Clinical Manifestations

Common presenting symptoms are nasal crusting, serosanguineous discharge, epistaxis, nasal swelling and obstruction, and constitutional ("B") symptoms (fever, night sweats, and weight loss). Destruction of the nasal cartilages and bone, occasionally with local infiltration into the adjacent facial areas (e.g., orbital swelling), may occur. Some cases present with a mass effect (i.e., a nasal mass or obstruction). Patients may experience longstanding symptoms before diagnosis is made. Secondary infection of necrotic tissue is not uncommon.

Localized disease may progress and involve the skin and subcutaneous tissue and less often the GI tract, testis, and upper respiratory tract.

A hemophagocytic syndrome may complicate the disease and adversely affect survival. It may occur at any time during the clinical course and is usually fatal after a period of several weeks.

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