Low-density lipoprotein apheresis for corticosteroid-resistant skin lesions caused by cholesterol crystal embolism: a case report and review of the literature.

Cholesterol crystal embolism (CCE) is an arterio-arterial embolism originating from the breakdown of atherosclerotic plaques in the aortic wall. The embolism affects the skin and kidney particularly, as well as frequently affects the gastrointestinal tract and other organs. Although there are no clearly effective direct therapies for CCE, corticosteroid therapy and combination therapy with low-density lipoprotein apheresis (LDL-A) followed by corticosteroids were recently reported to be effective for renal manifestations in some cases. However, few cases offer suggestions for the treatment of skin lesions caused by CCE. We report here a case of a 58-year-old man diagnosed with CCE with skin manifestations and kidney dysfunction who achieved complete remission after LDL-A. LDL-A may be a useful treatment for CCE, particularly in cases with skin manifestations.

Superficial femoral artery thrombosis as a cause for distal embolism in primary antiphospholipid syndrome.

Antiphospholipid syndrome is a diagnosis with the clinical manifestations of thromboses in the presence of an antiphospholipid antibody. A 25-year-old man with a history of deep venous thrombosis, pulmonary emboli, and myocardial infarction, and receiving long-term anticoagulation with warfarin, all due to primary antiphospholipid syndrome, presented with blue toe syndrome from a primary superficial femoral artery thrombus. He was anticoagulated with fondaparinux in addition to dipyridamole and aspirin perioperatively. The area of thrombus was resected and reconstructed using a cephalic vein interposition graft. This report reviews antiphospholipid syndrome and identifies potential questions and problems relating to a rare clinical presentation.

LDL apheresis for cholesterol embolism following coronary artery bypass graft surgery--a case report.

A 76-year-old man without any prior history of abnormal urinalysis findings or renal insufficiency demonstrated mild renal dysfunction after coronary bypass graft surgery (CABG). Two months after CABG, pain and blueness in the toes (blue toe syndrome) appeared and, the serum creatinine level (S-Cr) increased from 1.2 to 2.0 mg/dL. On admission (3 months later), the urinary protein level was 0.5 g/day, white blood cell count 8,300/microL with eosinophils (Eo) 10.5%, S-Cr 2.1 mg/dL, and low-density lipoprotein (LDL) 106 mg/dL. Acute renal failure and blue toe syndrome due to a cholesterol embolism (CE) were diagnosed. Alprostadil 40 microg/day orally for 2 weeks and alprostadil 40 microg/day intravenously were used for 5 weeks, and Eo were 250/microL, S-Cr 2.5 mg/dL; however, blue toe syndrome gradually developed. At 8 weeks after admission, limaprost alfadex 30 microg/day orally was used for 3 weeks. However, the Eo gradually rose to 1,520/microL, S-Cr to 3.0 mg/dL, and LDL to 135 mg/dL, and LDL apheresis was therefore performed 20 times for CE. The data just after LDL apheresis was performed 10 times were as follows: Eo 1,120/microL, S-Cr 4.0 mg/dL, and LDL 89 mg/dL, and blue toe syndrome had disappeared. At 10 months after the first LDL apheresis, the Eo were 630/microL, S-Cr 2.9 mg/dL, and LDL 109 mg/dL. As a result, LDL apheresis was found to be beneficial for the treatment of CE with acute renal failure and blue toe syndrome after CABG.

Angioplasty with stenting is effective in treating blue toe syndrome.

Blue toe syndrome is a manifestation of distal embolization associated with significant pain and risk of tissue loss. The recommended treatment options for this problem include endarterectomy or bypass with exclusion of the source of emboli. Although focal arterial stenosis can be effectively treated with angioplasty,it is unclear whether performing angioplasty in a lesion suspected of causing distal embolization might actually worsen the condition or what long-term effects this would have in preventing future embolization. The purpose of this study was to evaluate the treatment and outcome of a series of patients with unilateral blue toe syndrome treated with percutaneous angioplasty and stenting. During a 5-year period, a total of 8 patients were identified with unilateral blue toe syndrome. Ankle/brachial indices (ABIs) were obtained, followed by arteriography. The study group included 4 men and 4 women with an age range of 35 to 83 years. Their atherosclerotic risk factors included smoking (8), hypertension (5), diabetes mellitus (3), and hypercholesterolemia (1). One patient had a history of illicit drug use. The patients were followed up by repeat clinical examinations and vascular laboratory studies. Arteriography typically demonstrated a focal preocclusive lesion with thrombus at the distal end of the lesion. Angioplasty and stent placement was technically successful in all cases. The ABIs increased following angioplasty (before 0.81 +/- 0.05; after 1.02 +/-.05). The symptoms resolved in all 8 patients over the ensuing month, and there were no recurrences with a mean follow-up of 18.5 months (range 4 to 36 months). There was 1 death at 4 months associated with preexisting colon carcinoma. Unilateral arterial to arterial emboli were found in association with focal preocclusive lesions. Despite the presence of thrombus in some of the lesions, these patients were not acutely worse following angioplasty. There was good initial angiographic success in all cases. There was also hemodynamic improvement as shown by the increased ankle/brachial indices. Although long-term follow-up is not available, these intermediate results suggest that angioplasty and stenting should be considered a reasonable alternative to standard operative approaches for patients with blue to syndrome associated with embolization from a focal stenosis.

