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Objective Digital pitting scars (DPS) are frequent, but little studied in SSc to date. Methods An analysis of SSc patients enrolled in the EUSTAR database. Primary objectives were to (i) examine DPS prevalence; (ii) examine whether DPS are associated with digital ulcers (DUs) and active digital ischaemia (DUs or gangrene); and (iii) describe other associations with DPS including internal organ complications. Secondary objectives were whether DPS are associated with (i) functional impairment; (ii) structural microvascular disease; and (iii) mortality. Descriptive statistics and parametric/non-parametric tests were used. Binary logistic regression was used to examine the association between DPS and DUs, active digital ischaemia and mortality. Results A total of 9671 patients were included with reported DPS at any time point (n  = 4924) or 'never' DPS (n  = 4747). The majority (86.9%) were female and mean age was 55.7 years. DPS were associated with longer disease and Raynaud's duration (both P  ≤ 0.001). DPS were associated with interstitial lung disease, pulmonary hypertension, conduction blocks, telangiectases, calcinosis (all P  ≤ 0.001) and joint synovitis (P  = 0.021). Patients were more likely to have more severe capillaroscopic abnormality and greater hand functional impairment. Multivariable logistic regression analyses showed that DPS were associated (odds ratio) with DUs: 22.03 (19.51–24.87), active digital ischaemia: 6.30 (5.34–7.42) and death: 1.86 (1.48–2.36). Conclusion DPS are associated with a severe disease course including death. The impact of DPS on hand function and ischaemia is significant. The presence of DPS should alert the clinician to a poor prognosis and need to optimize the therapeutic strategy. [ABSTRACT FROM AUTHOR]

Objectives Patients presenting with digital upper limb ischaemia are occasionally referred to rheumatology services to rule out vasculitis. We aimed to present two cases of delayed diagnosis of arterial thoracic outlet syndrome (aTOS) in middle-aged patients presenting with digital ischaemia in order to raise awareness of this important pathology that requires timely surgical intervention. Methods Two cases of progressive ischaemia of the right upper extremity caused by primarily undiagnosed compression of the subclavian artery by an accessory cervical rib are presented. The case notes, radiological images, intra-operative and postoperative findings for both patients were reviewed. Patients were followed up after ≥6 months to assess prognosis. Results Both patients had a working diagnosis of Buerger's disease and had been treated with prostaglandin infusions before establishment of the diagnosis of arterial thoracic outlet syndrome. Both patients were heavy smokers, and one patient had bilateral symptoms and a history of axial SpA and positive HLA-B27. Late presentation in one patient led to the loss of three fingers and the need for plastic reconstructive surgery after cervical rib resection and revascularization. In the other patient, surgical thrombectomy of the upper limb arteries along with resection of a cervical rib and repair of the subclavian artery with an interposition graft were necessitated to heal digital ulcers successfully. Conclusion A high index of suspicion of aTOS should be maintained in middle-aged patients presenting with digital or upper limb ischaemia even in the presence bilateral symptoms or relevant risk factors of other diagnoses, such as smoking or a positive rheumatological history. [ABSTRACT FROM AUTHOR]

Aims: Accidental injury to digits with Adrenaline Auto-injectors (AAIs) is becoming increasingly common. Digital AAI injury causes painful ischaemia that can lead to necrosis and patient anxiety. There is a lack of understanding amongst surgeons regarding how to manage these injuries. We aimed to determine an optimal treatment algorithm for their management.Methods: We conducted a systematic review using the search engines MEDLINE, PubMed, EMBASE, CINAHL, BNI, AMED, Google Scholar. Search items included ("epinephrine OR adrenaline") AND ("Digit" OR "Finger" OR "Thumb") AND ("Injury" OR "Accidental").Results: A total of 49 articles were identified describing 111 cases. In 58 cases; 52% of cases were managed with phentolamine, 24% were managed with nitroglycerine and 7% were treated with warm soaks. The remaining 17% of cases were managed with a variety of alternative treatments. Mean recovery time following treatment with phentolamine infiltration was 33 min, whilst symptoms persisted for several hours in some cases with observation/warm soaks and nitroglycerine. Phentolamine was more effective when injected into the AAI puncture site (mean resolution time: 17 min) in comparison to injection as a digital block (74 min).Conclusion: Phentolamine is the most effective method of reversing symptoms and treating ischaemic digits when compared to alternative therapies. Symptoms resolved much quicker when phentolamine was infiltrated into the site of injury compared to being infiltrated as a digital block. We propose a treatment algorithm for management of these injuries. Hand surgeons should be aware of AAI injuries and be able to advise on their management. [ABSTRACT FROM AUTHOR]

