Reporte de casos de síndrome de dedo azul / Blue finger syndrome: Case reports

RESUMEN El síndrome de dedo azul (SDA) se caracteriza por la coloración violácea o azul de uno o más dedos, puede serla primera manifestación de múltiples enfermedades, tanto las que presentan alteraciones directamente en los dedos o ser la expresión de enfermedades sistémicas; los mecanismos fisiopatológicos más comunes son trombosis, embolia, vasoconstricción grave o afección del lecho vascular que puede ser inflamatoria o no inflamatoria. Describimos 5 casos de SDA, donde resaltamos la importancia del diagnóstico temprano y enfatizamos en el concepto de evaluación y abordaje como una urgencia médica, sin importar la causa, ya que su manejo y tratamiento inicial, más el intento de lograr un tratamiento dirigido a una etiología podría disminuir complicaciones irreversibles como la necrosis o amputación.

ABSTRACT Blue finger syndrome (BFS), usually noted by the violet or blue coloration of one or more fingers, may be the first manifestation of several diseases. These may present with alterations directly on the fingers or be the expression of systemic diseases. The most common pathophysiological causes are thrombosis, embolism, severe vasoconstriction, or vasculature involvement that may be inflammatory or non-inflammatory. A description is presented of 5 cases of BFS, where the emphasis is placed on the importance of early diagnosis. The concept of evaluation and approach as a medical emergency is also stressed, because depending on this, it could reduce irreversible complications, such as necrosis and/or amputation.

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 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...

Blue Toe Syndrome as an Early Sign of Disseminated Intravascular Coagulation

No abstract available.

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.

Skin lesions simulating blue toe syndrome caused by prolonged contact with a millipede

Venomous animals are those that, by means of a hunting and defense mechanism, are able to inject their prey with a toxic substance produced in their bodies, directly from specialized glands (e.g., tooth, sting, spur) through which the poison passes. Millipedes are poisonous animals; they can be harmful to humans, and their effects usually manifest as erythematous, purpuric, and cyanotic lesions; local pain; and paresthesia. Here, we report a case of skin contact with a millipede for 6h resulting in skin lesions similar to blue toe syndrome.

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.

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.

Blue toe syndrome treated with sympathectomy in a patient with acute renal failure caused by cholesterol embolization

Blue toe syndrome is the most frequent manifestation of tissue ischemia caused by cholesterol embolization (CE), which can lead to amputation of affected lower extremities, if severe. However, any effective treatment is lacking. We experienced a case of spontaneously presenting blue toe syndrome and concomitant acute renal failure in a patient with multiple atherosclerotic risk factors. CE was confirmed by renal biopsy. Despite medical treatment including prostaglandin therapy and narcotics, the toe lesion progressed to gangrene with worsening ischemic pain. Therefore, we performed lumbar sympathectomy, which provided dramatic pain relief as well as an adequate blood flow to the ischemic lower extremities, resulting in healing of the gangrenous lesion and avoiding toe amputation. This is the first reported case of a patient with intractable ischemic toe syndrome caused by CE that was treated successfully by sympathectomy. Our observations suggest that sympathectomy may be beneficial in some patients with CE-associated blue toe syndrome.

Persistent sciatic artery presenting as blue toe: diagnostic dilemma

A persistent sciatic artery [PSA] results from lack of regression of the fetal arterial blood supply of the leg and is often combined with an abnormally developed superficial femoral artery. Here in we would like to present a 63 year old woman presenting for the first time with blue toe and on further evaluation diagnosis of PSA was made. Our case illustrates that a complete physical examination and comprehensive evaluation of the peripheral arterial system of the lower limb including possible persistence of sciatic artery is essential in patients presenting as blue toe before embarking on a surgical or other type of interventional procedure

Blue Toe Syndrome: A Case Report

PURPOSE: Blue toe syndrome consists of blue or purplish toes in the absence of a history of obvious trauma, serious cold exposure, or disorders producing generalized cyanosis. It is a life-threatening and still underrecognized disease. It can be commonly occurred by vascular surgery, invasive cutaneous procedures or anticoagulant therapy. Our case is presented of blue toe syndrome related to atheromatous embolization that was presumably triggered by angio CT. METHODS: A 69-year-old man presented with the suddenly developed pain, cyanosis and livedo reticularis of the toes in right foot. Dorsalis pedis pulses were palpable. He had been performed a diagnostic angio CT 1 month earlier. Angio CT revealed diffuse aortic atheromatous plaque in lower abdominal aorta and both common iliac artery. One month after angio CT, he visited our clinic. There was no visible distal first dorsal metatarsal artery and digital artery of right first toe in lower extremity arteriography. A diagnosis was established of blue toe syndrome. Because his symptom was aggravated, we performed the exploration of the right foot. After exposure of first dorsal metatarsal artery, microsurgical atheroembolectomy was done. RESULTS: There were no postoperative complications. After three months the patient had no clinically demonstrable problems. CONCLUSION: Patient with blue toe syndrome is at high risk of limb loss and mortality despite treatment. Blue toe syndrome produces painful, cyanosed toes with preserved pedal pulses. It needs to be aware of blue toe syndrome. Careful history should reveal the diagnosis. Treatment is controversial, however, most believe that anticoagulation therapy should be avoided.

