Durvalumab-associated vasculitis presenting as 'the blue toe syndrome'.

Durvalumab is a selective, high-affinity human immunoglobulin monoclonal antibody in a class called check point inhibitors, that blocks PD-L1 on tumour cells. Despite clinical success in increasing progression-free survival rates in patients with stage III non-small-cell lung cancer, durvalumab has been associated with immune-related side effects such as pneumonitis and colitis. We present a case of an 84-year-old woman with acral vasculitis presenting as blue toe syndrome, associated with prolonged use of durvalumab. After 1 year of fortnightly durvalumab therapy postchemoradiation therapy, the patient came in with a left blue big toe, and later developed bilateral livedo racemosa. The diagnosis of durvalumab-associated vasculitis was made and treatment with prednisolone was started with clinical improvement.

[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.

Blue toe syndrome and sunitinib.

We present a patient with metastatic renal cell carcinoma treated with sunitinib, a multitargeted tyrosine kinase inhibitor. The patient experienced bilateral blue toe syndrome which we related to sunitinib use. Discontinuation of sunitinib to lower the patient's prothrombotic state and increase the ability to form collaterals, together with the addition of low-molecular-weight heparin to treat the occluding thrombi, resulted in waning of the blue toe syndrome. This case adds to the accumulating evidence of possible untoward cardiovascular side effects that should be taken into consideration in patients on tyrosine kinase inhibitors such as sunitinib.

[The blue toe syndrome and its pathogenic significance. A case report]. / A síndrome do dedo azul e o seu significado patogénico. A propósito de um caso clínico.

A 64-years-old woman complained of fixed cyanosis and rest pain of the 2nd, 3rd and 4th toes of the right foot, after a sudden onset one month previously to the clinical examination. The diagnosis of "blue toe syndrome" was then made. She was in a post-menopause state, with no hormonal substitution therapy, complaining also of obesity, arterial hypertension and hyperlipidemia, under medication but no laboratory control. Blood tests excluded an hypercoagulable state and the ECG revealed no significant abnormalities. Angio-CT scans and conventional angiography disclosed an atherosclerotic lesion at the femoropopliteal level, with an adherent and floating thrombus in the arterial lumen, causing microemboli to the collateral digital arteries. The complex lesion was removed through a local thromboendarterectomy, followed by a Carrel-DeBakey patch graft angioplasty, using autologous saphenous vein. Post operative course was uneventfull, with an immediate recovery of the clinical picture. Double antiplatelet therapy was advised and an extensive investigation of the possible relationship of this event with an occult malignancy was started, with no conclusive results, until now. The patient was placed in a clinical, laboratory and imagiologic surveillance program and the main features of this entity are emphasized and discussed, according to the data published in the literature on the subject.

[Blue toe syndrome; a sign of end-arterial occlusion]. / Blauwe-tenensyndroom; een teken van eindarteriële occlusie.

Three patients, two women aged 66 and 43 years, respectively, and a man aged 76 years, presented with sudden, painful, blue areas in the toes with intact peripheral pulsations. One patient had a myeloproliferative syndrome due to essential thrombocytosis, the second patient had thromboangiitis obliterans, and the third patient had a cholesterol embolism, possibly due to the use of oral anticoagulants. After treatment, one patient recovered fully and the other two improved significantly. The blue toe syndrome is the pathophysiological consequence of end-arterial occlusion and frequently the first manifestation of a systemic disorder, such as atheroembolism or vasculitis. Adequate treatment is possible in most cases. Therefore, it is very important to recognise this disorder and its possible causes so as to prevent further episodes of local symptoms and especially systemic complications.

A case of cholesterol embolism confirmed by skin biopsy and successfully treated with statins and steroids.

Although cholesterol embolism syndrome was recognized as a clinicopathologic entity more than 50 years ago, it is attracting growing attention recently. It is a multisystemic disorder in which cholesterol crystals released from atherosclerotic plaques obstruct small arterioles, resulting in local ischemia and end-organ damage. There are no established treatments, and with the limited treatment options available, it is important to make the diagnosis as early as possible. We present the case of a 68-year-old man with cholesterol embolism who had a few fluttering atheromas in the aorta, as demonstrated by transesophageal ultrasonography. The diagnosis was confirmed by skin biopsy, and treatment with statins and steroids proved effective, as renal failure progressively improved. This case emphasizes the importance of early diagnosis and shows the possible therapeutic effects of statins and steroids for patients with this syndrome.

