Sarcoidosis.

Sarcoidosis is a systemic granulomatous disease that has a predilection for the upper and pulmonary airways, particularly in black women. In the head and neck, sarcoidosis may involve the lymph nodes, skin, middle ear, eyes, and the lacrimal and salivary glands. Extranodal head and neck involvement occurs in as many as 40% of affected patients.

In addition to systemic symptoms and hypercalcemia (a result of the production of high levels of calcitriol by activated macrophages), local symptoms may include sinusitis, lymphadenopathy, hearing loss, facial nerve palsy, and dysphonia.

Noncaseating (non-necrotizing) granulomas are the histologic hallmark of sarcoidosis. These granulomas are typically made up of tightly packed epithelioid macrophages and occasional multinucleated giant cells separated by small aggregates of lymphocytes (figure, A). Patients with sarcoidosis may also exhibit asteroid bodies (stellate crystalline inclusions in multinucleated giant cells) (figure, B), Schaumann bodies (calcified laminated concretions within multinucleated giant cells), and occasionally Hamazaki-Wesenberg bodies (coccoid, golden-brown, acid-fast cytoplasmic inclusions). Long-standing granulomas may undergo fibrosis and eventual hyalinization.

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The etiology of sarcoidosis remains unclear, although an infectious cause is suspected. It is important to remember that sarcoidosis is a diagnosis of exclusion. Other granulomatous conditions, such as foreign body reactions and fungal or mycobacterial infections, must be excluded through the use of ancillary studies (e.g., microbiologic cultures and h istochemical stains such as acid-fast and silver impregnation stains). Although the clinical course may result in spontaneous remission, most patients experience a progressively downward and sometimes fatal sequela, usually as a result of respiratory or central nervous system complications.

Suggested reading

Baughman RP, Lower EE, du Bois RM. Sarcoidosis. Lancet 2003;361: 1111-18.

Shah UK, White JA, Gooey JE, Hybels RL. Otolaryngologic manifestations of sarcoidosis: Presentation and diagnosis. Laryngoscope 1997:107:67-75.