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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.