Spontaneous dissection of the popliteal artery in a young man. A rare cause of the blue toe syndrome.

Spontaneous arterial dissection in peripheral arteries of the extremities is an extremely rare event. We report a case of a spontaneous dissection of a nonaneurysmal popliteal artery in an otherwise healthy 36-year-old man that came to clinical attention as an acute blue toe syndrome. The diagnosis was primarily made by high-resolution duplex ultrasound that revealed a dissection flap (length: 15.5 mm; thickness: 0.4 mm) together with the partially thrombosed false lumen at the dorsal wall of the left popliteal artery (degree of local diameter reduction: 56%). Further work-up by means of contrast-enhanced MR-A and conventional DSA confirmed a moderate stenosis of the popliteal artery compatible with focal dissection and excluded other causes such as popliteal artery entrapment syndrome. Under full-dose intravenous anticoagulation with unfractionated heparin that was switched to oral anticoagulation with vitamin K antagonists (target INR: 2-3) and conservative management of the blue toe the patient made a gradual, but eventually complete clinical recovery over 8 weeks.

Cholesterol crystal embolization mimicking vasculitis: success with corticosteroid and cyclophosphamide therapy in two cases.

Cholesterol crystal embolization is a potential complication of atherosclerosis. Approximately one-third of the patients who develop this problem have a history of vascular surgery, angiography or angioplasty hours to weeks before onset. The skin and the kidneys are most frequently involved, but any organ can be affected. Livedo reticularis of the lower extremities and acrocyanosis (known as "blue toe syndrome") are the most common cutaneous manifestations. Histological examination is the only way to definitively diagnose cholesterol crystal embolization. Recently, it has been proposed that cholesterol embolization is associated with vasculitis, and some authors have labeled this condition a "vasculitis look-alike." There is still no specific treatment for this problem, even in cases that progress to renal failure. However, a few case reports in the literature have noted successful treatment with corticosteroids and cyclophosphamide in patients with deteriorating renal function. In this article, we describe two cases of severe cholesterol crystal embolization accompanied by renal dysfunction) and blue toe syndrome. Both patients benefited from corticosteroid and cyclophosphamide therapy.

[Cholesterol crystal embolization exacerbated after the off-pump coronary artery bypass; report of a case].

A 66-year-old man with left main trunk disease was treated under a diagnosis of acute myocardial infarction based on coronary angiography by off-pump coronary artery bypass (OPCAB). About 1 month after operation, his renal function deteriorated and purpura appeared on both feet, especially at the toe tips. In this case, steroid therapy was performed and the patient survived. Cholesterol embolism rarely occurs after angiographic procedure or cardiovascular surgery. In general, it is associated with high morbidity and mortality, but no optimal treatment has yet been developed. This underlines the importance of careful observation and skin biopsy for early diagnosis.

Blue toe syndrome: a rare complication of acute pancreatitis.

CONTEXT: Blue toe syndrome is an unusual complication of acute pancreatitis. It is characterized by tissue ischemia secondary to cholesterol crystal or atherothrombotic embolization leading to the occlusion of small vessels. Clinical presentation can range from a cyanotic toe to a diffuse multiorgan systemic disease that can mimic other systemic illnesses. CASE REPORT: Here we describe a young male who developed this complication after acute alcoholic pancreatitis.

Early experience using the Wallgraft in the management of distal microembolism from common iliac artery patholology.

Blue toe syndrome commonly occurs as a result of aneurysmal or atherosclerotic disease in the iliac arteries. Surgery, angioplasty, or intraarterial stent placement are the most common treatment options but the optimal management has not been defined. Here we report managing distal microembolization from iliac artery atherosclerosis associated with aneurysmal dilation with the Wallgraft Endoprosthesis, a self-expanding metallic stent covered with Dacron. Three common iliac arteries in two patients were treated using this device. A 79-year-old male presented with unilateral symptoms and an 83-year-old female with bilateral disease. Arteriography demonstrated complex plaque at the aortic bifurcation associated with aneurysmal dilation of the distal common iliac artery in both patients. This complex disease was successfully covered using the Wallgraft Endoprosthesis. Postoperatively the patients received aspirin, their toe lesions healed, and neither has had a recurrence after 16 months. Covered stents offer the theoretic advantage of completely excluding the diseased segment, preventing the escape of thrombus or plaque debris, and covering aneurysmal dilation in the artery.