Following a Coronary artery bypass graft and insertion of an intra-aortic balloon pump, the 63 year-old male patient developed signs of progressive multi-organ dysfunction and ischaemic changes in both of his lower limbs. The diagnosis of cholesterol embolization syndrome was made. This condition has been noted to be a complication of invasive procedures and endovascular therapies, and is often overlooked and difficult to treat. While the patient’s renal function and pancreatitis showed improvement, the lower limb ischaemia was progressing. Eventually, the patient underwent a bilateral below knee amputation.

Catastrophic antiphospholipid syndrome is an infrequent disease in children, but of major relevance because of its high morbidity and mortality. We report the case of a child with digital ischaemia in whom, after aetiological screening, the diagnosis of catastrophic antiphospholipid syndrome was made. [ABSTRACT FROM AUTHOR]

A cohort of patients presented to Queen Victoria Hospital, UK, with iatrogenic toe ischaemia following application of a different, newly available post-procedure dressing with different properties to those usually used. This resulted in ischaemia with extensive skin and soft tissue damage, requiring debridement surgery and, in some cases, skin grafting. We aim to highlight the risk of morbidity from dressing application to the digits. This is a key learning skill for anyone who may either perform dressings or evaluate dressings on digits in the community and across multiple specialties in hospital. This article follows a thorough root cause analysis and addresses other possible causes of an acutely painful erythematous toe post-Zadek's procedure. [ABSTRACT FROM AUTHOR]

It is now increasingly recognized that some monogenic autoinflammatory diseases and immunodeficiencies cause vasculitis, although genetic causes of vasculitis are extremely rare. We describe a child of non-consanguineous parents who presented with cutaneous vasculitis, digital ischaemia and hypocomplementaemia. A heterozygous p.R1042G gain-of-function mutation (GOF) in the complement component C3 gene was identified as the cause, resulting in secondary C3 consumption and complete absence of alternative complement pathway activity, decreased classical complement activity, and low levels of serum C3 with normal C4 levels. The same heterozygous mutation and immunological defects were also identified in another symptomatic sibling and his father. C3 deficiency due GOF C3 mutations is thus now added to the growing list of monogenic causes of vasculitis and should always be considered in vasculitis patients found to have persistently low levels of C3 with normal C4.

Objectives In patients with systemic sclerosis (SSc), fingertip digital ulcers (DUs) are believed to be ischaemic, and extensor surface DUs a result of mechanical factors/microtrauma. Our aim was to assess blood flow response to topical glyceryl trinitrate (GTN) compared to placebo in SSc DUs, looking for differences in pathophysiology between fingertip and extensor lesions. Method This was a double-blind, randomised, crossover, placebo-controlled study. Sixteen (6 fingertip, 10 extensor) DUs were each studied twice (one day apart): once with GTN and once with placebo ointment. Perfusion at the DU centre (‘DUCore’) and periphery (‘DUPeriphery’), as measured by laser Doppler imaging was performed before and immediately after ointment application, then every 10 min, up to 90 min post-application. We calculated the area under the response curve (AUC) and the ratio of peak perfusion to baseline, then compared these between GTN and placebo. Results Perfusion was lower in the DUCore compared to the DUPeriphery (ratio of 0.52). The microvessels of the DUCore were responsive to GTN, with an increase in perfusion, with a similar effect in both fingertip and extensor DUs. The AUC and peak/baseline perfusion difference in means (ratio, 95% confidence interval) between GTN and placebo at the DUCore were 1.2 (1.0–1.6) and 1.2 (1.0–1.5) respectively, and at the DUPeriphery were 1.1 (0.8–1.6) and 1.0 (0.9–1.2) respectively. Conclusion DUs (both fingertip and extensor) were responsive to topical GTN, with an increase in perfusion to the ischaemic DU centre. If both fingertip and extensor DUs have a (potentially reversible) ischaemic aetiology, this has important treatment implications. [ABSTRACT FROM AUTHOR]

