silica) and occupational exposure (farmers, fire fighters, military) have been suggested as etiological agents.

ETIOLOGY

• A randomized age, gender and race matched case–control study (Moller and Chen, 2002) showed:

• A positive association of sarcoidosis with environmental exposure to mold, musty odors and workers in agriculture, insecticides and pesticides.

• There was a negative association with animal dusts and tobacco smoking, and no association with wood dust, pine pollen, silica, metals, and health care workers and the evidence was inconclusive in fire fighters and military personnel.

ETIOLOGY

• The T-helper 1 (Th1) lymphocytes play a central role in granuloma formation, which is thought to be the result of deposition of poorly soluble antigenic material in the tissue.

• This antigenic material is taken up by antigen presenting cells such as macrophages or dendritic cells, which then expose it to T-lymphocytes.

• In response to these antigens, a local amplification of the cellular immune reaction takes place.

PATHOGENESIS

• In addition, mononuclear phagocytes and other inflammatory cells migrate to the site of the antigenic deposition under the influence of the chemokines and cytokines produced by Th1 cells.

• This results in the formation of a granuloma

PATHOGENESIS

• Bacterial, fungal, and viral infections should be borne in mind when establishing a differential diagnosis of oral sarcoidosis.

• Tuberculosis, syphilis, histoplasmosis, actinomycosis, and other infections may produce a sarcoidal type of tissue response or granulomas mimicking sarcoidosis.

• Differential diagnostic possibilities include foreign body granulomas and orofacial granulomatosis, such as oral Crohn's disease, granulomatous cheilitis, and Melkersson's syndrome.

• Carcinoma of the oral cavity should always be considered in the differential diagnosis of oral sarcoidosis.

DIFFERENTIAL DIAGNOSIS

• The chance of spontaneous remission favors a conservative approach to systemic therapy, which is usually carried out with corticosteroids.

• At any time the disease pattern may change, but an expectant policy is often better if the course is not progressive and if vital structures are not involved.

• In oral sarcoidosis any decision about the use of systemic therapy must take into account the seriousness of the accompanying internal involvement and the symptoms of oral lesions.

• Most oral sarcoidosis is treated with corticosteroids, but mild asymptomatic cases may not require systemic therapy.

• Surgical excision has been used to remove oral lesions or fibrotic bands.

• Diagnosis of systemic sarcoidosis prompted by orofacial manifestations: A review of the literature. Mahnaz Fatahzadeh. J Am Dent Assoc. 2006;137;54-60.

• Bilateral parotid swelling caused by sarcoidosis. Farisa Surattanont. JADA, Vol. 133, June 2002.

• Oral sarcoidosis: a review of literature. L Suresh, L Radfar. Oral Diseases (2005) 11, 138–145.

REFERENCES

• Sarcoidosis: oral and perioral manifestations. Kolokotronis ΑΕ. Hippokratia 2009, 13, 2: 119-121.

• Dental Clinics of North America. 49 (2005) 203–221.

• Maxillofacial Pathology, Marx and Stern.• Harrisons Internal Medicine, 17th

edition.

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