a 36-year - old man visited the emergency department of inje university haeundae paik hospital with severe pain in both lower limbs for 1 day . a physical examination revealed pale , pulseless , and cold lower limbs .
one year ago , he had been diagnosed with kimura disease ( kd ) after excision of a right upper limb mass ( fig .
the initial laboratory findings were as follows : leukocyte count of 45.510/l with 74% eosinophils , elevated serum fibrinogen degradation product ( fdp ) level ( 18.7 g / ml ) , elevated d - dimer level ( 5.1 g / ml ) , and extremely elevated serum immunoglobulin ( ig ) e ( > 2,500 iu / dl ) .
a computed tomography ( ct ) angiogram showed total occlusion of both popliteal arteries ( pas ) ( fig .
under the presumptive diagnosis of acute limb ischemia ( ali ) , the patient underwent an emergency thrombectomy of both femoral arteries using a fogarty catheter ( edwards laboratories , santa ana , ca , usa ) .
after the operation , the pain and color changes resolved , but the right foot drop remained . to prevent thromboembolic events ,
immunochemical tests for autoimmune diseases , including lupus anticoagulant , anticardiolipin igg and igm antibodies , anti - beta2-glycoprotein igg and igm , and anti - phospholipid antibodies , were negative .
protein s activity was low , but the levels of the total and free protein s antigen were normal , suggesting a type - ii protein s deficiency .
an echocardiogram was performed ; it revealed no evidence of a cardiac thrombus or decreased systolic function of the left ventricle ( ejection fraction=47% ) . under the presumptive diagnosis of kd - related ali
, the patient was administered steroid therapy . a follow - up ct angiogram on postoperative day ( pod )
brachial index ( abi ) was 0.87 on the right side and 0.62 on the left .
coronary angiography ( cag ) on pod 5 showed triple - vessel disease with chronic total occlusion of the left anterior descending artery ( lad ) , left circumflex artery ( lcx ) , proximal obtuse marginal artery , and posterior descending artery ( fig .
stent insertion for the coronary artery was delayed because we needed to observe the kd activity and plan the second operation for the left pa .
we performed the second operation on pod 17 because of the low abi of the left lower limb .
a thrombus and a hypertrophic , injured intimal layer were observed after the longitudinal arteriotomy ( fig .
after the removal of the thrombus and the injured intimal layer , a patch angioplasty using bovine pericardium ( vascu - guard biovascular inc . , st .
2e ) : ( 1 ) thrombosis occluding the vessel lumen and intimal thickening with fibroblastic proliferation ( the intima showed reactive changes with fibroblastic proliferation ) were observed .
( 2 ) the thrombotic material contained a prominent number of eosinophils , and mixed inflammatory cell infiltration composed of eosinophils and lymphocytes was observed in the vessel wall .
two months after the first discharge , the patient returned to the emergency department with right lower extremity pain and a color change that had developed the day before .
a ct angiogram showed total occlusion from the right superficial femoral artery to the distal pa ( fig .
we performed an emergency thrombectomy , partial endarterectomy , and patch angioplasty on the right side .
the operative findings on the right side were the same as the findings on the left side .
a coronary stent was inserted into the lad , and the lcx was balloon dilated ( fig .
the patient was then treated with aspirin , clopidogrel , and cilostazol for preventing in - stent thrombosis and prednisolone for kd .
kd is characterized by marked eosinophilia , an elevated ige level , and recurrent subcutaneous nodules around the head and neck .
however , the increased serum ige level and eosinophilia suggest an allergic disease [ 14 ] . despite an unclear mechanism
, hypereosinophilia can lead to a hypercoagulable condition and vasculitis according to some published reports [ 57 ] . a possible mechanism
is that the cytokines secreted by eosinophils , including eosinophil cationic protein , eosinophil peroxidase , and major basic protein , activate platelet aggregation .
some researchers have demonstrated that human eosinophils contain various amounts of the tissue factor ( tf ) and have concluded that relatively high tf expression in patients with hypereosinophilia may contribute to an increased thrombotic risk . in this case
however , kd has been treated with local mass excision , steroids for systemic inflammation , revascularization , and anticoagulation for organ ischemia .
some researchers have reported that radiotherapy was a successful treatment for kd . in our patient ,
furthermore , we performed a partial endarterectomy and patch angioplasty for revascularization . additionally , the patient was administered prednisolone to control the activity of kd . his serum leukocyte and eosinophil counts and fdp and serum ige levels normalized .
many revascularization methods are available for ali , including thrombectomy , stent insertion , patch angioplasty , and extra - anatomic bypass with a synthetic or saphenous vein graft .
patch angioplasty is more effective for removing a thrombus and widening the lumen than a stent and is easier than a bypass .
cag to evaluate other forms of vasculitis revealed coronary artery obstructive disease ( caod ) , and we inserted a coronary stent instead of performing a bypass . because of the patient s young age , coronary artery bypass grafting was the treatment of choice for caod .
the mechanism of caod in our patient was different from that of caod in other cases .
however , antiplatelet therapy was needed , and the patient required long - term follow - up . in conclusion , kd is a systemic disease that affects many organs and presents with thromboembolism and vasculitis . in a patient with kd , physicians should evaluate the vascular system , including the coronary arteries .
 kimura disease ( kd ) is an immune - mediated chronic inflammatory disease of unknown etiology . 
 kd has many complications associated with hypereosinophilia , including various forms of allergic reactions and eosinophilic lung disease . 
 additionally , hypereosinophilia is associated with hypercoagulability , which may lead to thromboembolic events . 
 a 36-year - old man with kd presented with acute limb ischemia and coronary artery occlusion . 
 he underwent thrombectomy , partial endarterectomy of both popliteal arteries , and coronary artery stent insertion . 
 kd is a systemic disease that affects many organs and presents with thromboembolism and vasculitis . in a patient with kd 
 , physicians should evaluate the vascular system , including the coronary arteries . 
Case report
Discussion