Iron deficiency-induced thrombocytosis presenting with blue toe syndrome.

Although the etiology of blue toe syndrome is varied, the association between blue toe syndrome and iron deficiency-induced thrombocytosis (IDIT) has not been well established. We report the case of a 38-year-old Saudi male who presented with blue toe syndrome and laboratory investigations revealed severe thrombocytosis secondary to iron deficiency. The patient was managed with analgesics, antiplatelets, anticoagulation, intravenous fluids, and iron supplementation. Subsequently, his symptoms resolved within a few days. IDIT is crucial to consider as a possible cause of microvascular thrombosis disorders, especially in patients with severe thrombocytosis.

Dermatological Lesions of Cholesterol Embolisation Syndrome and Kaposi Sarcoma Mimic Primary Systemic Vasculitis: Case report study.

Primary systemic vasculitis can present with a wide spectrum of manifestations ranging from systemic non-specific features such as fever, malaise, arthralgia and myalgia to specific organ damage. We describe two cases of cholesterol embolisation syndrome and Kaposi sarcoma mimicking primary systemic vasculitis, both of which were characterised by features such as livedo reticularis, blue toe syndrome, a brown purpuric skin rash and positive perinuclear anti-neutrophil cytoplasmic antibodies associated with Kaposi sarcoma. Establishing the right diagnosis was challenging and thus this report aimed to highlight the possible ways to distinguish them from primary systemic vasculitis.

[Blue toe syndrome as an initial manifestation of metastatic prostate cancer : A case report with literature review]. / Un syndrome des orteils bleus révélant un cancer de la prostate métastatique : une observation avec analyse de la littérature.

INTRODUCTION: Cutaneous and vascular manifestations of cancer are numerous. Among paraneoplastic acral vascular syndrome, we report a case of blue toe syndrome as the first manifestation of a prostate cancer following with analysis of this syndrome according literature. OBSERVATION: A 56-year-old man, with Raynaud's phenomenon of the upper limbs for 2 to 3 years, had 4 blue toes of the left foot evolving for 18 months, without ulceration, the pulses being present. Vascular and cardiac explorations (ultrasound, angio-MRI) were normal. There was no biological or immunological abnormality except an elevated PSA level. Prostate biopsies confirmed diagnosis and abdomino-pelvic CT scan proved the bone and lymph node metastasis. CONCLUSION: The revelation of a prostate cancer with bone metastases by a blue toe syndrome is a rare situation. In a patient with a blue toe syndrome with no obvious clinical or biological abnormality, especially atheromatous, investigations should include a search for cancer, which can be revealed by blue toes.

Rare Thrombotic Complications of COVID-19: A Case Series.

Coronavirus disease 2019 (COVID-19) is known to manifest with bilateral pneumonia and acute respiratory distress syndrome. This infection with severe acute respiratory syndrome coronavirus 2 (SAR-CoV-2) is alarming because it not only affects the respiratory system but may also cause thromboembolic events. Multiple studies have reported procoagulation/hypercoagulable complications in COVID-19. This case series is a valuable addition to the literature because it reflects unique presentations of thrombotic events in COVID-19 patients. We report two cases in which patients presented with thromboembolic complications secondary to COVID-19 infection: one with severe bowel ischemia and the other with blue toe syndrome. To formulate management strategies to prevent fatal outcomes for patients with COVID-19, physicians must be vigilant in identifying life-threatening thromboembolic complications from this disease.

Blue toe syndrome caused by emboli from anomalous left atrial septal pouch thrombus: a case report.

