Atypical Kawasaki disease with peripheral gangrene and myocardial infarction: therapeutic implications.
We describe a 2-month-old girl with atypical Kawasaki disease (KD) complicated by peripheral gangrene and myocardial infarction. Peripheral ischaemia leading to gangrene is a rare but serious complication of KD in infants younger than 7 months of age. Treatment has been targeted at reducing arterial inflammation, arteriospasm and thrombosis. We report the first patient with incomplete KD and peripheral ischaemia in whom therapy with prostaglandin E1 (PGE1) as vasodilating and antithrombotic agent appeared successful, restoring hand and foot perfusion without significant long-term sequelae. However, PGE1 could have supported development of myocardial infarction by shunting blood away from ischaemic areas distal to a giant coronary artery aneurysm with beginning thrombosis. CONCLUSION. Atypical KD with peripheral gangrene appears to react favourably to treatment with PGE1, but needs careful monitoring to detect early signs of cardiac ischaemia.
Division of Immunology/Haematology, University Children's Hospital Zürich, Switzerland.
M
M
von Planta
M
M
Fasnacht
C
C
Holm
S
S
Fanconi
R A
RA
Seger
D000527:Alprostadil / Q000008:administration & dosage* / Q000009:adverse effects; D003323:Coronary Aneurysm / Q000175:diagnosis / Q000188:drug therapy; D003326:Coronary Circulation / Q000187:drug effects; D004452:Echocardiography / Q000187:drug effects; D004562:Electrocardiography / Q000187:drug effects; D005260:Female; D005343:Fibrinolytic Agents / Q000008:administration & dosage* / Q000009:adverse effects; D005385:Fingers / Q000098:blood supply*; D005734:Gangrene; D006801:Humans; D007223:Infant; D007511:Ischemia / Q000175:diagnosis / Q000188:drug therapy*; D009080:Mucocutaneous Lymph Node Syndrome / Q000175:diagnosis / Q000188:drug therapy*; D009203:Myocardial Infarction / Q000139:chemically induced / Q000175:diagnosis / Q000188:drug therapy*; D014034:Toes / Q000098:blood supply*; D014665:Vasodilator Agents / Q000008:administration & dosage* / Q000009:adverse effects
D005343:Fibrinolytic Agents; D014665:Vasodilator Agents; D000527:Alprostadil