Temporary spinal cord stimulation for peripheral cholesterol embolism.

Cholesterol embolism is often an unrecognized complication of some cardiac and vascular procedures (i.e. coronarography, angioplasty, aortocoronary bypass, abdominal aortic aneurysmectomy) and of therapies affecting coagulation (thrombolysis, anticoagulation). The degree of pain associated with ischaemic and necrotic lesions secondary to cholesterol embolism involving the lower limbs is disproportionate to the extension of tissue involvement. Spinal cord stimulation (SCS) has been recognized as effective in relief of pain of ischaemic and neuropathic nature, although its mechanism of action is still not completely clear. The authors are unaware of previous reports of peripheral cholesterol embolism treated by SCS. Two case reports of inferior limb ischaemia secondary to cholesterol embolism in patients who had undergone cardiac invasive procedures. Temporary surgical implantation of SCS devices, which were removed after 4 to 6 weeks. Pain relief was achieved within 1 to 4 hours of surgical procedure. Any analgesic medications could be immediately discontinued. Pain control was effective and normal daily activities were rapidly regained. Ischaemic lesions healed within 4 to 6 weeks of SCS. Pain control is the most critical aspect of the management of peripheral cholesterol embolism without visceral organ involvement. SCS provided effective pain relief in the reported cases and its established ability to improve peripheral microcirculation allowed rapid resolution of necrotic lesions. Temporary SCS should be considered in the management of painful necrotic skin lesions secondary to iatrogenic cholesterol embolism.

Microembolization from atheroembolic disease or aneurysm. A case study.

Cyanosis of the digits may have several etiologies ranging from trauma to connective tissue disease; however, the most common cause of the so-called blue toe syndrome is atheroembolic disease or aneurysm and is frequently misdiagnosed on initial presentation. Pedal pulses are often palpable which may misdirect the physician from a diagnosis of vascular pathology. Furthermore, the proximal source of embolic shower may be far from the sight of symptoms. Noninvasive vascular testing, peripheral angiography, abdominal and popliteal ultrasonography, and echocardiography are all techniques that may be beneficial in discovering the origin of emboli. Atheroembolisms and aneurysms can be limb-threatening or life-threatening and hence early diagnosis is imperative.

[Blue toe syndrome after coronary artery bypass grafting].

We report three cases of blue toe syndrome (BTS) after coronary artery bypass grafting (CABG). All patients were cyanotic and exhibited painful toes two to four weeks after CABG. They were treated with antiplatelet and anticoagulant agents, and one patient underwent replacement of the abdominal aorta. Thus, BTS may occur after CABG with coronary angiography, extra-corporeal circulation or intraaortic balloon pumping. For the treatment of BTS, surgery remains the most effective option.

Compression of the dorsalis pedis artery: a novel cause of blue toe syndrome.

Blue toe syndrome (BTS) is an important vascular condition characterized by painful blue discoloration of one or more digits. It is frequently due to emboli and is important because of the risk of progressive ischemia and tissue loss. A 53-year-old male presented with recurrent episodes of painful blue discoloration and blistering of the skin of the right hallux. On examination, the patient was found to have a cool, blue-purple great toe; all peripheral pulses were present. The patient was investigated for coagulopathy and potential sources of emboli, but the only abnormality was significant stenosis of the dorsalis pedis artery due to extrinsic compression by the extensor hallucis brevis tendon. In the absence of any other embolic source or abnormality, we believe that this case presents a novel and potentially remediable cause of BTS and indicates the need for a careful search for an underlying lesion when common causes of BTS have been excluded.