BACKGROUND: Left atrial septal pouches (LASPs) are a relatively newly described but common anatomical cardiac variant thought to be associated with atrial fibrillation (AF) and cardio-embolic stroke. Blue toe syndrome (BTS) describes ischemic changes in the toes due to microembolisation of the digital arteries. Establishing the etiology of BTS is vital so that the underlying cause can be treated. Here we describe the first case of BTS arising due to emboli from LASP thrombus arising on a background of new-onset AF. CASE PRESENTATION: A 65-year-old man presented with a two-day history of progressive painful swelling and bluish-purple discoloration of the second and fourth toes of his left foot and new-onset AF. Tests for hypercoagulability disorders were negative. Duplex ultrasound and CT angiography excluded deep venous thrombosis and an absence of embolus, thrombus, or occlusion in the arterial tree in the lower extremities bilaterally, so BTS was diagnosed. While transthoracic echocardiography and chest CT initially showed no cardiac abnormalities or mural thrombus, subsequent transesophageal echocardiography revealed a LASP with an associated pedunculated thrombus. The affected toes were amputated due to wet gangrene, but the patient recovered well with thrombus resolution after anticoagulation. CONCLUSION: The presence of a LASP in the absence of any other identifiable cause of BTS should trigger careful investigation of the interatrial septum, preferably using a multimodality imaging approach. The possibility that LASPs may not merely be an innocent bystander but a causative mechanism for peripheral ischemia must be considered.

Cholesterol Embolization Syndrome From Penetrating Aortic Ulcer.

Penetrating aortic ulcer (PAU) is an important, albeit, rarer cause of embolization to internal organs and distal extremities. Embolization occurs as a result of the disruption of cholesterol deposition in the wall of the aorta by a PAU. The classic presentation of cholesterol embolization syndrome (CES) includes pain, pallor, poikilothermia, paresthesia, and paralysis with intact pulses. The patient will classically have livedo reticularis or "blue toes." We present a case of a patient who presented to the emergency department with the complaint of a painful, blue toe. The patient had intact distal pulses on exam with the distal 2/3 of the first toe having a markedly blue/black color with livedo reticularis spreading proximally on the other 1/3 of the toe. CT angiogram with runoff to the lower extremities revealed a 3.6-cm infrarenal abdominal aortic aneurysm with a 5-mm penetrating aortic ulcer with a three-vessel runoff to the distal lower extremities. The diagnosis of CES secondary to a PAU was made. While thrombotic embolization from PAU causing acute limb ischemia is less common, it is well described. In contrast, cholesterol embolization from PAU remains a rare phenomenon without adequate treatment options.

Gastric remnant necrosis secondary to cholesterol crystal embolization after distal gastrectomy in a gastric cancer patient: a case report.

BACKGROUND: Distal gastrectomy with lymph node dissection, a standard operative technique for gastric cancer treatment, is safely performed because the stomach has a rich vascular supply. Gastric remnant necrosis caused by cholesterol crystal embolization following distal gastrectomy has not been described previously. We report a case of gastric remnant necrosis in a patient with cholesterol crystal embolization. CASE PRESENTATION: A 70-year-old man with a history of cholesterol crystal embolization presented to our surgery department with complaints of anorexia and dysphasia. He was diagnosed with gastric cancer invading the pyloric antrum and underwent distal gastrectomy with Billroth 2 reconstruction. On postoperative day 11, he developed abdominal pain without fever. Emergency laparotomy revealed that most parts of the remnant stomach were necrosed. Total gastrectomy with Roux-en-Y reconstruction and abscess drainage were performed. After surgery, anastomotic leakage occurred and was treated conservatively. However, the superior pancreaticoduodenal artery aneurysm suddenly ruptured and he expired. CONCLUSIONS: Gastric remnant necrosis after distal gastrectomy can be a gastrointestinal presentation of cholesterol crystal embolization. Perioperative/intraoperative risk assessments such as preventive total gastrectomy or intraoperative assessment with indocyanine green fluorescence angiography may be desirable to avoid this complication.

[Update on cholesterol crystal embolism]. / État des lieux de la maladie des emboles de cholestérol.

Cholesterol crystal embolism is a systemic pathology associated with diffuse atherosclerosis. Pathophysiology corresponds to tissue necro-inflammation secondary to arteriolar occlusion associated with microembolism from atherosclerotic plaques of large diameter arteries. The clinical presentation is heterogeneous and polymorphic. Multiple organs may be the targets, but preferential damage is skin, kidneys and digestive system. It is a serious pathology, underdiagnosed, with a poor prognosis. The risk factors for developing the disease remain the same risk factors as atheroma. The factors favouring migration of microembolism remain mainly vascular interventional procedures; easy to diagnose, they oppose spontaneous embolic migrations or secondary to the introduction of antithrombotic treatment, whose diagnosis is more difficult and the prognosis more severe. The diagnosis of the disease remains mostly a diagnosis of elimination and often refers to a bundle of clinical, biological, morphological and histologic arguments. The treatment is poorly codified and the subject of few publications. It will favour both symptomatic treatment (and mainly that of pain) and complications (high blood pressure, renal insufficiency). The aetiological support remains less consensual. The treatment of atherosclerotic plaques consists, of course, in the correction of classical cardiovascular risk factors, the introduction of a statin. It will be discussed in the implementation of surgery or angioplasty to exclude potentially responsible atherosclerotic lesions. Eviction of antithrombotic therapy should be considered in terms of the benefit-risk balance, but often in favour of maintaining it. Finally, other treatments may be proposed in a case-by-case basis, such as oral or intravenous corticosteroid therapy, colchicine or LDL aphaeresis.

