Blue Toe Syndrome in Behçet's Disease: A Case Report.

BACKGROUND Vascular Behçet's disease (VBD) is a rare but potentially life-threatening subtype of Behçet's disease that is characterized by multisystemic vasculitis. It primarily affects males with ancestry traced back to regions along the ancient Silk Road. Both arteries and veins, regardless of size, may exhibit complications, including aneurysmal degeneration or occlusion. While venous involvement is observed in two-thirds of VBD cases, arterial complications are notably the most severe and lethal. Arterial aneurysmal degeneration is more common than occlusive complications, with larger arteries being predominantly affected in VBD. Data regarding isolated small-vessel arterial occlusive disease in VBD are limited. Given the rarity of this presentation in this patient population, it becomes mandatory to thoroughly evaluate such patients to differentiate small-vessel vasculitis from other similar diseases, such as Raynaud's phenomenon, which has a different etiology and management and generally has a more benign course. Here, we delineate the concept of isolated small-vessel vasculitis as a cause of blue toe syndrome in patients with VBD. CASE REPORT This report describes a distinctive case of vascular Behçet's disease in a 51-year-old man who initially exhibited unilateral blue toe syndrome, which swiftly progressed to dry gangrene of the toes. Despite reports of large-vessel involvement, there is a paucity of data on isolated small-vessel vasculitis-induced digital ischemia in VBD. CONCLUSIONS This atypical case underscores the necessity of clinical discernment in differentiating inflammatory microvascular occlusive disease from vasospastic Raynaud's syndrome, both of which can complicate Behçet's disease.

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.

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.

The Solitary Blue Toe: A Unique Presentation of Antiphospholipid Syndrome.

Antiphospholipid syndrome is an autoimmune disease characterized by vascular thrombosis involving both the arterial and venous systems that can lead to tissue ischemia or end-organ damage. Much of the literature describes various symptoms at initial presentation, but isolated tissue ischemia manifesting as a solitary blue toe is unusual. We discuss a case of a 23-year-old man who presented to the emergency department with a solitary blue fourth digit with minimal erythema and edema, who was suffering from exquisite pain. Following an extensive workup, the patient was diagnosed with antiphospholipid syndrome with thrombi of the vasculature in their lower extremity. With therapeutic anticoagulation, the patient's symptoms subsided and amputation of the digit was prevented.

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.

[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.

[Benign Presentation of a Potentially Fatal Disease]. / Ateroembolismo cutâneo: relato de três casos clínicos.

Atheroembolism is a rare multisystemic disorder that is characterized by release of cholesterol crystals and particles from atheromatous plaques, which can occlude distal vessels and induce an inflammatory response. Most affected individuals are males, older than 60 years of age, with advanced atherosclerotic disease. The abdominal aorta is the most common origin of cholesterol emboli, being the peripheral arteries a rarer source. Cholesterol embolization syndrome is often associated with invasive vascular procedures, although, more rarely, it may occur spontaneously. In this paper, the authors present three cases of spontaneous atheroembolism with cutaneous manifestations and their clinical management. Being an underdiagnosed pathology, knowledge about its clinical manifestations is essential in order to allow an early diagnosis and treatment, to ensure a better prognosis for the patient.

O ateroembolismo é uma doença multissistémica rara caraterizada pela libertação de cristais de colesterol e partículas de placas ateroscleróticas, que podem ocluir vasos sanguíneos periféricos e induzir uma resposta inflamatória. A maioria dos indivíduos afetados é do sexo masculino, com idade superior a 60 anos e doença aterosclerótica avançada. A origem mais frequente de embolização de colesterol é a aorta abdominal, sendo as artérias periféricas uma fonte mais rara. A síndrome de embolização por colesterol surge frequentemente associada a procedimentos vasculares invasivos, embora, mais raramente, possa ocorrer de forma espontânea. Neste artigo os autores apresentam três casos clínicos de ateroembolismo espontâneo com envolvimento cutâneo e respetiva abordagem clínica. Sendo uma patologia subdiagnosticada, torna-se fundamental o conhecimento acerca das suas manifestações clínicas, para permitir um diagnóstico e tratamento precoces de forma a garantir um melhor prognóstico para o doente.

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 as a first sign of systemic sclerosis.

We describe an unusual case of blue toe syndrome as the primary and solitary manifestation of systemic sclerosis. The possible cause was long-term occupational exposure in construction work. Blue toe syndrome is a small vessel disease, characterised by the sudden development of painful, blue discolouration in one or more toes. The most common aetiology is atheroembolic disease; however, it can also appear in several conditions ranging from hypercoagulability disorders to underlying systemic diseases such as vasculitis or autoimmune diseases. Here, we describe the case of a 57-year-old man who presented with blue toe syndrome without underlying atheroembolic disease. He was found to have positive anticentromere antibodies, which indicated that systemic sclerosis was the likely primary underlying cause. An extensive systemic evaluation and a thorough physical examination revealed no other symptoms associated with systemic sclerosis. He was prescribed nifedipin and rosuvastatin, and showed complete resolution of symptoms after 3 months.

[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.