Peyronie's Disease

As the channel for semen and urine, the penis serves two important functions in men. But a disease described as early as the mid-18th century by a French physician, Francois Gigot de la Peyronie, which causes hardened patches on the penile shaft, can severely impact a man's sexual performance. If you have pain and penile curvature characteristic of Peyronie's disease, the following information should help you understand your condition.

What happens under normal conditions?

The penis is a cylindrical organ consisting of three chambers: paired corpora cavernosa that are surrounded by a protective tunica albuginea; a dense, elastic membrane or sheath under the skin; and the corpus spongiosum, a singular channel, located centrally beneath and surrounded by a thinner connective tissue sheath. It contains the urethra, the narrow tube that carries urine and semen out of the body.

These three chambers are made up of highly specialized, sponge-like erectile tissue filled with thousands of venous cavities, spaces that remain relatively empty of blood when the penis is soft. But during erection, blood fills the cavities, causing the corpora cavernosa to balloon and push against the tunica albuginea. While the penis hardens and stretches, the skin remains loose and elastic to accommodate the changes.

What is Peyronie's disease?

Peyronie's disease (also known as fibrous cavernositis) is an acquired inflammatory condition of the penis. It is the formation of a plaque or hardened scar tissue beneath the skin of the penis. This scarring is non-cancerous, but often leads to painful erection and curvature of the erect penis (a "crooked penis").

What are the symptoms of Peyronie's disease?

This scarring, or plaque, typically develops on the upper side of the penis (dorsum). It reduces the elasticity of the tunica albuginea in that area and, as a result, causes the penis to bend upward during an erection. Although Peyronie's plaque is most commonly located on the top of the penis, it may occur on the underside or on the lateral side of the penis, causing a downward or lateral bend. Some patients may even develop a plaque that goes all the way around the penis, causing a "waisting" or "bottleneck" deformity of the penile shaft. The majority of patients complain of generalized shrinkage or shortening of their penis.

Painful erections and difficulty with intercourse usually lead men with Peyronie's disease to seek medical help. Since there is great variability in this condition, sufferers may complain of any combination of symptoms: Penile curvature, obvious penile plaques, painful erection and diminished ability to achieve an erection.

Any of those physical deformities make Peyronie's disease a quality-of-life issue. Not surprisingly, it is linked to erectile dysfunction in 20 to 40 percent of sufferers. While studies have shown that 77 percent of men demonstrate significant psychological effects, the numbers, medical researchers believe, are under-reported. Instead, many men affected with this truly devastating condition suffer in silence.

How frequently does Peyronie's disease occur?

Peyronie's disease affects a reported one to 3.7 percent (about one to four in 100) of males between ages 40 and 70, even though severe cases have been reported in younger men. Medical researchers believe the actual prevalence may be higher due to patient embarrassment and limited reporting by physicians. Since the introduction of an oral therapy for impotence, doctors have reported an increased incidence of Peyronie's cases. With more men being treated successfully for erectile dysfunction in the future, an increasing number of cases presenting to urologists are anticipated.

What causes Peyronie's disease?

Ever since Francois Gigot de la Peyronie, personal physician to King Louis XV, first reported penile curvature in 1743, scientists have been mystified by the causes of this well-recognized disorder. Yet medical researchers have speculated on a variety of factors that might be at work.

Most experts believe that acute or short-term cases of Peyronie's disease are likely the consequence of a minor penile trauma, sometimes caused by sports injuries, but more often by vigorous sexual activity (e.g., the penis accidentally being jammed into a mattress). In injuring the tunica albuginea, that trauma triggers a cascade of inflammatory and cellular events resulting in the abnormal fibrosis (excess fibrous tissue), plaque and calcifications characteristic of this disease.

Such trauma, however, may not account for those Peyronie's cases that begin slowly and become so severe that they require surgery. Researchers believe genetics or relationship with other connective tissue disorders may play a role. Studies already suggest that if you have a relative with Peyronie's disease you have a greater risk of developing it yourself.

How is Peyronie's disease diagnosed?

A physical examination is sufficient to diagnose curvature of the penis. The hard plaques can be felt with or without erection. It may be necessary to use injectable medications to induce an erection for proper evaluation of the penile curvature. The patient may also provide pictures of the erect penis for evaluation by the physician. Ultrasound of the penis may demonstrate the lesions in the penis but is not always necessary.

How is Peyronie's disease treated?