[Blue toe syndrome as a clinical finding of pheochromocytoma]. / Síndrome del dedo azul como manifestación de feocromocitoma.

Pheochromocytomas are tumors that arise from chromaffin cells of the sympathetic nervous system and act by synthesizing and releasing catecholamines. They usually occur between the fourth and fifth decade of life and have a very wide clinical presentation. They occur only in 0.1-0.2% of the hypertensive population and represent a treatable and curable cause of arterial hypertension, as well as other symptoms derived from the uncontrolled secretion of catecholamines. Peripheral arterial ischemia secondary to massive amines release by a pheochromocytoma is a very uncommon condition. Here we report a case of pheochromocytoma manifested as blue finger syndrome in a patient with palpable distal pulses and history of poor blood pressure control despite treatment with two drugs.

Síndrome del dedo azul como manifestación de feocromocitoma / Blue toe syndrome as a clinical finding of pheochromocytoma

Los feocromocitomas son tumores que proceden de las células cromafines del sistema nervioso simpático y actúan sintetizando y liberando catecolaminas. Suelen presentarse entre la cuarta y quinta década de la vida y tienen presentaciones clínicas muy diversas. Ocurren solamente en 0.1-0.2% de la población hipertensa, constituyen una causa tratable y curable de hipertensión arterial, así como de otras manifestaciones derivadas de la liberación incontrolada de catecolaminas. La isquemia arterial periférica secundaria a la liberación masiva de aminas por un feocromocitoma es muy infrecuente. Aquí se presenta un caso clínico de feocromocitoma manifestado como síndrome del dedo azul en un paciente con pulsos distales conservados y el antecedente de mal control tensional a pesar de tratamiento con dos fármacos.

Pheochromocytomas are tumors that arise from chromaffin cells of the sympathetic nervous system and act by synthesizing and releasing catecholamines. They usually occur between the fourth and fifth decade of life and have a very wide clinical presentation. They occur only in 0.1-0.2% of the hypertensive population and represent a treatable and curable cause of arterial hypertension, as well as other symptoms derived from the uncontrolled secretion of catecholamines. Peripheral arterial ischemia secondary to massive amines release by a pheochromocytoma is a very uncommon condition. Here we report a case of pheochromocytoma manifested as blue finger syndrome in a patient with palpable distal pulses and history of poor blood pressure control despite treatment with two drugs.

[A POEMS syndrome revealed by a blue toe syndrome]. / Un syndrome POEMS révélé par un syndrome des orteils bleus.

INTRODUCTION: Arterial and venous thromboses occur in almost one in five patients with POEMS syndrome and usually in macrocirculation. CASE REPORT: We report a 67-year-old male with a POEMS syndrome who presented initially with a blue toe syndrome. He complained of Raynaud's syndrome and left foot paresthesia. Physical examination showed gynecomastia, lymphadenopathies and skin lesions. Cardiovascular investigations excluded atrial fibrillation, unstable atherosclerotic lesions and vascular calcifications. Imaging studies showed diffuse osteosclerotic lesions. Monoclonal protein with lambda light chain was discovered and serum level of VEGF was increased at 2900pg/ml. CONCLUSION: This is to our knowledge the first case of thrombotic microangiopathy in POEMS syndrome without embolic cause or calciphylaxis.

The usefulness of Duplex Doppler ultrasound in the angiological and dermatological diagnosis of patients with blue toe syndrome.