Síndrome del dedo azul / Blue finger syndrome

El síndrome o signo del dedo azul (SDA) es una entidad poco frecuente causada por la oclusión de vasos periféricos distales. Se manifiesta como una coloración inicialmente azulada de uno o más dedos, referidos primero en las extremidades inferiores, en ausencia de traumatismo previo, y de etiología múltiple. La importancia de establecer un diagnóstico temprano y tratamiento oportuno es evitar la evolución extrema de necrosis o la pérdida de la vida del paciente.

The blue finger syndrome (or sign) is a rare entity caused by distal occlusion of peripheral vessels, which initially manifested as bluish discoloration of one or more fingers, first descriptions described in lower extremities in the absence of previous trauma of multiple etiologies; the importance of early diagnosis and treatment to prevent extreme changes in necrosis or loss of patient life.

Síndrome de dedos azules asociado a síndrome antifosfolipídico secundario a sífilis / Blue toe syndrome related to antiphospholipidic syndrome secondary to syphillis

El síndrome de dedos azules o fenómeno de acrocianosis, descrito en la literatura, se presenta secundario a una alteración en la vasculatura periférica que puede obedecer a fenómenos inflamatorios o vasculopatías oclusivas, de etiología infecciosa (directa o inmunológica por inmunocomplejos) así como no infecciosa (trastornos de la coagulación, enfermedades autoinmunes primarias o secundarias). Se presenta el caso de un paciente con acrocianosis secundaria a síndrome antifosfolipídico cuya etiología fue la infección por sífilis. El diagnóstico y tratamiento oportuno tuvo un desenlace favorable sin secuelas discapacitantes para el paciente.

Blue toe syndrome or acrocyanosis phenomenon have been previously described in the literature; it is secondary to peripheral vasculature dysfunction due to inflammation or occlusive vasculopathies which can also be secondary to infectious etiologies (directly or immunologically by immunocomplexes) and non infectious etiologies (coagulation disorders, primary or secondary autoimmune diseases). We report the case of a patient with acrocyanosis secondary to antiphospholipidic syndrome which etiology was syphilis. Prompt diagnose and treatment had a favorable outcome without dysfunctional consequences.

Blue toe syndrome: treatment with intra-arterial stents and review of therapies.

PURPOSE: To determine if intra-arterial stent placement can adequately treat lesions producing microemboli to the lower extremities. MATERIALS AND METHODS: During a 6.5-year period, 15 patients presenting with blue toe syndrome had 16 presumed embolic lesions treated with intra-arterial stents. These patients were evaluated during routine clinical follow-up during a 6-month period. This evaluation included physical and noninvasive arterial examinations. When patients could not return for follow-up, hospital, clinical, vascular laboratory, and radiology records were reviewed to assemble the appropriate information. Outcomes included symptoms of recurrent emboli, amputation, and death. RESULTS: Treated embolic lesions included two aortic stenoses, three bilateral iliac artery stenoses, nine unilateral iliac artery stenoses (one patient received separate treatment of unilateral iliac lesions), and two superficial femoral artery stenoses. Patients were followed-up for a mean of 18 months. Eight of 15 patients (53%) were improved or stable without complications. There were eight negative outcomes experienced in seven patients. Three patients (20%) were deceased at follow-up. Four patients (27%) had undergone amputation; one transmetatarsal amputation and three below-the-knee amputations. Only one of these was related to progressive disease in the treated extremity (7%). One patient (7%) experienced recurrent embolic symptoms. Stents were patent in all patients. CONCLUSION: Patients with blue toe syndrome are at high risk of limb loss and mortality despite treatment. Intra-arterial stent placement provides an alternative to standard surgical treatment. Further studies are needed to define the optimum therapy.

Evaluation and management of cholesterol embolization and the blue toe syndrome.

The blue toe syndrome is characterized by tissue ischemia secondary to cholesterol crystal or atherothrombotic embolization leading to occlusion of small vessels. Embolization occurs typically from an ulcerated atherosclerotic plaque located in the aorto-iliac-femoral arterial system. Clinical presentation can range from a cyanotic toe to a diffuse multiorgan systemic disease that can mimic other systemic illness. Mortality can be higher than 70% depending on the scope of the illness. Embolization can occur spontaneously or from a variety of insults such as invasive vascular procedures, anticoagulation, or thrombolytic therapy. Angiography, duplex ultrasonography, computerized tomographic scanning, and magnetic resonance imaging have been used to image the offending lesions, with angiography considered the "gold standard" despite its inherent risks. Recently, transesophageal echocardiography has been shown to be a helpful tool in imaging the thoracic aorta and delineating in great detail the anatomy of the aortic atheroma. At present, surgery remains the most viable treatment option. However, we look to the future for large randomized trials to help predict embolization and thus the proper medical therapy.