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Clinical Problem Solving: Radiology
August 2002

Diagnosis Radiology Quiz

Arch Otolaryngol Head Neck Surg. 2002;128(8):980. doi:10.1001/archotol.128.8.979
Diagnosis: Nasal sarcoidosis, in association with pulmonary sarcoidosis

Sarcoidosis is a systemic granulomatous disease of unknown etiology. It affects a variety of systems, including the nose, lungs (most common), skin, liver, eyes, spleen, and lymph nodes. Although it may affect any ethnic group, it is more prevalent and severe in black persons, usually presenting in the third or fourth decade of life.1

Diagnosis is based on clinical presentation, histologic evidence of noncaseating granulomas, and exclusion of other granulomatous diseases. Differential diagnosis should include histoplasmosis, tuberculosis, berrylliosis, hypersensitivity pneumonitis, lymphoma, and eosinophilic granuloma.2

Concerning pulmonary sarcoidosis, patients often present asymptomatically, with an incidental observation of bilateral hilar adenopathy on x-ray films. Sarcoidosis of the paranasal sinuses usually presents with nasal obstruction and chronic sinusitis.3 A consistent finding in the nose is an erythematous, edematous, hypertrophied, friable mucosa. Radiologic studies often show extensive opacification of the sinuses. Our studies revealed a destructive mass arising from the right nasal ethmoidal area, extending into the right inferior turbinate and nasal septum, with bony destruction in the ethmoid and nasal bones on the right side. Interestingly, our patient did not exhibit erythematous, friable mucosa.

Histologically, sarcoidosis presents with multiple epithelioid cell granulomas,4 which are organized collections of mature mononuclear macrophages. Other features, including hyaline fibrosis, leukocyte infiltration, occasional necrosis, and refractile structures in giant cells, may be present. These epithelioid cells secrete more than 40 cytokines and mediators, including angiotensin-converting enzyme (ACE). Although a high ACE level is associated with active sarcoidosis, it is not diagnostic. Many disorders, including cirrhosis, diabetes, and hypersensitivity pneumonitis, present with elevated ACE levels. Schaumann bodies are seen in the majority of the patients (48%-88%). They are concentric, calcified, laminated structures that indicate chronic granulomatous disease. Histologic examination of the biopsy specimen in the present case revealed a diffuse nonnecrotizing granulomatous process (Figure 3 and Figure 4 [original magnification ×40 and ×200, respectively]). Higher magnification (Figure 5 [original magnification ×400]) showed a predominance of epithelioid histiocytes with scattered lymphocytes.

In summary, nasal sarcoidosis should be included in the differential diagnosis when a history of nasal obstruction is present, with or without a history of sarcoidosis. Appropriate radiologic and serologic testing should be performed along with a biopsy for correlation with the other clinical findings.

The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the US Navy or the Department of Defense.

References
1.
Johns  CJMichele  TM The clinical management of sarcoidosis: a 50-year experience at the Johns Hopkins Hospital.  Medicine (Baltimore).1999;78:65-111. Google Scholar
2.
Robson  KJPiette  WW Cutaneous manifestations of systemic diseases.  Med Clin North Am.1998;82:1359-1379. Google Scholar
3.
deShazo  RDO'Brien  MMJustice  WWPitcock  J Diagnostic criteria for sarcoidosis of the sinuses.  J Allergy Clin Immunol.1999;103:789-795. Google Scholar
4.
Sheffield  EA Pathology of sarcoidosis.  Clin Chest Med.1997;18:741-754. Google Scholar
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