Embolización cutánea por microcristales de colesterol: presentación de un caso y revisión de la literatura / Cholesterol cutaneous crystal embolization: a case report and literature review

La embolización de cristales de colesterol genera un síndrome multiorgánico inespecífico, severo, relativamente infrecuente y de difícil diagnóstico. Se produce por la oclusión de pequeños vasos en diferentes sistemas, entre ellos la piel, órgano diana frecuente. Se comunica el caso de un varón de 69 años con múltiples factores de riesgo cardiovascular y varios eventos desencadenantes que presentó embolización por microcristales de colesterol con compromiso cutáneo y renal.

Cholesterol crystal embolization is a rare and severe multiorganic syn-drome of diffi cult diagnosis. It occurs as a result of the occlusion of smallvessels in diff erent organs, being the skin a frequent diana.We present the case of a 69 years-old male with multiple cardiovascularrisk factors and many precipitant events. He developed cholesterol em-bolization syndrome with cutaneous and renal involvement.

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.

Locked-in syndrome and blue toe syndrome caused by cardiopulmonary bypass

Severe inflammation after cardiopulmonary bypass with the vasculitis of the acral extremity and vertebro-basilar arterial system leads to the locked-in syndrome and blue toe syndrome. In broad terms, systemic, idiopathic, and environmental factors provoke syndromes that present with digital discoloration or the blue toe syndrome. Painful digital discoloration, accompanied by ulceration, suggests vasculitis, involving small blood vessels. Definitive diagnosis usually requires histological documentation because vasculitic syndromes have no pathognomonic clinical features or laboratory test results. The case introduced herein is that of a woman who developed the locked-in syndrome in conjunction with quadriplegia, loss of facial movement, speech loss, and loss of horizontal eye movements. She had initially presented with severe mitral stenosis and left atrial clot and undergone mitral valve replacement and clot extraction. The patient expired from multiple organ failure despite prolonged ventilatory support, including tracheotomy, and meticulous nursing care and antibiotic prophylaxis. Given the previously reported partial recovery from this syndrome with the use of steroids, we would advocate the use of such pharmacological agents

Cumulative Patency Rate of Percutaneous Transluminal Angioplasty and Stent Placement for Aortoiliac Occlusive Disease

PURPOSE: To evaluate the results of iliac artery angioplasty and stent placement as an option for the treatment of aortoiliac occlusive disease. METHOD: The records of 30 patients (mean age, 65.5 years) who underwent iliac artery angioplasty and/or stent placement were reviewed retrospectively. Presenting symptoms included asymptomatic (6.7%), claudication (73.3%), rest pain (10%), ulceration/tissue loss (3.3%), and blue toe syndrome (6.7%). Follow-up included angioplasty, Doppler ultrasound, and clinical examination. Mean follow-up time was 32 months. RESULT: Forty iliac lesions were treated. Thirty-seven percent of patients had hypertension, 33% had diabetes mellitus, 23% had coronary arterial disease, 6.6% had cerebrovascular disease, 3.3% had hyperlipidemia and 3.3% had renal insufficiency. TASC (Trans Atlantic Inter-Society Consensus) A, B, C and D disease types were 11 (36.7%) patients, 5 (16.7%), 10 (33.3%) and 4 (13.3%). Ipsilateral superficial femoral artery occlusion was present in 6 (20%) patients. Concomitant femoral artery bypass surgery was performed in 10 (33.3%) patients. The cumulative primary patency rates were 83.4%, 71.9% and 64.7% at 1, 2, and 3 years, respectively. CONCLUSION: Iliac artery angioplasty and stent placement is a technically safe and effective treatment modality in patients without ipsilateral superficial femoral artery occlusion.

