Figure 2- Microscopic view of giant cell bodies and Asteroid bodies and bundles of histiocytes.
With the consultation of a rheumatologist, Nisopred 50 mg was prescribed and after a 2-month follow-up, the patient showed a relative response to the drug, and was referred to a pulmonologist. and Due to the relative response to the previous drug, and single coughs that had started prior to taking it, Salbutamol spray was also prescribed. A chest x-ray was performed and not showed anything in particular.
Within another follow-up, and after quitting Nisopred, the intra-oral signs and lesions recurred, and therefore with the consultation of another rheumatologist, this time Hydroxychloroquine therapy was considered. Although currently the patient hasn’t checked in for a visit and did not start taking Hydroxychloroquine(4), but with follow-up on the phone, the patient reported betterment of the lip redness.
Discussion
Sarcoidosis is described as a disease in which the clinical signs and symptoms are not severe enough to cause alarm. Although the prognosis of sarcoidosis is good and in 60% of cases it regresses on its own, it is still a disease associated with heart, kidney, CNS, and lung involvement. And therefore must be taken seriously, due to the fact that almost 4-10% mortality rate is based on these involvements(5).
Some researchers claim that lesions in the soft tissue of oral cavity due to sarcoidosis are not very common(6), but our patient had a lesion on her buccal mucosa.
Swelling of the cheek and lips suggested it to be Orofacial Granulomatosis, although systemic diseases such as Tuberculosis, Sarcoidosis, Crohn’s disease, and Melkersson-Rosenthal syndrome had to be ruled out first(7), therefore further microscopic testing and examination were required.
Also, in the intra-oral aspect of OFG, cobble-stone swellings, grooved tongue, recurrent labial swelling, and gingival inflammation can be seen(8). On the other hand, intra-oral signs of sarcoidosis includes the occurrence of multiple nodules, xerostomia, and involvement of salivary glands(9). The microscopic view of OFG shows aggregates of non-caseating granulomatosis inflammations(10), while in Sarcoidosis we can see aggregates of epithelial histiocytes and a surrounding rim of lymphocytes and Asteroid bodies and Schaumann bodies.
Some studies suggest a biopsy of affected tissue for demonstration of non-caseating granulomas that strongly support the diagnosis of sarcoidosis(11). And in our case, as mentioned above, the biopsy result revealed a granulomatous inflammation along with non-caseating granulomas.
Measurement of serum angiotensin-converting enzyme (ACE) can be helpful in diagnosis and monitoring the response to treatment, as the level of this enzyme is raised in about 60-80% of patients with sarcoidosis(11, 12). Although due to its poor sensitivity, an increase in ACE levels does not necessarily indicate the diagnosis of sarcoidosis(13). Laboratory tests were performed on our patient and the results were much like expected. ACE levels were higher than the normal range (78 IU/L) which could support the diagnosis.
Another factor that could support the diagnosis of this condition is the Erythrocyte Sedimentation Rate (ESR) which has a normal range of 0-22 in women, and a recent case report study claims that some patients with sarcoidosis can have an increase of ESR level(14). Laboratory tests supported the slight increase of ESR levels to 25 mm/h in our patient as well, although not significantly enough to verify the diagnosis.
Management of this particular disease can range from no interventions to systemic corticosteroids to surgical excision(15). Because it is known that corticosteroids are generally considered beneficial in the acute phase of sarcoidosis, some studies have found that oral glucocorticoids are the first option(16), much like our case in which the first-line treatment was the prescription of Nisopred 50mg.
In cases of sarcoidosis, usually, an absence of treatment response is rare and urges for verifying the absence of a diagnosis error(17). But in this case, we can see resistance to treatment response from the patient.
Nevertheless, studies have shown that oral sarcoidosis can be a manifestation of a systematic disorder(18), therefore follow-up of the patient in this stage is necessary. We hope to bring awareness to other dentists and fellow researchers to notice the oral symptoms of sarcoidosis which is usually assumed to be irrelevant to the disease.
1. Glick M. Burket’s Oral Medicine. India: Jaypee Brothers Medical Publishers; 2021. 732 p.
2. Brad Neville DDD, Carl Allen, Angela Chi. Oral and Maxillofacial Pathology. St Louis, Missouri: Elsevier; 2016. 871 p.
3. Joseph Regezi JS, Richard Jordan. Oral Pathology: Clinical Pathologic Correlations2015. 496 p.
4. Lee Goldman AIS. Goldman-Cecil Medicine: Elsevier; 2019. 2944 p.
5. Kobak S. Catch the rainbow: Prognostic factor of sarcoidosis. Lung India. 2020;37(5):425-32.
6. Kolokotronis AE, Belazi MA, Haidemenos G, Zaraboukas TK, Antoniades DZ. Sarcoidosis: oral and perioral manifestations. Hippokratia. 2009;13(2):119-21.
7. Troiano G, Dioguardi M, Giannatempo G, Laino L, Testa NF, Cocchi R, et al. Orofacial granulomatosis: clinical signs of different pathologies. Med Princ Pract. 2015;24(2):117-22.
8. 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(213):101-13.
9. Takada K, Ina Y, Noda M, Sato T, Yamamoto M, Morishita M. The clinical course and prognosis of patients with severe, moderate or mild sarcoidosis. J Clin Epidemiol. 1993;46(4):359-66.
10. 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(4):200-5.
11. Poate TW, Sharma R, Moutasim KA, Escudier MP, Warnakulasuriya S. Orofacial presentations of sarcoidosis–a case series and review of the literature. Br Dent J. 2008;205(8):437-42.
12. Lee SW, Lee MH, Lee JE, Choi SY, Yi BH, Jung JM. Peritoneal sarcoidosis: A case report. Medicine (Baltimore). 2019;98(24):e16001.
13. Ungprasert P, Carmona EM, Crowson CS, Matteson EL. Diagnostic Utility of Angiotensin-Converting Enzyme in Sarcoidosis: A Population-Based Study. Lung. 2016;194(1):91-5.
14. Achakzai IK, Majid Z, Khalid MA, Khan SA, Laeeq SM, Luck NH. Hepatic Sarcodosis presenting as portal hypertension in a young boy. Gastroenterol Hepatol Bed Bench. 2018;11(1):83-5.
15. Müller-Quernheim J, Kienast K, Held M, Pfeifer S, Costabel U. Treatment of chronic sarcoidosis with an azathioprine/prednisolone regimen. Eur Respir J. 1999;14(5):1117-22.
16. Gupta S, Tripathi AK, Kumar V, Saimbi CS. Sarcoidosis: Oral and extra-oral manifestation. J Indian Soc Periodontol. 2015;19(5):582-5.
17. El Jammal T, Jamilloux Y, Gerfaud-Valentin M, Valeyre D, Sève P. Refractory Sarcoidosis: A Review. Ther Clin Risk Manag. 2020;16:323-45.
18. Bagchi S, Shah N, Sheikh MA, Chatterjee RP. Oral sarcoidosis aiding in diagnosis of underlying systemic disease. BMJ Case Rep. 2019;12(11).