Definition of Peyronie disease

Peyronie disease: A condition of uncertain cause in which a plaque (a hard lump) forms on the upper or lower side of the penis in layers containing erectile tissue resulting sometimes in severe curvature of the penis. Surgery is usually only recommended in long-term cases in which the deformity causes erectile dysfunction and prevents intercourse. The French surgeon, François de la Peyronie's described the disease in 1743.

Peyronie's disease begins as a localized inflammation and can develop into a hardened scar. Cases range from mild to severe. Symptoms may come on slowly or appear overnight. In severe cases, the hardened plaque reduces flexibility, causing pain and forcing the penis to bend or arc during erection. In many cases, the pain decreases over time, but the bend in the penis may remain a problem, making sexual intercourse difficult. The sexual problems that result can disrupt a couple's physical and emotional relationship and lead to lowered self-esteem in the man. In a small percentage of patients with the milder form of the disease, inflammation may resolve without causing significant pain or permanent bending.

The plaque itself is benign, or noncancerous. A plaque on the top of the shaft (most common) causes the penis to bend upward; a plaque on the underside causes it to bend downward. In some cases, the plaque develops on both top and bottom, leading to indentation and shortening of the penis. At times, pain, bending, and emotional distress prohibit sexual intercourse.

Who gets it? One study found Peyronie's disease occurring in 1% of men. Although the disease occurs mostly in middle-aged men, younger and older men can acquire it. About 30% of people with Peyronie's disease develop fibrosis (hardened cells) in other elastic tissues of the body, such as on the hand or foot. A common example is a condition known as Dupuytren contracture of the hand. In some cases, men who are related by blood tend to develop Peyronie's disease, which suggests that familial factors might make a man vulnerable to the disease.

Course of the disease: The plaque of Peyronie's disease may develop sometimes following trauma (hitting or bending) that causes localized bleeding inside the penis. Two chambers known as the corpora cavernosa run the length of the penis. The inner-surface membrane of the chambers is a sheath of elastic fibers. A connecting tissue, called a septum, runs along the center of each chamber and attaches at the top and bottom.

If the penis is abnormally bumped or bent, an area where the septum attaches to the elastic fibers may stretch beyond a limit, injuring the lining of the erectile chamber and, for example, rupturing small blood vessels. As a result of aging, diminished elasticity near the point of attachment of the septum might increase the chances of injury.

The damaged area might heal slowly or abnormally for two reasons: repeated trauma and a minimal amount of blood flow in the sheath-like fibers. In cases that heal within about a year, the plaque does not advance beyond an initial inflammatory phase. In cases that persist for years, the plaque undergoes fibrosis, or formation of tough fibrous tissue, and even calcification, or formation of calcium deposits.

Other possible causes: Trauma does not explain why most cases develop slowly and with no apparent traumatic event. It also does not explain why some cases disappear quickly, and why similar conditions such as Dupuytren contracture do not seem to result from severe trauma. Some researchers theorize that Peyronie's disease may be an autoimmune disorder.

A number of drugs list Peyronie's disease as a possible side effect. Most of these drugs belong to a class of blood pressure and heart medications called beta blockers. One beta blocker is an eye drop preparation used to treat glaucoma. Other drugs that may cause Peyronie's disease are interferon, used to treat multiple sclerosis, and phenytoin, an anti-seizure medicine. The chances of developing Peyronie's disease from any of these medicines are very low. Patients should check with their doctor before discontinuing any prescribed drug.

Treatment: Because the course of the disease is different in each patient and because some patients experience improvement without treatment, experts usually recommend waiting 1 to 2 years or longer before attempting to correct it surgically.

No controlled studies have established the effectiveness of vitamin E therapy. Similar inconclusive success has been attributed to oral application of para-aminobenzoate, a substance belonging to the family of B-complex molecules. Researchers have injected chemical agents such as verapamil, collagenase, steroids, and calcium channel blockers directly into the plaques. These interventions are still considered unproven because studies have included low numbers of patients and have lacked adequate control groups. Steroids, such as cortisone, have produced unwanted side effects, such as the atrophy or death of healthy tissues.

Radiation therapy, in which high-energy rays are aimed at the plaque, has also been used. Like some of the chemical treatments, radiation appears to reduce pain, but it has no effect at all on the plaque itself and can cause unwelcome and serious side effects.

Surgery: Peyronie disease has been treated with some success by surgery. The two most common surgical methods are removal or expansion of the plaque followed by placement of a patch of skin or artificial material, and removal or pinching of tissue from the side of the penis opposite the plaque, which cancels out the bending effect. The first method can involve partial loss of erectile function, especially rigidity. The second method, known as the Nesbit procedure, causes a shortening of the erect penis.

Some men choose to receive an implanted device that increases rigidity of the penis. In some cases, an implant alone will straighten the penis adequately. In other cases, implantation is combined with a technique of incisions and grafting or plication (pinching or folding the skin) if the implant alone does not straighten the penis.

Most types of surgery produce positive results. But because complications can occur, and because many of the phenomena associated with Peyronie's disease (for example, shortening of the penis) are not corrected by surgery, most doctors prefer to perform surgery only on the small number of men with curvature so severe that it prevents sexual intercourse.

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