Mondor’s Disease

About Mondor’s Disease

What is it, history henri mondor

Back in 1931, a French physician called Henri Mondor documented a detailed case of  Thrombophlebitis, however previous random incidents of the disease have been dated all the way back to the 1850s. Mondor’s disease, also known as Thrombophlebitis, is generally a benign condition that resolves on its own within up to 8 weeks. It is characterised by an unexpected manifestation of tangible subcutaneous fibrotic induration under the skin dermal layer. Such increases in the fibrous elements have been associated to appear on the abdomen, penis, axilla and the mammalian cervical region. Usually, most cases of Mondor’s disease manifest as thrombophlebitis of the superficial vein, however in some cases they can also show as lymphangitis. The affected region does not necessarily show inflammatory dermal signs or difficulty in mobility, actually most of the time it is asymptomatic. However, due to the disease being extremely rare the precise aetiology, pathophysiology and epidemiology are not fully understood, because of the limited research and knowledge amongst scientists. The original Mondor’s disease is generally recognised as induration in the anterolateral thoracoabdominal wall, and the condition in other regions is considered a variant of the disease. Penile Mondor’s disease was initially in 1958, while the axillary web syndrome (AWS) was first seen in the early 2000s.¹ The estimated annual occurrence of venous thromboembolism in modern Western society is 1 in 1000 individuals. The annual incidence of symptomatic venous thromboembolism is decreased compared with asymptomatic venous thromboembolism, at approximately 0.5 to 1.6 per 1000 individuals.

What does it affect?

It has been difficult to differentiate tiny blood vessels that have been congested from obstructed lymph vessels via histopathological methods, hence still to this day the pathogenesis of Mondor’s Disease has been unclear. The associated complications from thrombophlebitis are very rare, however, if deep vein thrombosis (DVT) is developed then any further impediments can be extremely dangerous. The condition known as the post-thrombotic syndrome is characterised by lasting leg pain and swelling, it can progress over many months and years after being diagnosed with DVT.²

Signs and symptoms

The only clinical exhibition of lesions caused by Mondor’s disease is cord-like profound indurations on the surface of the skin, as the lesions are simply made of an obstructed hypodermal vessel. The skin exhibits extensive changes in elasticity and restricted inflammatory changes. Due to the abundant, vertically-oriented vein network, indurations most commonly appear as longitudinal on the anterolateral thoracoabdominal wall. Indurations during PMD emerge on the dorsal and dorsolateral aspects of the penis because the inferior part of the superficial vein system of the abdominal wall is involved. Some scientists also claim that a case of uncharacteristic PMD can alter the circumflex vein; during AWS, the distal parts of the axially surgical scar acquire indurations that lengthen to numerous distances from the mid-upper arm to the base of the thumb. 

Patients regularly experience pain with associated restricted shoulder movement. In addition to that, tightness, fever, erythematous skin change and movement distress in muscles are commonly displayed amongst individuals. These physical symptoms can also trigger psychological distress in some patients due to the unfamiliarity with symptoms and the disease, especially when the lesions appear on sensitive areas like the penis or cervix. When the disease arises due to an underlying source, affiliated symptoms, including a high-grade fever or malaise manifest.¹

Causes and risk factors

The exact cause of the disease is unknown, but susceptible factors involving vessel-wall damage and stasis which contribute to developing the disease may include:

• Trauma – which can be caused by surgery, muscular strains, and sexual actions
• Infections – associated with secondary syphilis, herpes and tuberculosis
• Venous compression – usually shown after wearing tight clothes, presence of tumours and having large breasts 
• Vasoconstrictor medications

The prevalence of the disease is unknown but is perhaps higher than the cases reported. Currently, there are roughly only 400 cases recorded in the medical literature. But research has shown that for most breast cancer surgery and aesthetic mammoplasties, the rates have been estimated at 1%. Mondor’s disease generally affects middle-aged women more frequently than males, and when it comes to PMD, the risk is higher in  sexually active men.