Because Peyronie's disease is a wound-healing disorder, changes are constantly occurring in the early stages. In fact, this disease can be classified into two stages: 1) an acute inflammatory phase persisting for six to 18 months during which men experience pain, slight penile curvature and nodule formations and 2) a chronic phase during which men develop a stable plaque, significant penile curvature and erectile dysfunction.

Occasionally the condition regresses spontaneously with symptoms resolving themselves. In fact, some studies show that approximately 13 percent of patients have complete resolution of their plaques within a year. There is no change in 40 percent of cases, with progression or worsening of symptoms in 40 to 45 percent. For these reasons, most physicians recommend a non-surgical approach for the first 12 months.

Conservative approaches: Instead of requiring invasive diagnostic procedures or treatments, men who experience only small plaques, minimal penile curvature and no pain or sexual limitations, need only be reassured that the condition will not lead to malignancy or another chronic disease. Pharmaceutical agents have shown promise for early-stage disease but there are drawbacks. Because of a lack of controlled studies, scientists have yet to establish their true effectiveness. For instance:

• Oral vitamin E: It remains a popular treatment for early-stage disease because of its mild side effects and low cost. While uncontrolled studies as far back as 1948 demonstrated decreases in penile curvature and plaque size, investigation continues concerning its effectiveness.

• Potassium aminobenzoate: Recent controlled studies have shown that this B-complex substance popular in Central Europe yields some benefits. But it is somewhat expensive, requiring 24 pills each day for three to six months. It is also often associated with gastrointestinal issues, making compliance low.

• Tamoxifen: This non-steroidal, antiestrogen medication has been used in the treatment of desmoid tumors, a condition with properties similar to Peyronie's disease. Researchers claim that inflammation and the production of scar tissue are inhibited. But early-stage disease studies in England have found only marginal improvement with tamoxifen. Like other research in this area, however, these studies include few patients, and no controls, objective improvement measures or long-term follow up.

• Colchicine: Another anti-inflammatory agent that decreases collagen development, colchicine has been shown to be slightly beneficial in a few small, uncontrolled studies. Unfortunately, up to 50 percent of patients develop gastrointestinal upset and must discontinue the drug early in treatment.

Injections: Injecting a drug directly into the penile plaque is an attractive alternative to oral medications, which do not specifically target the lesion, or invasive surgical procedures, which carry the inherent risks of general anesthesia, bleeding and infection. Intralesional injection therapies introduce drugs directly into the plaque with a small needle after appropriate anesthesia. Because they offer a minimally invasive approach, these options are popular among men with either early phase disease or who are reluctant to have surgery. Yet their effectiveness is also under investigation. For instance:

• Verapamil: Early uncontrolled studies demonstrated that this substance interferes with calcium, a factor shown by in vitro cattle connective tissue cell studies to support collagen transport. As such, intralesional verapamil reduced penile pain and curvature while improving sexual function. Other studies have concluded that it is a reasonable treatment in men with non-calcified plaques and penile angles of less than 30 degrees.

• Interferon:: The use of these naturally-occurring antiviral, antiproliferative and anti-tumorigenic glycoproteins to treat Peyronie's disease was born out of experiments demonstrating the antifibrotic effect on skin cells of two different disorders - keloids, overgrowth of collagenous scar tissue and scleroderma, a rare autoimmune disease affecting the body's connective tissue. In addition to inhibiting proliferation of fibroblast cells, interferons, such as alpha-2b, also stimulate collagenase, which breaks down collagen and scar tissue. Several uncontrolled studies have demonstrated intralesional interferon's effectiveness in reducing penile pain, curvature and plaque size while improving some sexual function. A current multi-institutional, placebo-controlled trial will hopefully answer many of the questions about intralesional therapy in the near future.

Other investigative therapies: The medical literature is replete with reports on less invasive methods for treating Peyronie's disease. But the effectiveness of treatments such as high-intensity focused ultrasound and radiation therapy, topical verapamil and iontophoresis, introducing soluble salt ions into the tissue via electric current, must still be investigated before these alternative therapies are considered clinically useful. Likewise, controlled studies using larger patient groups with longer follow ups are necessary to prove that the same high-energy shock waves used to break up kidney stones will have positive effects on Peyronie's disease.

