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Advances in dermatology and venereology Acta Dermato-Venereologica
Toe Gangrene Associated with Macroangiopathy in Systemic Sclerosis : A Case Series on the Unreliability of the Ankle-brachial Pressure Index
Takashi HASHIMOTO 1 , 2 , Takahiro SATOH 1 , 2 and Hiroo YOKOZEKI 1
1
Department of Dermatology , Graduate School , Tokyo Medical and Dental University , Tokyo , Japan , and 2 Department of Dermatology , National Defense Medical College , 3-2 Namiki , Tokorozawa , 359-8513 , Saitama , Japan . E-mail : tasaderm @ ndmc . ac . jp Accepted Jan 29 , 2018 ; Epub ahead of print Jan 30 , 2018
Systemic sclerosis ( SSc ) is a multi-organ fibrotic autoimmune disease ( 1 ). Although the aetiologies underlying SSc remain unclear , vasculopathies ( both micro- and macro-angiopathies ) are thought to play important roles in the pathogenesis ( 2 ). Toe gangrene due to lower limb arterial occlusion often occurs in patients with SSc ( 3 ). The ankle-brachial pressure index ( ABI ) is widely used and considered useful for detecting lower limb arterial occlusion , particularly peripheral artery disease ( PAD ) ( 4 ). We report here a series of 7 SSc patients with toe gangrene due to macroangiopathy , in whom ABI values did not reflect the severity of arterial ischaemia .
PATIENTS , MATERIALS AND METHODS ( see Appendix S1 1 )
RESULTS
Seven patients with SSc and toe gangrene were admitted to our department between April 2005 and March 2016 . One patient had gangrene of a single toe , and 6 had multiple gangrene sites ( Fig . 1a ). Detailed profiles of the patients are shown in Table SI 1 . Patients comprised 2 men and 5 women , with a mean age of 67.9 years ( range
1 https :// www . medicaljournals . se / acta / content / abstract / 10.2340 / 00015555-2897
51 – 79 years ). Five patients had limited cutaneous SSc with positive results for only anti-centromere antibody ( ACA ), one had diffuse cutaneous SSc with positive results for only anti-topoisomerase I ( Topo-I ) antibody , and one had diffuse cutaneous SSC with positive results for both ACA and anti-Topo-I antibody . All patients had Raynaud ’ s phenomenon . With regard to other vascular risk factors ( diabetes mellitus , smoking habits , hypertension , and hypercholesterolaemia ), one patient had a smoking habit , but no other risk factors , one was a smoker with hypertension , and 5 had none of these risk factors . Antiphospholipid antibodies were detected in only one patient ( patient 4 ). No patients had any other connective tissue diseases , including systemic lupus erythematosus or Sjögren ’ s syndrome .
To determine whether macroangiopathy was present , we initially assessed the traditional ABI . Generally , an ABI < 0.4 suggests severe arterial occlusion , often in association with ulcers and / or gangrene , and is defined as representing critical limb ischaemia ( CLI ) ( 4 ). Unexpectedly , 5 patients showed almost normal ABI ( ≥ 0.8 ) and 2 patients showed ABIs of 0.62 and 0.61 ( Table SI 1 ).
The “ alternative ” ABI has recently been reported to improve the prediction of mortality risk compared with traditional ABI ( 6 ). In our patients , however , only 2 patients showed values < 0.4 and 5 displayed values between 0.4 and 1.0 ( Table SI 1 ). These results led to the assumption that macroangiopathy was not present in these patients .
Next , we conducted angiographic examinations to evaluate the involvement of medium-sized arteries in the lower extremities . No evidence of atherosclerosis was seen , such as involvement of multiple vascular beds , varied lesion lengths , calcifications , or irregular plaque in any of the
Fig . 1 . ( a ) Toe gangrene and acrocyanosis ( patient 6 ). ( b , c ) Features of magnetic resonance angiography ( b , patient 1 ) and conventional catheter angiography ( c , right limb of patient 4 ) of the lower limbs . Smooth luminal tapering ( arrows ) and obliteration ( arrowheads ) with poor collateral vessel formation are detected for below-the-knee arteries . ( d – f ) Histopathological features of posterior tibial artery ( patient 6 ). ( d ) Intimal fibrosis or hyperplasia with luminal narrowing and presence of intraluminal thrombosis ( haematoxylin and eosin stain ; original magnification , × 40 ). ( e ) Acid mucopolysaccharide deposition in the intima ( colloidal iron stain , × 40 ). ( f ) Adventitial fibrosis ( Masson ’ s trichrome stain , × 40 ). doi : 10.2340 / 00015555-2897 Acta Derm Venereol 2018 ; 98 : 532 – 533
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2018 Acta Dermato-Venereologica .