Objective. The aim of the study was to assess the association between plethysmographically measured vasospasms during stepwise cooling and recovery, as an index for digital ischaemia, and nailfold capillaroscopic pattern (NCP) severity in patients with primary or secondary RP, including SSc. Methods. In 381 consecutive patients with suspected RP without a history of digital ulcers, NCP (assessed by widefield videocapillaroscopy), fingertip photoelectric plethysmography during cooling and recovery and clinical characteristics were analysed. NCPs were graded as follows: normal, non-specific, early and active. The mean ischaemic time was defined as the mean time of perfusion loss during cooling and recovery of five fingers. Results. In the patients with loss of perfusion during cooling and recovery, the NCP was normal in 152, non-specific in 96, early in 61 and active in 39 patients. The mean ischaemic time was positively associated with the severity of NCP, with P < 0.05 for each two- or three-grade increase and independent of underlying SSc. The difference was most pronounced during recovery. Conclusion. We demonstrate that the degree of vasospasm and ischaemia provoked by stepwise cooling and recovery are positively associated with NCP in patients with RP of different aetiologies and without a history of digital ulcers. [ABSTRACT FROM AUTHOR]

Thrombosis may play an important role in the pathophysiology of certain complications of sickle cell disease (SCD). While the association between SCD and ulcerative colitis (UC) is still debatable, inflammatory bowel disease is known to be associated with an increased incidence of thromboembolic disease. We report a case of a 16-year old girl known to have homozygous SCD and also diagnosed with UC who presented with digital ischemia of her right lower limb. This led to gangrene and subsequent amputation of the first, second and third digits of that limb. This case highlights that patients with both UC and SCD may have an increased risk of thromboembolism and raises the question as to whether patients with UC and SCD should be screened for thrombophilia.

Following a Coronary artery bypass graft and insertion of an intra-aortic balloon pump, the 63 year-old male patient developed signs of progressive multi-organ dysfunction and ischaemic changes in both of his lower limbs. The diagnosis of cholesterol embolization syndrome was made. This condition has been noted to be a complication of invasive procedures and endovascular therapies, and is often overlooked and difficult to treat. While the patient’s renal function and pancreatitis showed improvement, the lower limb ischaemia was progressing. Eventually, the patient underwent a bilateral below knee amputation. [ABSTRACT FROM AUTHOR]

Objectives. Acro-osteolysis (bony resorption of the terminal digital tufts) is a well-recognized, but under-researched, manifestation of SSc. Our aim was to investigate the hypothesis that acro-osteolysis is associated with (i) the severity of digital ischaemia and (ii) the presence of calcinosis.Methods. This was a retrospective study of 101 patients with SSc in whom hand radiographs taken between 2001 and May 2008 were available for review. These radiographs were graded for severity of acro-osteolysis on a 0–4-point scale for each finger (0 = normal bone structure, 4 = severe pencilling of the terminal phalanges). From these scores, patients were subdivided into the following two groups: normal/minimal acro-osteolysis and moderate/severe acro-osteolysis. The presence or absence of calcinosis (mild, moderate or severe) was also documented.Results. Of the 101 patients, 68 were grouped as normal/minimal acro-osteolysis and 33 as moderate/severe acro-osteolysis. Forty-five had severe digital ischaemia: 25 (76%) of the patients with moderate/severe acro-osteolysis compared with 20 (29%) of those with normal/minimal acro-osteolysis (multifactorial analysis: P < 0.001). Patients with moderate/severe acro-osteolysis were more likely to have severe calcinosis (33% vs 13%), but this was not statistically significant after adjustment for potential confounders.Conclusion. Acro-osteolysis was strongly associated with severe digital ischaemia. The potential association with severe calcinosis merits further study. Prospective studies are required to investigate acro-osteolysis as a marker of digital vascular disease progression and of treatment response. [ABSTRACT FROM PUBLISHER]

