title: Sweet syndrome, cutis laxa, and fatal cardiac manifestations in a 2-year-old girl.
abstract: A 2-year-old black girl was diagnosed with Sweet syndrome and cutis laxa, and she was given corticosteroid therapy. At that presentation, a cardiac evaluation revealed nothing unusual. Nine days later, she emergently presented with respiratory distress, and circulatory collapse rapidly developed. A 2-dimensional Doppler echocardiogram showed a dilated and poorly contractile left ventricle, severe aortic regurgitation, and a large aneurysm of the sinus of Valsalva. Despite resuscitative efforts, the child died. Inspection on autopsy revealed a markedly enlarged heart and 2 large aneurysms of the sinus of Valsalva. Histologic analysis disclosed acute necrosis in the cardiac apex and interventricular septum, and focal chronic inflammatory and granulation tissue in the aortic wall.Because cardiac lesions may remain clinically silent, we recommend that children with Sweet syndrome and cutis laxa undergo complete cardiac evaluation, including 2-dimensional Doppler echocardiography, by a pediatric cardiologist. Subsequent monitoring is also appropriate. Early recognition and aggressive therapeutic treatment could prevent fatal cardiac complications.
authors: Section of Pediatric Cardiology, University of Chicago Comer Children's Hospital, Chicago, Illinois 60637-1470, USA.
authors: Malini
authors: M
authors: Guhamajumdar
authors: Brojendra
authors: B
authors: Agarwala
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001014:Aortic Aneurysm; D001022:Aortic Valve Insufficiency / Q000209:etiology; D001344:Autopsy; D002675:Child, Preschool; D003483:Cutis Laxa / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D015150:Echocardiography, Doppler; D017809:Fatal Outcome; D005260:Female; D006331:Heart Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D006801:Humans; D012850:Sinus of Valsalva; D016463:Sweet Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D018487:Ventricular Dysfunction, Left / Q000209:etiology
chemical_list: D000305:Adrenal Cortex Hormones