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Table 1 Review of cases of Takayasu arteritis with pyoderma gangrenosum in the literature

From: Takayasu arteritis with pyoderma gangrenosum: case reports and literature review

Authors, country, year Sex Racial or ethnic group Location of PG Age at onset of the first manifestations, years Therapy Systemic symptoms First vascular manifestation Vascular involvement Other complications
Systemic Cutaneous Vascular
Okamura et al. [6] Japan, 2017 M Japanese First in left buccal region and then spread to cheek, trunk and lower legs 33 37 33 Betamethasone 1 g/d and tacrolimus 3 mg/d for TA, prednisolone 50 mg/d for PG Fever Left cervical pain followed by dizziness   
Vettiyil et al. [7], India, 2017 F Indian Dorsum of left foot 8 10 8 Prednisolone (1 mg/kg/d) and mycophenolate mofetil for TA and PG   Pain andclaudication pain over legs and back with breathlessness on exertion Dilation of the ascending aorta, the left common carotid artery, left subclavian, right subclavian and axillary were narrowed at the origin and proximal portion Chronic recurrent multifocal osteomyelitis
Loetscher et al. [3], Switzerland, 2016 F Caucasian Lower extremities No record 42 43 Prednisone 50 mg/d, cyclosporine, methotrexate    Proximal occlusion of the left subclavian artery, wall thickening of the aortic arch and the subclavian artery Erythema nodosum
Barrera-Vargas et al. [2], Mexico, 2015 F Mexican First in lower limbs and then to head, neck, upper and lower extremities 20 20 26 Prednisone 1.5 mg/kg/d and thalidomide 200 mg/d for PG, three methylprednisolone pulses (1 g/d) and prednisone 1 mg/kg/d and methotrexate 15 mg/week for TA Fever Sudden amaurosis in left eye Both the carotid and vertebral system, all the supra-aortic branches, right pulmonary artery CT revealed a cystic lesion with an air-fluid level in the lower right pulmonary lobe
Barrera-Vargas et al. [2], Mexican, 2015 M Mexican Neck, trunk, upper and lower extremities 25 17 26 Prednisone and cyclosporine, and later thalidomide 200 mg/d, dapsone 100 mg/d, azathioprine 100 mg/d for PG. Prednisone 1 mg/kg/d, thalidomide 200 mg/d, dapsone 100 mg/d, azathioprine 100 mg/d for TA No Light-headedness and syncope Aortic dilatation, narrowing thickening of the wall of the left carotid artery, and celiac trunk stenosis, as well as thickening at the origin of the superior mesenteric artery and left renal artery Pulmonary nodules,histopathologic analysis showing granulomatous chronic inflammation with giant cells
Futaki et al. [8], Japan, 2009 F Japanese Neck, face and forearm 28 28 29 Prednisolone 30 mg/d Intermittent fever, sore throat and cough   Pulmonary artery TA without typical symptoms
Minagawa et al. [9], Japan, 2009 F Japanese Left lower leg 21 16 21 Tacrolimus for PG, prednisolone 25 mg/d for TA and necrotizing vasculitis Episodic general fatigue and low-grade fever Dysaesthesia in arms Stenosis and thickened walls in the subclavian, left carotid and right renal arteries Necrotizing vasculitis with lobular panniculitis in the deep dermis
Ghosn et al. [10], Lebanon, 2008 F Lebanese Face, trunk and extremities 7 7 7 Solumedrol 0.5 mg/kg/d, followed by infliximab 100 mg at weeks 0, 2, and 6   Acute-onset right wrist drop Marked aneurysmal dilatation of the ascending aorta with occlusion of the left and right subclavian arteries, in addition to a large aneurysmal pouch in the right axillary area compressing the nerve plexus Diagnosed as RP for 5 years
Aoussar et al. [11], France, 2007 F Caucasian Forearm No record 21 23 Prednisone     
Kanaemistu et al. [12], Japan, 2005 F Japanese Neck, chest and arm No record 34 34 Prednisolone and cyclosporine A, surgery    Aneurysm from the ascending aorta to the aortic arch Thoracic aortic aneurysm
Ujiie et al. [13], Japan, 2004 F Japanese First limited to lower legs and then extended to buttocks, lower trunk, upper limbs and scalp 21 29 21 Prednisolone 20 mg/d for TA, prednisone 50 mg/d, cyclosporine 6 mg/kg/d Hypertension and dizziness Diminished arterial pulse intensity and bruits Stenosis in the right subclavian, right vertebral and right carotid arteries and occlusions in the left subclavian and left carotid arteries  
Fearfield et al. [14], U.K., 1999 F Caucasian Perineum, forearms and upper arms 27 27 33 Prednisone 150 mg/d, intravenous immunoglobulin, minocycline 200 mg/d, later methotrexate 7.5 mg/week and cyclosporine 5 mg/kg/d Mild influenza-
like symptoms, including general muscle aches
Heaviness in arms Bilateral proximal subclavian artery occlusions with slight narrowing of the right vertebral artery origin  
Dagan et al. [15], Israel, 1995 M Israeli Face and extremities 4 9 months 4 Prednisone 2 mg/kg/d and dapsone 5 mg/kg for PG, high-dose prednisone and surgery for TA Restless, tired and febrile Absence of both radial pulses Severe aneurysmatic dilation of the ascending aorta, severe stenosis of the left subclavian artery Sterile osteomyelitis
Fullerton et al. [16], 1991 F Saudi Arabian Abdomen, followed by rapid spread to legs No record 28 No record Prednisone, cyclosporine     
Frances et al. [4], France, 1990 F Portuguese Left arm 32 32 33 Prednisolone 0.5 mg/kg/d Fever, arthralgia Absence of left radial pulse Supra-aortic arteries, abdominal aorta  
Perniciaro et al. [17], USA, 1987 M   Left popliteal area 17.5 19 19 Systemic corticosteroids Intermittent fever    
Case 1 F Chinese Bilateral thighs and buttocks, upper extremities 24 24 28 Prednisolone 40 mg/d, cyclosporine 0.1 g/d, triptolide 60 mg/d Fever Dizziness, pulseless in both upper limbs The right innominate artery (subclavian artery, common carotid artery) was completely obliterated, and the left common carotid artery was entirely occluded. The initial segment of the left vertebral artery had stenosis, and the left vertebral artery was enlarged  
Case 2 F Chinese Both lower and upper extremities, left buccal region, face No record 17 24 Methylprednisolone   Left arm weakness and dizziness The left carotid artery, left subclavian artery and bilateral femoral arteries, the superficial femoral arteries and left popliteal artery, the ascending aorta,aortic arch and its branches, descending aorta, abdominal aorta. The celiac stem and mesenteric artery were occluded, with multiple collateral circulation formation. The main hepatic artery and splenic artery  
  1. PG pyoderma gangrenosum, TA Takayasu arteritis, RP relapsing polychondritis