Objectives. Advances in nail-fold capillaroscopy allow capillary abnormalities to be quantified. Our aim was to investigate, in patients with SSc, the relationships between the degree of nail-fold capillary abnormality and disease subtypes (lcSSc and dcSSc), duration of RP and the presence of (i) severe digital ischaemia (as defined by previous i.v. vasodilators, debridements or amputations), (ii) a positive ACA, (iii) clinically evident calcinosis, (iv) pulmonary arterial hypertension and (v) telangiectases. [ABSTRACT FROM PUBLISHER]

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Objective. This study addresses the issue of risk factors and management of critical peripheral ischaemia (CPI) and gangrene in SLE and proposes rituximab as a novel therapy. [ABSTRACT FROM PUBLISHER]

This study compared fingertip capillary blood gas parameters before and after digital anaesthesia using lidocaine with and without Adrenalin. Twenty patients who underwent surgery on their fingers under digital ring block anaesthesia were randomly anaesthetised with 2% lidocaine or 2% lidocaine with 1:80,000 Adrenalin. Capillary blood parameters were measured prior to the digital blocks and 15 minutes after the injections. In the plain lidocaine group, PO2 and SaO2 increased significantly after injection (P = 0.025 and 0.017, respectively). In the lidocaine with Adrenalin group, PO2 and SaO2 decreased after the injections. However, this decrease was not statistically significant. Patients in the plain lidocaine group had return of sensation to the finger tip 4.8 (0.6) hours after the operation. This period was 8.1 (0.8) hours for the lidocaine with Adrenalin group. [ABSTRACT FROM PUBLISHER]

Late onset Raynaud's, rapidly worsening, poses a diagnostic challenge. Rarely may it be associated with underlying malignancy. Associated autoantibody positivity may cause diagnostic delay. We describe three cases who sought treatment for Raynaud's subsequently diagnosed with malignancy, treatment of which improved the Raynaud's. We discuss the possible mechanisms. [ABSTRACT FROM AUTHOR]

Hypothenar hammer syndrome (HHS) is a rare entity of arterial insufficiency to the hand and is seen in patients, especially men, with a history of repetitive blunt trauma to the hypothenar eminence. It is characterized by unilateral digital ischaemia of the dominating hand, which spares the thumb, with the most often manifestations being pain, paresthesias and cold intolerance. In advanced cases trophic skin changes and necrosis of the fingertips may develop. The diagnosis is verified by angiography, showing thrombosis or aneurysm of the distal ulnar artery. Treatment and prevention of HHS consists mainly of hand protection against mechanical injury and cessation of smoking. Anticoagulants antiplatelel drugs, surgical revascularization and cervical sympathectomy may be useful in its management. Here we present the case of a fight sports instructor with critical right hand ischaemia in the course of HHS, in whom early diagnosis and treatment enabled almost complete remission of changes. [ABSTRACT FROM AUTHOR]

Hypothenar hammer Syndrome (HHS) is a condition characterised by digital ischaemia as a result of repetitive trauma to the hypothenar eminence of the hand. It occurs in people who repeatedly use the palm of the hand as a hammer to push, grind or twist objects. It is a curable and a preventable cause of upper digital ischemia. In this report we present a case of HHS and discuss the causes and pathogenesis of this syndrome. We review the incidence, clinical characteristics, differential diagnosis, investigation and treatment. [Copyright &y& Elsevier]