INTRODUCTION: Peripheral microembolism is one of the most frequent causes of acute limb ischemia. In order to effectively prevent relapses it is essential to localize and eliminate the source of embolism. AIM: To evaluate the role of Duplex Doppler ultrasound examination in identifying the causes of blue toe syndrome (BTS). MATERIAL AND METHODS: The group of 165 patients with clinical symptoms of BTS on their upper limbs (n = 16) and lower limbs (n = 149) was investigated. They all underwent Duplex Doppler ultrasound of the major arteries of the extremities, where ischemic changes occurred. RESULTS: Morphological and functional changes which might be potential sources of microembolism were identified in 146 patients. These changes included significant short-length stenoses or unstable atherosclerotic plaque (n = 73), true aneurysms (n = 42) and pseudoaneurysms (n = 17). In 11 cases, pathology of vascular prostheses in the form of anastomotic aneurysms, infection and residual thrombi after fibrinolysis was detected. In all cases, Duplex diagnosis was confirmed by other imaging and intraoperative tests. CONCLUSIONS: Duplex Doppler ultrasound of the arteries in the affected limb with a full length view should be the first-line examination in diagnosing patients with BTS. In the absence of hemodynamic blood flow disturbances in the major arteries in patients with symptoms of BTS, it is advisable to start haematological tests to identify/exclude congenital or acquired thrombophilia.

Blue Toe Syndrome due to Mobile Atheromatous Aorto-iliac Plaque Treated Successfully by Endovascular Aortic Repair

Blue toe syndrome is characterized by tissue ischemia secondary to cholesterol crystal or atherothrombotic embolization. It leads to the occlusion of small vessels. The treatment option is usually surgery for most causes of blue toe syndrome. However, endovascular aortic repair by aorto-iliac stent graft become more and more popular because of its effectiveness and its less invasive characteristic. We present a 57-year-old man who suffered from blue toes syndrome on both legs caused by embolizing aorto-iliac lesions. Successful Endurant stent graft (Medtronic Vascular, Santa Rosa, CA, USA) was performed on infrarenal abdominal aorta and on proximal portion of right and left common iliac artery.

Síndrome del dedo azul secundario a ateroembolia por colesterol: reporte de un caso y revisión de la literatura / Síndrome do dedo azul secundário a ateroembolia por colesterol: reporte de um caso e revisão da literatura / Blue toe syndrome secondary to cholesterol atheroembolia: Report of a case and literature review

Se describe el caso de una mujer de 68 años, con muy alto riesgo cardiovascular, quien consultó por cianosis en los dedos de los pies, asociada a síntomas neurológicos focales transitorios de 5 días de evolución. Se hospitalizó con la impresión diagnóstica de síndrome del dedo azul e isquemia crítica arterial de miembros inferiores de posible origen embólico. Luego de un procedimiento endovascular, presentó deterioro neurológico súbito y se documentaron múltiples infartos cerebrales y falla renal aguda. En la biopsia de los dedos afectados se observaron cristales de colesterol en el interior de los vasos sanguíneos. Con base en el caso se presenta una corta revisión del síndrome del dedo azul y su principal causa: la ateroembolia...

We describe the case of a 68 year-old woman with very high cardiovascular risk. She consulted because of cyanosis in the toes, associated with transient focal neurological symptoms. Evolutionhad been 5 days. She was hospitalized with the diagnostic impression of blue toe síndrome and critical arterial ischemia of the lower limbs possibly due to embolic events. After an endovascular procedure, she developed sudden neurological impairment due to multiple strokes, as well as acute renal failure. Biopsy of the affected toes revealed cholesterol crystals inside the blood vessels. Based on the case, a short review about the blue toe syndrome and its main cause, atheroembolism, is presented...

Se descreve o caso de uma mulher de 68 anos, com alto risco cardiovascular, quem consulto por cianose nos dedos dos pés, associada a síntomas neurológicos focais transitórios de 5 dias de evolução. Se hospitalizou com a impressão diagnóstica de síndrome do dedo azul e isquemia crítica arterial de membros inferiores de possível origem embólico. Logo de um procedimento endovascular, presentou deterioro neurológico súbito e se documentaram múltiplos infartos cerebrais e falha renal aguda. Na biopsia dos dedos afetados se observaram cristais de colesterol o interior dos vasos sanguíneos. Com base no caso se apresenta uma curta revisão da síndrome do dedo azul e sua principal causa: a ateroembolia...