Management of Acute Peripheral Arterial Occlusion

PURPOSE: Treatment modalities of acute limb ischemia have evolved over the last decades, but the morbidity and mortality of the disease still remains high. We performed a retrospective study to analyze the demographics, risk factors, and prognosis of this disease. METHOD: Our subjects included a total of 49 patients (55 limbs) with acute peripheral arterial occlusion who underwent operative procedures between September 1996 and August 2001 at Gil Medical Center. Cases with graft occlusion or blue toe syndrome were excluded. The SVS/ISCVS reporting standards was used. RESULT: Mean age was 64.2 years (range: 25~85) and male-to-female ratio was 1.7 : 1. Clinical categories of ischemia were classified as the following: Viable (I) in 10 cases, Marginally threatened (IIa) in 14, Immediately threatened (IIb) in 17, and Irreversible (III) in 8. There were 40 lower extremity and 8 upper extremity arterial occlusions, and 1 aortic occlusion. The causes of occlusion were thrombosis in 14 and embolism in 35. Thromboembolectomy was performed in 42 cases, bypass graft in 9, primary amputation in 7, thrombolysis in 1, and stent insertion in 1. The 30-day mortality rate was 8%, mainly due to reperfusion injury and underlying cardiopathy. The 30-day major amputation rate was 23.8%. CONCLUSION: An aggressive, prompt operative management is important in saving patients with acute arterial occlusion. Higher amputation rates were related to more severe categories of ischemia at initial presentation. Patient education along with early referral and intervention will possibly reduce the amputation rate.

Blue Digit Syndrome: Treatment with Endarterectomy and Intra-Arterial Stent Placement: 2 Cases Report

Blue digit syndrome, peripheral atheroembolism, and atheromatous embolization, all refer to microembolization and occlusion of the smaller distal arteries. Despite the longstanding recognition that atheroemboli arise from severely degenerative atherosclerotic plaques in the proximal circulation, many questions remain about the pathophysiology and natural history of this disorder. The threat to the survival of a single digit may not appear to be of great consequence, but repeated episodes of atheroembolism with continued destruction of the collateral circulation may portend disaster for the digit. Diagnostic efforts should be promptly concentrated on the location, stabilization and preferably, eradication of the embolic source. We report 2 cases of blue digit syndrome were managed by endarterectomy and intra-arterial stenting. Case 1: A 61-year-old man was presented with the blue toe syndrome at the third, fourth, fifth toes. The bilateral pedal pulses were normally palpable and ankle-brachial pressure indices (ABI) were within normal range. At the findings of duplex ultrasonography and CT angiography, right common femoral artery showed a focal eccentric stenosis with mural thrombus. The right common femoral artery endarterectomy was performed for the athersclerotic ulcerating plaque. Case 2: A 64-year-old man was presented with 11-month history of his left leg pain and 1-week history of his left third, fourth fingers. He had a history of flap operation for his left fourth finger tip due to necrosis. At the findings of angiography, multiple stenosis of left common iliac and left subclavian arteries were found. The lesion of left subclavian artery lesion was presumed to be the source of blue finger syndrome and treated with intra-arterial stent placement after balloon angioplasty.

Clinical Experiences of Stented Graft

PURPOSE: Endovascular repair is a alternative treatment with favorable short-term result, but long term results are not available compared to standard operation. The authors have reviewed experiences of Asan Medical Center on stented graft, performed from Aug. 1998 to Feb. 2000, and the results as to age, operative indication, clinical condition, anatomic location, complication, follow-up are reported. METHOD: Seven patients who has received stented graft between Aug. 1998 and Feb. 2000 are reviewed retrospectively. RESULT: Of the seven patients, 6 were male and 1 was female. The mean age was 46.4 years (17~71). Indication for stented graft were two pseudoaneurysms, two iatrogenic arterio-venous fistulas, one thoracic aorta aneurysm, one abdominal aortic aneurysm, and one iliac artery occlusion with blue toe syndrome. Three different types of stented graft devices were utilized: Dacron-covered nitinol stented graft (made by the Asan Medical Center medical device team), Vanguard II (by Boston Scientific Corp.), and PTFE with nitinol stented graft (by Tae-woong). There was no perioperative mortality, and the technical success was achieved in five cases (71%). The patency at 14-month were 100% (5/5). Two immediate complications are hemorrhage due to iliac & femoral artery avulsion requiring emergency bypass grafting, and deployment failure of stented graft requiring removal of retained graft device. CONCLLUSION: Stented graft may be utilized even in complicated vascular situation but will need further development of device and more experience to prevent possible catastrophic complication. Indication should be decided by team approach and vascular surgeon should be prevent or participate during the procedure.