What Is Penile Mondor's Disease?

Penile Mondor's disease (PMD) is a rare disorder that causes thrombophlebitis (inflammation leading to blood clots) in the superficial veins of the penis, manifesting with transient pain and swelling. PMD often occurs as a result of trauma to the penis or prolonged or vigorous sex and is believed by some to be of genetic origin.

The diagnosis of PMD can be made with a physical exam and ultrasound. The treatment of this otherwise benign and self-limiting condition is typically supportive to relieve pain and inflammation.

What Is Penile Mondor's Disease?

Penile Mondor's disease is one manifestation of Mondor's disease, a condition first described by French physician Henri Mondor in 1939. Mondor's disease is characterized by thrombophlebitis of the chest wall and breast that can also extend to the arm and penis.

The first case of Mondor's disease isolated to the penis was described in 1958 and was subsequently dubbed PMD.

Thrombophlebitis is an inflammatory process occurring in veins that leads to the formation of blood clots (thrombi). It is typically caused by disorders that increase blood clotting. These include certain genetic disorders that run through families. For example, hereditary antithrombin III deficiency is linked to deep vein thrombosis (DVT).

With PMD, thrombophlebitis typically occurs in the superficial dorsal vein situated on the top of the penis that runs the length of the penis. The smaller tributary veins that branch off the dorsal vein can also be affected.

Despite their absence in medical literature, many cases of PMD are likely to go undiagnosed because people may be too embarrassed to see their doctor. And, when they do, the symptoms may already have begun to resolve or have disappeared completely.

Symptoms

PMD tends to occur after a precipitating event, such as prolonged and vigorous sex. The first sign is usually the palpable hardening of part of the dorsal vein on top of the penis, usually within 24 to 48 hours of the event. This will create a rope-like mass just beneath the skin running anywhere from 1 to 4 inches in length.

In addition to the formation of a hardened lesion, symptoms of PMD may include:

• Episodic or continuous pain or throbbing
• Pain with an erection
• Edema (tissue swelling due to fluid build-up)
• Erythema (superficial reddening of the skin)
• Difficulty urinating

Some cases may only involve the formation of a hardened vein on top of the penis with no other symptoms. Other cases may occur in tandem with the formation of similar thrombotic lesions on the breast, chest, or arm.

Some cases of PMD are a one-time, isolated event. Others may be recurrent and be instigated by the same or similar precipitating event.

Causes

Because PMD is so rarely diagnosed, the exact cause of the condition is poorly understood. Based on the current body of case reports, PMD usually arises as a result of mechanical trauma to the penis.

At the same time, there is evidence that PMD may be secondary to disease, infections, or surgeries that directly or indirectly affect the penis.

The list of possible causes is extensive and may include:

• Prolonged, vigorous, or frequent sex
• Vigorous sex after prolonged abstinence
• Penile injury
• Use of penile vacuum pumps
• Use of injected intracavernous drugs to treat erectile dysfunction
• Syphilis
• Penile candidiasis (thrush)
• Behçet's disease
• Pelvic cancers, including prostate cancer and bladder cancer
• Metastatic pancreatic cancer
• Surgeries such as inguinal hernia repair, orchiopexy (used to repair an undescended testicle), and varicocelectomy (used to treat varicoceles)

Genetics

Clearly, not anyone with the risk factors listed above will develop PMD. Because of this, many scientists believe that certain people have a genetic predisposition for PMD.

There are several gene mutations linked to PMD that can place a person in a hypercoagulative state (meaning prone to excessive blood clotting). This includes the aforementioned antithrombin III deficiency as well as protein S deficiency, protein C deficiency, factor V Leiden mutation, and PT 20210 mutation.

What all these disorders share is an autosomal dominant pattern of inheritance, meaning that only one gene mutation from one parent is needed for the child to develop the disease (in this case, hypercoagulation). Moreover, the parent with the gene mutation will also have the disorder.

(By contrast, an autosomal recessive pattern is one in which both parents contribute a gene mutation that, together, leads to the disorder. In most cases, the parents will be "carriers" of the mutation but not have the disorder themselves.)

Despite the association, not everyone with PMD will have these or any other gene mutation linked to hypercoagulation. As such, it is still unclear how much genetics influence the likelihood of PMD in relationship to other known risk factors.

Diagnosis

You can take your concerns to a primary care physician or make an appointment with a specialist called a urologist who specializes in diseases of the male reproductive system.

PMD can often be diagnosed with a physical exam and a review of the person's medical history. An ultrasound and other tests may be used to confirm the diagnosis.

Physical Exam

The physical examination will usually reveal classic signs of PMD, most predominately the hardened, rope-like vein along the top of the penis. It is not uncommon for the lesion to extend above the pubic bone.

In reviewing the person's medical history, the doctor will assess whether there are any risk factors linked to PMD (such as a history of STIs or the use of intracavernous drugs).

In many cases, the appearance of the lesion will have occurred 24 to 48 hours after prolonged or vigorous sex. Other cases may be idiopathic (of unknown origin), possibly due to penile injury a long time ago.

Ultrasound

To better ensure that PMD is the cause of the symptoms, the doctor may order a color Doppler ultrasound to differentiate it from a similar condition called nonvenereal sclerosing lymphangitis (NVSL). Also caused by vigorous sex, NVSL of the penis involves the obstruction of lymphatic vessels rather than blood vessels.

A color Doppler ultrasound is an imaging test that uses sound waves to show blood moving through blood vessels. It shows the flow in the arteries into and the veins out of the penis. (A traditional ultrasound also uses sound waves to create images, but it can't show blood flow.) Changes in color correspond to the speed and direction of the blood flow.

On a color Doppler ultrasound, the blood flow in the dorsal vein will be slowed in someone with PMD but not in someone with NVSL.

In addition to the abnormal curve (which typically does not occur with PMD), Peyronie's disease is characterized by scarring not in the blood vessels but in the membrane surrounding the spongy interior of the penis (called the tunica albuginea). On a color Doppler ultrasound, there will be no evidence of restricted blood flow in the dorsal vein.

Other Tests

Other tests may be ordered if PMD is believed to be secondary to an underlying disease. This may include an STI screen if syphilis is suspected. Enlarged lymph nodes in the groin may warrant a preliminary investigation of cancer, including the use of the prostate-specific antigen (PSA) test to help detect prostate cancer.

On rare occasions, genetic tests may be ordered to screen for hypocoagulative disorders. Even so, they are not commonly used, as a positive result would do little if anything to alter the treatment plan.

Treatment

PMD is typically a self-limiting, benign condition that will resolve on its own without treatment. The treatment of PMD is generally supportive to relieve pain and inflammation.

Topical and Oral Therapies

Topical preparations containing nonsteroidal anti-inflammatory drugs (NSAIDs) like Voltaren (diclofenac) are sometimes used to reduce inflammation in people with PMD. Topical creams containing the anticoagulant heparin may also be prescribed to help break down the blood clot. Neither preparation is known to be consistently beneficial.

More controversial is the use of oral heparin to treat refractory (treatment-resistant) PMD. Although it may be considered if the condition is severe and doesn't resolve after six weeks, the side effects of oral heparin (including easy bleeding and liver toxicity) tend to outweigh the possible benefits.

Surgery

If PMD is persistent and severe, surgery may be a more reasonable—albeit invasive—option. This would typically involve a thrombectomy to surgically remove the blood clot accompanied by the resection (removal) of the affected dorsal vein.

Penile thrombectomy with resection can usually be performed on an outpatient basis. The healing and recovery time takes around eight weeks.