The believed risk factors related to developing the disease are:

• Prolonged menstruation in women
• Having a history of cancer
• Living an unhealthy lifestyle (smoking and obesity-related)
• Varicose veins that develop a case of superficial thrombophlebitis
• Pregnant women whom recently given birth
• Women who are currently on hormonal birth control
• Individuals who are living with a pacemaker or catheter 
• Having a family history of problems with the blood clotting process
• Stroke patients
• People over the age of 60

Diagnosis

When working with such a complex and rare disease it is essential to take the detailed medical history of patients and run as many diagnostic tests as possible. A differential diagnosis of the risk factors based on a case history can help confirm the diagnosis. A physical assessment should present a small cord-like lesion with a hard surface beneath the dermal layer. If there are signs of inflammation around the region, then the disease has reached an advanced state. A very key element in the differential diagnosis process is to understand whether the disease is due to an idiopathic or underlying cause.

Patients prone to hypercoagulation should be tested for protein C and S deficiency, antithrombin-3 deficiency, Leiden mutation and systemic metastasis of carcinomas. In addition to that, inflammatory vascular diseases should also be taken into consideration when tested for chronic diseases.¹ Thrombosed veins associated with the disease are generally detected using a sonogram, which would display a subcutaneous tubular structure. Generally, biopsies are also useful for confirming other potential causes of the symptoms, but they should only be considered if the patient is suspected of vascular inflammation for over 8 weeks.

There are different forms of clinical diagnosis that all aid in reaching a final diagnosis. Ultrasonography typically shows a swollen flowless vein with some sort of beaded appearance. Whereas histopathology is useful for rare lesions, where the vessel walls show proliferative connective tissue. Immunohistochemical staining can characterize blood and lymphatic vessels, which may be helpful in advanced proliferation exhibited within the vessels.² Some cases involve performing a venogram, in which a dye is injected into the vein which can be displayed on an X-ray machine. 

Treatment options

There is no proper standardised method to treat the disease, naturally benign cases can resolve on their own within 4 to 8 weeks. Non-steroidal anti-inflammatory drugs can relieve any painful symptoms associated with the disease. Some physicians recommend the use of common anticoagulant medications, but it is still very controversial as some doctors do not agree. Mondor’s Disease can sometimes occur in different cancers and so regular examinations for different types of cancers is a good form of preventative management. Additionally, for variants of the disease, the management would involve treatments for the local regions. So, for PMD, sexual abstinence, heparin ointment, and oral non-steroidal anti-inflammatory drugs are recommended for male patients. For AWS, physical therapy, and NSAIDs, may be used in conjugation to eliminate the discomforting symptoms.¹

Blood thinners such as low molecular heparin, halt the thrombosis process making it longer to develop a clot, however, they can cause excessive bleeding. Clot dissolving medications initiate thrombolysis to dissolve blood clots in DVT and pulmonary embolism patients. Furthermore, compression stockings help reduce leg swelling throughout the day and consequently decrease the chances of other complications.²

Prognosis

Can Mondor’s disease increase my risk of breast cancer?

Again due to the nature of the disease and how rare it is, no proper long-term reports have been documented. The correlation between Mondor’s Disease and other sites of superficial thrombophlebitis remains ambiguous. The development of secondary Mondor’s Disease would then depend on the prognosis of the underlying root cause of the disease. Lesions in the chest wall are regularly diagnosed often with women’s breast screening, whilst PMD is screened at urology clinics for men. The disease is likely to be a lot more common than reported at clinics, this is because the lesions are habitually non-painful and resolve without external intervention. Therefore, most patients cease seeking direct medical attention.¹

If a patient is living with Mondor's disease, it doesn't increase their chances of developing breast cancer. It is understood that Mondor's disease sometimes is caused by breast carcinoma, but this association is by no means exceptional and implies that mammography should always be performed for Mondor's disease. Nonetheless, it is still very important to be aware of how common breast cancer is and go back to your GP if you notice any minor changes.²

Conclusion

Mondor's disease is a very rare disease that appears as a cord-like induration under the skin layer. The majority of the patient's cases have thrombophlebitis, which is swelling of a vein usually caused by a blood clot. The disease under normal conditions is self-limited and can resolve by itself within 4 to 8 weeks, without the need to receive external treatments. Despite that, some cases may occur as a result of another underlying disease, such as vasculitis. In those cases, physicians must be able to effectively perform a differential diagnosis to identify the main disorder contributing to the discomfort of the patient and perform the necessary treatments and disease management procedures.