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NORD is very grateful to Wayne J. Hellstrom, MD, for updating the content of this report and Stephanie McAllister Bartlett for editorial assistance, at the Tulane University Health Sciences Center, Department of Urology.
Peyronie's disease is a rare connective tissue disorder characterized by the development of fibrous plaques on surrounding fascial layer of the adult male penis. Affected individuals may experience pain, have cord-like lesions on the penis, and/or exhibit abnormal curvature of the penis when erect. In some cases, these conditions may make normal sexual intercourse impossible for affected individuals unless treated. Symptoms may be chronic or spontaneously resolve in 3.2-12% of patients. The exact cause of Peyronie's disease is not known.
Peyronie's disease is characterized by dense infiltration of fibrous tissue into the surrounding layer (tunica albuginea) of the penis. These strands of fibrosis or scar may also appear in patches of various sizes on the penis (plaques). Formation of the plaque limits the elasticity of the penis and can cause pain and curvature upon erection. In some cases, symptoms may eventually lead to erectile dysfunction (ED). In addition, the affected tissue may become calcified. Some individuals with Peyronie's disease have been found to have deposits of excess collagen in connective tissue in other parts of the body as well.
In some cases, a condition known as Dupuytren's contracture has also been associated with Peyronie’s disease. Dupuytren's contracture is a rare connective tissue disorder characterized by fixation of the joints (e.g., proximal interphalangeal joints and metacarpophalangeal joints) and certain fingers permanently in a flexed position (joint contractures). Due to abnormal thickening and shortening of the bands of fibrous tissue beneath the skin of the palm (palmar fascia), a hardened nodule may develop, eventually forming an abnormal band of hardened (fibrotic) tissue. As a result, the fingers of the affected area begin to be "drawn in" toward the palm over several months or years and cannot be pulled back (contracture). (For more information on this disorder, choose "Dupuytren's Contracture" as your search term in the Rare Disease Database.)
The exact cause of Peyronie's disease is not known, but is believed to involve repeated micro-trauma in men with an underlying healing disorder. The disorder was thought to possibly be induced in some cases by the use of beta-adrenergic blocking drugs, which is no longer the case.
Other researchers believe it may be inherited as an autosomal dominant genetic trait, as these appear to be a family predisposition in certain cases. Dominant genetic disorders occur when only a single copy of an abnormal gene is necessary to cause a particular disease. The abnormal gene can be inherited from a parent or can be the result of a new mutation (gene change) in the affected individual. The risk of passing the abnormal gene from affected parent to offspring is 50%.
Peyronie's disease is a rare connective tissue disorder that affects adult males, usually during the fourth and fifth decades of life. Affected individuals have been diagnosed with this disorder ranging from 18 to 80 years of age. Peyronie's disease was first described in 1743 by Francois de la Peyronie, court physician to King Louis XV. Recent studies have suggested that up to 3-9% of the male population in the United States may be affected to varying degrees.
Symptoms of the following disorders can be similar to those of Peyronie's disease. Comparisons may be useful for a differential diagnosis:
Balanitis xerotica obliterans is the result of chronic inflammation and is characterized by a hardened (indurated), pale area surrounding the end of the penis that may cause constriction. Treatment with antibacterial and anti-inflammatory drugs may be useful, but surgery may be required in some cases.
Erythroplasia of Queyrat is a premalignant lesion characterized by an area of reddish, velvety discoloration on the penis. Biopsy should be considered for diagnostic purposes. Treatment consists of local application of fluorouracil cream.
A medical history and physical examination are usually sufficient for the diagnosis. The plaque formed can usually be felt upon examination and in most cases may be found on the upper (dorsal) or sometimes lower (ventral) side of the shaft of the penis.
In some cases, 10-15%, treatment of Peyronie's disease may not be required since symptoms may resolve spontaneously over a period averaging from 8 to 12 months. In most cases, the condition may persist and become disabling.
Conservative treatment protocols include vitamin E, colchicine, or paraaminobenzoic acid. Unfortunately, no long-term controlled studies with oral drug placebos have shown any benefits. Leading clinical researchers in this field now prescribe intralesional therapy with verapamil and interferon-alpha 2b injections. Phase 2 trials with intralesional collagenase (Xiaflex manufactured by Auxilium Pharmaceuticals) documented a significant decrease compared to placebo in both degree of penile curvature (-32.4% vs -2.5%, P<0.001) and Peyronie's bother-score end points (-3.6 vs -0.2, P=0.004) when combined with a modelling procedure. Because collagenase injection was well tolerated and safe, phase 3 trials with collagenase in combination with modelling are currently in progress.
It is well-documented that gradual expansion of tissue results in the formation of new bone and connective tissue. Penile traction has conventionally been used to increase penile length but has recently been evaluated for reducing the curvature associated with Peyronie's disease and the initial reports of studies are promising.
Radiation therapy is contraindicated in cases that fail to respond to drug treatment. Surgery to correct the curvature of the penis is generally effective, although side effects such as erectile dysfunction and loss of penile sensation may develop.
Oral medication and intralesional medication are the usual first steps in treatment. If the condition persists, after a period of approximately 12 months, surgery may also be considered as a treatment option.
Surgical procedures include plicating tunical tissue on the side of the penis opposite the concavity to correct bending, incising or excising the plaque and grafting tissues from other parts of the body or from other sources to cover the deficient area, and in some cases implanting a penile prosthesis to straighten the penis and help the individual achieve a satisfactory erection.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
The drug AA4500 has been granted orphan status by the U.S. Food and Drug Administration (FDA) (2005) as a possible treatment for Peyronie's disease. The sponsors, Auxilium Pharmaceuticals of Malvern, Pa., and Cobra Biomanufacturing of Keele, UK, are completing Phase II's and planning Phase III trials. For information, contact:
The drug collagenase (lyophilized) for injection received an orphan drug designation in 1996 for the experimental treatment of Peyronie's disease. Collagenase is an enzyme that lyses the fibrous tissues. Clinical trials have shown treatment success and collagenase is currently (2005) one of the non-surgical treatment options in some cases of Peyronie's disease. For more information on collagenase (injectable) and collagenase (ointment), contact:
BioSpecifics Technologies Corp.
35 Wilbur Street
Lynbrook, NY 11563
Several non-surgical treatments are prescribed to manage Peyronie's disease. All of them are considered investigational and have shown various degrees of success. In addition to the above-mentioned collagenase, shock wave therapy has been prescribed as well as a range of oral and topical medications. Research continues on these investigational therapies.
A calcium channel-blocking drug known as verapamil and alpha 2b interferon has been used to treat individuals with Peyronie's disease. The drug is injected directly into the affected area (intraplaque injection). Prescription Dispensing Laboratories of San Antonio, Texas, is investigating the use of a topical compound made from verapamil for the treatment of Peyronie's disease, however recent evidence for the topical approach is not supportive.
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Report last updated: 2012/02/07 00:00:00 GMT+0