Thrombosis may play an important role in the pathophysiology of certain complications of sickle cell disease (SCD). While the association between SCD and ulcerative colitis (UC) is still debatable, inflammatory bowel disease is known to be associated with an increased incidence of thromboembolic disease. We report a case of a 16-year old girl known to have homozygous SCD and also diagnosed with UC who presented with digital ischemia of her right lower limb. This led to gangrene and subsequent amputation of the first, second and third digits of that limb. This case high- lights that patients with both UC and SCD may have an increased risk of thromboembolism and raises the question as to whether patients with UC and SCD should be screened for thrombophilia. [ABSTRACT FROM AUTHOR]

Catastrophic antiphospholipid syndrome is an infrequent disease in children, but of major relevance because of its high morbidity and mortality. We report the case of a child with digital ischaemia in whom, after aetiological screening, the diagnosis of catastrophic antiphospholipid syndrome was made., (Copyright © 2020 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

• Suggested postoperative management of ischaemic lesser toes after correction. • Immediate recommendations involve encouraging return of blood flow. • Demarcation should be allowed to occur before pursuing definitive treatments. Ischemia after correction of lesser toe deformities is usually due to temporary vasospasm and can rarely cause gangrene. The published literature on dealing with the issue and been reviewed and used to suggest an algorithm for a logical step by step approach to a pale or white toe when encountered in the postoperative period. [ABSTRACT FROM AUTHOR]

: A 37-year-old man with ischaemic finger necrosis and recent-onset Raynaud’s phenomenon associated with cocaine abuse is reported. Initial therapy with systemic vasodilators, low-molecular-weight heparin and aspirin failed. Resolution of the ischaemia and ulcer healing was rapidly achieved with intravenous infusions of the prostacyclin analogue iloprost. The mechanism of vascular ischaemic injury and the development of secondary Raynaud’s phenomenon due to cocaine use is discussed. [ABSTRACT FROM AUTHOR]

Objectives: Patients presenting with digital upper limb ischaemia are occasionally referred to rheumatology services to rule out vasculitis. We aimed to present two cases of delayed diagnosis of arterial thoracic outlet syndrome (aTOS) in middle-aged patients presenting with digital ischaemia in order to raise awareness of this important pathology that requires timely surgical intervention., Methods: Two cases of progressive ischaemia of the right upper extremity caused by primarily undiagnosed compression of the subclavian artery by an accessory cervical rib are presented. The case notes, radiological images, intra-operative and postoperative findings for both patients were reviewed. Patients were followed up after ≥6 months to assess prognosis., Results: Both patients had a working diagnosis of Buerger's disease and had been treated with prostaglandin infusions before establishment of the diagnosis of arterial thoracic outlet syndrome. Both patients were heavy smokers, and one patient had bilateral symptoms and a history of axial SpA and positive HLA-B27. Late presentation in one patient led to the loss of three fingers and the need for plastic reconstructive surgery after cervical rib resection and revascularization. In the other patient, surgical thrombectomy of the upper limb arteries along with resection of a cervical rib and repair of the subclavian artery with an interposition graft were necessitated to heal digital ulcers successfully., Conclusion: A high index of suspicion of aTOS should be maintained in middle-aged patients presenting with digital or upper limb ischaemia even in the presence bilateral symptoms or relevant risk factors of other diagnoses, such as smoking or a positive rheumatological history., (© The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology.)

