Cardiac involvement in Wegener granulomatosis is uncommon. We report a case of Wegener granulomatosis that presented as culture-negative endocarditis with aortic valvular vegetation. The clinical manifestations included gingival hyperplasia, gangrenous digital infarcts, mononeuritis multiplex, high fever, inflammatory arthritis, pansinusitis, splenic infarct, and aortic valvular vegetation, which underscore the difficulty of distinguishing systemic vasculitis from bacterial endocarditis. Contrary to the common notion that valvular vegetation is invariably associated with bacterial endocarditis, this case proves that such findings can occur in Wegener granulomatosis as well. Clinicians are guided toward early treatment with corticosteroids and cyclophosphamide to prevent fatal complications.
A, Infarct on tip of right index finger and cutaneous lesions on right palm. Biopsy sutures are present. B, Punctate nonraised ecchymotic lesions on dorsum of left foot.
Histologic findings in a hematoxylin-eosin–stained skin biopsy specimen obtained from a lesion overlying the left lateral hip area. A, A ×4 magnification reveals the deep dermal location of the well-demarcated inflammatory focus. B, A ×10 magnification of the same section reveals central eosinophilic amorphous deposit infiltrated by neutrophils and surrounded by epithelioid histiocytes (arrow). C, A ×40 magnification of the area indicated by the arrow in B. The arrow in this section highlights a smooth muscle remnant, indicating that this thrombotic neutrophilic and granulomatous process involves a medium-sized muscular artery.
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