Isolated Sarcoidal Granulomas on the Tongue

Sarcoidosis is a multisystemic disorder characterized by noncaseating granulomas made up of macrophages and T cells. Isolated extrapulmonary disease has been reported in 10% of cases.¹ We present a rare case of isolated tongue involvement that responded to cyclosporine.

A 46-year-old healthy woman presented with tender lesions in her tongue of 2 years' duration. She denied a history of smoking, trauma, amalgams, or piercings. Examination revealed several firm nodules on the dorsal aspect of her tongue (Figure 1). A punch biopsy specimen showed well-defined, noncaseating epithelioid granulomas affecting the submucosal and muscular layers (Figure 2). Foreign material was not identified. Findings of analysis with special stains and fungal and mycobacterial cultures were negative. The tuberculin skin test result was negative.

The patient underwent a full sarcoidosis workup (complete blood cell count, liver and renal function test, angiotensin-converting enzyme determination, 24-hour calcium in urine test, and serologic analysis for syphilis and hepatitis C and B viruses, ophthalmologic examination, pulmonary function testing, chest high-resolution computed tomography, and abdominal ultrasonography) that revealed no abnormalities. She was diagnosed as having sarcoidal granulomas of the tongue, as a probable isolated form of sarcoidosis.

Prednisone treatment (45 mg/d) was initiated, and the nodules improved dramatically over 5 weeks. The lesions relapsed after glucocorticoid treatment was discontinued. We sequentially administered methotrexate (15 mg/wk) and etanercept (50 mg/wk) without improvement. Finally, we performed a wide excision of the lesions, but 1 year later, the lesions relapsed. We started cyclosporine treatment (3 mg/kg/d) with full and quick clinical remission. After 1 year of continuing cyclosporine treatment, the lesions have not relapsed  .

Oral sarcoidosis manifestations include tongue nodules,² split papules of the lips, diffuse gingival enlargement, and strawberry gums.³ To our knowledge, there is only 1 reported case of isolated sarcoidal involvement of the tongue presenting as multiple nodules.⁴ Infectious granulomas of the oral mucosa were ruled out in our case by the negative findings of analysis with cultures and stains. Furthermore, the patient's condition did not worsen but responded to glucocorticoids and cyclosporine. Other noninfectious granulomatous disorders that were considered are foreign-body granulomas and orofacial granulomatosis (OG), a clinicopathological spectrum comprising Melkenson-Rosenthal syndrome, granulomatous cheilitis, and Crohn disease. There are histologic differences that may help to differentiate OG from sarcoidosis: (1) granulomas in OG are subtle and poorly formed and close to blood vessels (especially lymphatics); and (2) edema and vascular dilatation are frequent findings in OG.⁵ However, since we did not performed endoscopy, and taking into account that asymptomatic cases of Crohn disease exist, we cannot fully exclude this entity in our patient.

The patient demonstrated a quick, complete, and sustained remission with cyclosporine treatment. Cyclosporine may inhibit sarcoid granulomas by suppressing surrounding T-cell activity. Sarcoid skin lesions and optic neuropathy have been reported to respond to cyclosporine.⁶ Patients who underwent liver transplantation for hepatic sarcoidosis and were undergoing cyclosporine therapy had no disease recurrence.⁷ However, in a trial on the efficacy of cyclosporine in pulmonary sarcoidosis, this agent did not show benefit.⁸ Nonetheless, it is possible that the efficacy of cyclosporine may be influenced by the organ involved.

In our case, a full study to detect sarcoid involvement in other organs detected no involvement. One might argue that our case does not meet the criteria of sarcoidosis. Although the clinical likelihood of sarcoidosis rises when there is more than 1 affected organ, cases of “monorganic ” sarcoidosis have been reported.¹ In these cases, a sarcoidal reaction (maybe induced by the same stimulus present in conventional sarcoidosis) may take place compartmentalized to just 1 anatomic site. Alternatively, our patient might have had subclinical sarcoid involvement of other organs that we were not able to detect with the currently available tests. In any case, periodic assessment for involvement of other organs seems reasonable in cases of isolated sarcoidal granulomas.