Aortic and mitral valve replacement in a patient with acute febrile neutrophilic dermatosis (Sweet's syndrome): Report of a case

A 29-year-old woman was admitted to our hospital with severe orthopnea, fever, and acute dermatosis. She had a 5-year history of episodic acute neutrophilic dermatosis and peripheral leukocytosis following a high fever, which were symptoms consistent with a diagnosis of Sweet's syndrome. Echocardiography revealed remarkable dysfunction of the left ventricle due to severe aortic regurgitation, which had not been present at a previous admission when mild mitral regurgitation was detected. The aortic and mitral valves were replaced with prosthetic valves on an emergency basis. The leaflets of the aortic valve were very thin and appeared fragile. The anterior leaflet of the mitral valve showed severe prolapse due to the torn chordae and hypoplasia of the posterior strut chordae. Her postoperative course was uneventful. Microscopic examination revealed fibrosal degeneration and the infiltration of lymphocytes and macrophages into both heart valves. This may be the first case report of valvulitis and Sweet's syndrome occurring simultaneously.