HOLSTEIN A., BÄTGE R. & EGBERTS E.-H. (2010) European Journal of Cancer Care 19, 408–409 Gemcitabine induced digital ischaemia and necrosis A 70-year-old woman presented with a 7-day history of severe pain, paresthesia, oedema, acrocyanosis and punctate haemorrhagic lesions on her fingertips. The complaints began 2 days after the second cycle of a first-line chemotherapy consisting of cisplatin or carboplatin, and gemcitabine due to advanced urothelial carcinoma. At the fingertips of both hands, haemorrhagic and partly ulcerative lesions were found; these were attributed to vascular toxicity of gemcitabine. Therapeutically sympathicolysis by bilateral blockade of the brachial plexus was performed, accompanied by intravenous administration of the prostacyclin analog iloprost, fractionated heparin subcutaneously and oral therapy with corticosteroids and aspirin. Digital amputation could be avoided. Acral ischemia is a rare but probably underreported adverse effect of gemcitabine therapy and a potential source of misdiagnosis. [ABSTRACT FROM AUTHOR]

Adrenaline autoinjectors are widely prescribed for self-treatment of anaphylactic syndromes. Accidental misfire into digits can cause prolonged significant ischaemia with serious outcomes. Phentolamine is a readily available treatment, which is easily and safely given in the emergency setting. [ABSTRACT FROM AUTHOR]

Coronary angiographies that are performed via the radial artery generally have lower bleeding complications, however, patients are at risk of radial artery occlusion, with resultant digital ischaemia. This report describes a case of digital ischaemia after transradial coronary angiography in a patient with essential thrombocytosis. Risk factors for thrombo-occlusive complications, and potential prevention strategies are also explored. [ABSTRACT FROM AUTHOR]

Raynaud's phenomenon (RP) is a major cause of pain and disability in patients with autoimmune connective tissue diseases (CTDs), particularly systemic sclerosis (SSc). The clinician must perform a comprehensive clinical assessment in patients with RP to differentiate between primary (idiopathic) and secondary RP, in particular (for rheumatologists), secondary to an autoimmune CTD, as both the prognosis and treatment may differ significantly. Key investigations are nailfold capillaroscopy and testing for autoantibodies (in particular, those associated with SSc). Patients with RP and either abnormal nailfold capillaroscopy or an SSc-specific antibody (and especially with both) have a high risk of transitioning to an autoimmune CTD. Both nailfold capillaroscopy and autoantibody specificity may help the clinician in predicting organ-based complications. The management of CTD-associated RP requires a multifaceted approach to treatment, including patient education and conservative ('non-drug') measures. Patients with CTD-associated RP often require pharmacological treatment, which in the first instance is usually a calcium channel blocker, although other agents can be used. There is an increasing tendency to use phosphodiesterase type 5 inhibitors early in the treatment of CTD-associated RP. Oral therapies are commonly associated with side effects (often due to systemic vasodilation) that may result in failure of dose escalation and/or permanent discontinuation. Intravenous prostanoid therapy and surgery (e.g., botulinum toxin injection and digital sympathectomy) can be considered in severe RP. Patients with CTD-associated RP can develop a number of ischaemic digital complications (primarily ulcers and critical ischaemia), which may be associated with significant tissue loss. Future research is required to increase the understanding of the pathogenesis and natural history of RP (to drive therapeutic advances), and to explore/develop drug therapies, including those that target the mechanisms mediating cold-induced vasoconstriction, and locally acting therapies free of systemic side effects., (Copyright © 2016. Published by Elsevier Ltd.)

Abstract: Introduction: Elbow arterio-venous fistulas (AVFs) may lead to debilitating hand ischaemia, even after several years of successful haemodialysis. If impaired arterial inflow is identified as the cause of ischaemia, endovascular techniques are usually successful in restoring hand perfusion. Report: This paper reports of a patient with severe haemodialysis access-induced distal ischaemia (HAIDI) due to subclavian artery occlusion 5 years after AVF construction. As both endovascular and open carotid or subclavian surgical techniques were contraindicated, recovery from hand ischaemia was achieved by the use of a femoro-axillary bypass graft. Discussion: The common femoral artery is a viable alternative inflow location for bypass grafting in HAIDI if commonly used upper body arterial sources are less suitable or unavailable. [Copyright &y& Elsevier]