A Controlled Study of Alprostadil Liposomal Preparation in the Treatment of Blue Toe Syndrome.

The aim of this study is to examine the efficacy of alprostadil liposomal preparation in the treatment of blue toe syndrome. As many as 32 patients with blue toe syndrome were randomized into the test group and a control group. Patients out of the test group were treated with alprostadil liposomal preparation, while those out of the control group received placebo administration. Inter-group comparisons were conducted for the post-therapeutic changes of microcirculation and improvements of clinical symptoms. In the test group, there were eight subjects with marked response (50.0 %), six subjects with partial response (37.5 %), and two subjects with no response (12.5 %), with the overall response rate of 87.5 %. In the control group, there were three cases (18.8 %), one case (6.4 %), and 12 cases (75 %), respectively, with the overall response rate of 25.0 %. The inter-group difference of response was statistically significant (Χ (2) = 12.987, P = 0.002 < 0.05). In the test group, there was one case of administration site redness which could be resolved spontaneously. No other adverse drug reactions were reported. No any complaints were reported for the control group. The inter-group difference of nail-fold microcirculation was not statistically significant (P > 0.05). The post-therapeutic points of nail-fold microcirculation in the test group decreased significantly (P < 0.05), but no significant changes were observed for the control group (P > 0.05). The post-therapeutic waveform changes of photoelectric plethysmography were significant for the test group in comparison to the control. The safety and efficacy of alprostadil liposomal preparation have been demonstrated in the treatment of blue toe syndrome.

Blue Toe Syndrome after Percutaneous Coronary Intervention / 대한피부과학회지

Blue toe syndrome involves blue or purplish toes in the absence of trauma, serious cold exposure, or disorders causing general cyanosis. Clinical presentation can range from a cyanotic toe to a diffuse, multi-organ systemic disease. A 75-year-old man presented with claudication, sudden bilateral painful discoloration of the sole, blue-colored toes, and anuria. Three weeks earlier, he had been diagnosed with acute myocardial infarction and had undergone catheterization for percutaneous coronary intervention. Histopathologic findings showed vascular ectasia with mild perivascular inflammation. Based on patient history, physical examination, and laboratory findings, he was diagnosed with blue toe syndrome. Our patient presented with clinical manifestations, including peripheral cutaneous involvement and acute deterioration of renal function. This case highlights the importance of prompt diagnosis of blue toe syndrome by careful history-taking and physical examination in order to avoid multi-organ systemic disease.

A case of antiphospholipid syndrome refractory to secondary anticoagulating prophylaxis after deep vein thrombosis-pulmonary embolism.

Antiphospholipid syndrome (APS) is an acquired systemic autoimmune disorder characterized by a combination of clinical criteria, including vascular thrombosis or pregnancy morbidity and elevated antiphospholipid antibody titers. It is one of the causes of deep vein thrombosis and pulmonary embolism that can be critical due to the mortality risk. Overall recurrence of thromboembolism is very low with adequate anticoagulation prophylaxis. The most effective treatment to prevent recurrent thrombosis is long-term anticoagulation. We report on a 17-year-old male with APS, who manifested blue toe syndrome, deep vein thrombosis, pulmonary thromboembolism, and cerebral infarction despite adequate long-term anticoagulation therapy.

A Case of Antiphospholipid Syndrome Refractory to Secondary Anticoagulating Prophylaxis after Deep Vein Thrombosis-Pulmonary Embolism / 결핵및호흡기질환

Antiphospholipid syndrome (APS) is an acquired systemic autoimmune disorder characterized by a combination of clinical criteria, including vascular thrombosis or pregnancy morbidity and elevated antiphospholipid antibody titers. It is one of the causes of deep vein thrombosis and pulmonary embolism that can be critical due to the mortality risk. Overall recurrence of thromboembolism is very low with adequate anticoagulation prophylaxis. The most effective treatment to prevent recurrent thrombosis is long-term anticoagulation. We report on a 17-year-old male with APS, who manifested blue toe syndrome, deep vein thrombosis, pulmonary thromboembolism, and cerebral infarction despite adequate long-term anticoagulation therapy.