Surgery: Surgery is reserved for men with severe disabling penile deformities that prevent satisfactory sexual intercourse. But, in most cases, it is not recommended for the first six to 12 months, until the plaque has stabilized. Since a spin-off of this disease is an abnormal blood supply to the penis, a vascular evaluation using vasoactive agents (drugs that cause erections by opening the vessels) is done prior to any surgery. A penile ultrasound if performed can also illustrate the anatomy of the deformity. The images allow the urologist to determine which patients are most likely to benefit from reconstructive procedures versus a penile prosthesis. The three surgical approaches include:

• Nesbit procedure: First described to correct congenital penile curvature by cutting a portion of tissue from the tunica albuginea and shortening the unaffected side of the penis, this procedure is used by many surgeons today for Peyronie's disease. Variations on the approach include the plication technique, where sutured tucks are placed into the side of maximum curvature to shorten and straighten the penis and the corporoplasty technique, where a longitudinal or lengthwise incision is closed transversely to correct the curvature. Nesbit and its variations are simple to perform and involve limited risk. They are most beneficial in men with ample penile length and lesser degrees of curvatures. But they are not recommended in individuals with short penises or severe curvatures as this procedure is recognized to shorten the penis somewhat.

• Grafting procedures: When plaques are large and curvatures severe, the surgeon may choose to incise or cut out the hardened area and replace the tunica defect with a graft material of some type. While the choice of materials depends on the doctor's experience, preferences and what is available, some are more attractive than others. For instance:

  • Autograft tissue grafts: Taken from the patient's body during surgery and thus less likely to cause an immunologic reaction, these materials usually require a second incision. They are also known to undergo postoperative contracture or tightening and scarring.

  • Synthetic inert substances: Materials such as Dacron® mesh or GORE-TEX® can cause significant fibrosis, a spreading of connective tissue cells. Occasionally palpated or felt by the patient, these grafts may cause more scarring.

  • Allografts or xenografts: Harvested human or animal tissues are the focus of most grafting material today These substances are uniformly strong, easy to work with and readily available because they are "off-the-shelf" in the operating room, so to speak. They act as scaffolds for the tunica albuginea tissue to grow over as the graft is naturally dissolved by the patient's body.

• Penile prostheses: A penile prosthesis may be the only good option for Peyronie's disease patients with significant erectile dysfunction and insufficient blood vessels verified by ultrasound. In most cases, implanting such a device alone will straighten the penis, correcting its rigidity. But when that does not work, the surgeon may manually "model" the organ, bending it against the plaque to break the deformity, or the surgeon may need to remove the plaque over the prosthesis and apply a graft to completely straighten the penis.

What can be expected after treatment for Peyronie's disease?

Routinely, a light pressure dressing is applied for 24 to 48 hours after the surgery to prevent any accumulation of blood. The Foley catheter is removed after the patient recovers from anesthesia and most patients are discharged later the same day or the following morning. During the healing process, medications to counteract erections are usually prescribed. The patient is also asked to take antibiotics for seven to 10 days postoperatively to ward off infection, and analgesics for any discomfort. If patients have no penile pain or other complications, they can resume sexual intercourse in six to eight weeks.

Frequently asked questions:

What happens to the cells following penile trauma?

In theory, following any penile trauma, there is a release of growth factors and cytokines or daughter cells that activate fibroblasts, cells that produce connective tissue. They, in turn, cause abnormal collagen deposition or scarring, which damages the internal elastic framework of the penis. Similar wound-healing disorders are commonly seen in the practice of dermatology, with conditions such as keloids and hypertrophic scarring, both involving tissue overgrowth in wound healing.

Are Peyronie's disease sufferers prone to other related conditions?

About 30 percent of Peyronie's disease sufferers also develop other systemic fibrosis in other connective tissue in the body. Common sites are the hands and feet. In Dupuytren's contracture, scarring or thickening of the fibrosis tissue in the palm leads progressively to a permanent bending of the pinkie and ring fingers into the hand. While the fibrosis occurring in both diseases is similar, it is not clear yet what causes either plaque type or why men with Peyronie's disease are more likely to develop Dupuytren's contracture.

Will Peyronie's disease evolve into cancer?

No. There are no documented cases of progression of Peyronie's disease to malignancy. However, if your doctor observes other findings that are not typical with this disease-such as external bleeding, obstructed urination, prolonged severe penile pain - he or she may elect to perform a biopsy on the tissue for pathological examination.

What should men remember about Peyronie's disease?

Peyronie's disease is a well-recognized but poorly understood urological condition. Interventions need to be individualized to each patient, based on the timing and severity of the disease. The objective of any treatment should be on reducing pain, normalizing penile anatomy so that intercourse is comfortable and restoring erectile function in patients who suffer erectile dysfunction. Although surgical correction is ultimately successful in the majority of cases, the early acute phase of this disease is customarily treated by either oral and/or intralesional approaches. As medical researchers continue to develop basic and clinical research for a better understanding of this disease, more therapies and targets for intervention will become available.