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Thrombophlebitis Migrans

Thrombophlebitis Migrating

Thrombophlebitis migrans is a prothrombotic state of the peripheral circulation most likely due to an underlying malignant disease. Symptoms appear months or years prior to the onset of cancer-related symptoms, most commonly in the form of recurrent tender nodules that develop on the trunk or the extremities. A detailed workup is necessary for these patients, while non-steroidal anti-inflammatory drugs, compression, and anticoagulation are used in therapy.


Presentation

Tender, erythematous nodules that resolve and recur spontaneously on the chest or extremities are a diagnostic hallmark of thrombophlebitis migrans [1], and their appearance may precede signs and symptoms of underlying malignancy by several months, or even years [3] [5].

Workup

If a recurrent development of erythematous nodules is reported, a strong suspicion toward a hypercoagulable state should be made and a thorough diagnostic workup must be conducted, especially if no other signs and symptoms are present [5]. The primary reason is that signs and symptoms of thrombophlebitis migrans often precede the onset of cancer-related complaints [5]. Laboratory studies should include a complete blood count, full coagulation panel, and basic biochemistry. Imaging studies of the thorax and abdomen are also necessary.

Treatment

Treatment of thrombophlebitis migrans comprises use of NSAIDs together with an elevation of the extremity, application of hot and wet compresses and use of compression garments [1] [6] [7]. Anticoagulant therapy is introduced in the presence of threatening deep vein thrombosis (DVT). Low-molecular-weight heparin (LMWH) is the drug of choice in that setting. Some authors, in fact, recommend prophylaxis with heparin in patients with recurrent or chronic superficial venous thrombosis [7]. In severe cases, phlebectomy may be indicated.

Prognosis

The condition itself can be managed successfully, but its presence must facilitate a thorough diagnostic workup in order to find out which condition triggered its occurrence.

Etiology

In approximately 50% of cases, malignant diseases are the underlying cause [1]. Lung and pancreatic neoplasms are considered to be the most common associated types, but cancers of the breast, ovaries, colon, stomach, and kidneys have all been described as potential causes [1] [3]. On the other hand, infections such as syphilis and vasculitic disorders - Thromboangiitis obliterans (Buerger's disease) and Behcet's disease are most important non-malignant causes [4] [8].

Epidemiology

Thromboembolic disease is encountered in approximately 1-3 per 1,000 individuals in the general population, but four-to-seven fold higher rates are observed in patients suffering from a malignant disease [3]. Unfortunately, the exact data regarding the appearance of thrombophlebitis migrans remains to be revealed [3]. In thromboangiitis obliterans (Buerger's disease), however, studies have shown that about 65% of patients show typical signs of thrombophlebitis migrans during the disease [8]. In fact, it was established to be the first sign of Buerger's disease in 25% of patients [8]. So far, predisposing factors include extensive bed rest, the presence of infections, surgical procedures, and chemotherapy, particularly known for its propensity to cause severe prothrombotic events [1].

Sex distribution
Age distribution

Pathophysiology

The exact mechanism of the disease remains to be discovered, but it is known that cancer cells are able to promote coagulation by inducing expression of tissue factor (TF) and production of pro-inflammatory cytokines. TF is able to convert factor VII to factor VIIa, which initiates the coagulation cascade via the extrinsic pathway. Additionally, direct activation of platelets was proposed as well [1].

Prevention

The exact mechanisms that lead to its onset in cancer patients are unclear, making prevention impossible at this moment.

Summary

Thrombophlebitis migrans was initially described more than 150 years ago as a cancer-related thrombosis of peripheral veins (also known as Trousseau's syndrome) [1], characterized by the recurrent appearance of skin lesions in otherwise healthy veins of the extremities and torso [2]. In more than 50% of cases, a malignant disease is an underlying cause, with lung cancer being the most common type, followed by pancreatic, colorectal, renal and prostate malignancy [1], but virtually any cancer can induce this hypercoagulable state [3]. Infections and vasculitis disorders (thromboangiitis obliterans and Behcet's disease) are recognized non-malignant conditions [4]. The pathogenesis of this disorder stems from continuous stimulation of coagulation by malignant cells through incompletely understood mechanisms and the clinical presentation involves the recurrent appearance and spontaneous resolution of tender, erythematous nodules on the extremities and chest [1] [2]. It is not uncommon for this condition to precede the onset of cancer-related symptoms for months or even years [3] [5], which is why a thorough diagnostic workup should be conducted in order to identify the underlying cause. Treatment principles rely on administration of non-steroidal anti-inflammatory drugs (NSAIDs), use of compression garments, and anticoagulation therapy in the setting of threatening deep venous thrombosis [1] [6] [7].

Patient Information

Thrombophlebitis migrans is a disorder of abnormal blood coagulation and thrombosis of superficial veins, most commonly as a result of a malignant disease. Presumably, cancer cells are able to promote abnormal coagulation by stimulating platelet production and various other molecules involved in the process of clot formation, but the exact mechanism remains unknown. In addition to cancer (most common being lung and pancreatic, but virtually any type of malignancy can induce this disorder), infections such as syphilis and blood vessel disorders including Behcet's disease and thromboangiitis obliterans (also known as Buerger's disease) may cause thrombophlebitis migrans. The most important manifestation is the recurrent spontaneous appearance of red and tender nodules on the skin of the chest, arms, and legs. The diagnosis should be relatively easy to make if these lesions are seen. However, cancer-related symptoms may appear months or even years after the onset of thrombophlebitis migrans, which is why a meticulous diagnostic workup is necessary to determine the cause early on. Treatment includes administration of nonsteroidal anti-inflammatory drugs (NSAIDs), application of hot, wet compresses, and use of compression garments. Anticoagulant therapy is indicated if there are signs of threatening deep venous thrombosis (DVT). Surgery in necessary only in rare cases.

References

Article

  1. Thayalasekaran S, Liddicoat H, Wood E. Thrombophlebitis migrans in a man with pancreatic adenocarcinoma: a case report. Cases J. 2009;2:6610.
  2. Porter RS, Kaplan JL. Merck Manual of Diagnosis and Therapy. 19th Edition. Merck Sharp & Dohme Corp. Whitehouse Station, N.J; 2011.
  3. Kadlec B, Skrickova J, Merta Z, Dusek L, Jarkovsky J. The Incidence and Predictors of Thromboembolic Events in Patients with Lung Cancer. The ScientificWorldJournal. 2014;2014:125706.
  4. Fiehn C, Pezzutto A, Hunstein W. Superficial migratory thrombophlebitis in a patient with reversible protein C deficiency and anticardiolipin antibodies. Ann Rheum Dis. 1994;53(12):843-844.
  5. Litzendorf ME, Satiani B. Superficial venous thrombosis: disease progression and evolving treatment approaches. Vasc Health Risk Manag. 2011;7:569-575.
  6. Nasr H, Scriven JM. Superficial thrombophlebitis (superficial venous thrombosis). BMJ. 2015;350:h2039.
  7. Litzendorf ME, Satiani B. Superficial venous thrombosis: disease progression and evolving treatment approaches. Vasc Health Risk Manag. 2011;7:569-575.
  8. Fazeli B, Modaghegh H, Ravrai H, Kazemzadeh G. Thrombophlebitis migrans as a footprint of Buerger's disease: a prospective-descriptive study in north-east of Iran. Clin Rheumatol. 2008;27(1):55-57.

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Last updated: 2018-06-21 22:41