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Peyronie's disease

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

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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.

Synonyms of Peyronie's disease

Disorder Subdivisions

General Discussion

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.

Symptoms

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.)

Causes

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%.

Affected Populations

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.

Related Disorders

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.

Standard Therapies

Diagnosis
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.

Treatment
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.

Investigational Therapies

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
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

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:

Auxilium Pharmaceuticals
Telephone: 1-484-321-5902
www.auxilium.com

Cobra Biomanufacturing
44-(0)-1782-714-181
www.cobrabio.com

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
Tel: 516-593-7000
Website: www.biospecifics.com

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.

Peyronie's disease Resources

Organizations:

References

TEXTBOOKS
Hellstrom WJG, Reddy SK. Peyronie's Disease. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:25-26.

Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:1833-34.

Berkow R., ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:1058-59.

REVIEW ARTICLES
Serefoglu EC, Hellstrom WJ. Treatment of Peyronie's Disease: 2012 Update. Curr Urol Rep. 2011;12(6):444-52.

Hellstrom WJG. Medical Management of Peyronie's disease. J Androl. 2009 Jul-Aug:30(4)379-405.

Hellstrom WJ, Usta MF. Surgical approaches for advanced Peyronie's disease patients. Int J Impot Res. 2003;15 Suppl 5:S121-24.

Levine LA. Review of current nonsurgical management of Peyronie's disease. Int J Impot Res. 2003;15 Suppl 5:S113-20.

Levine LA, Greenfield JM. Establishing a standardized evaluation of the man with Peyronie's disease. Int J Impot Res. 2003;15 Suppl 5:S103-12.

Mulhall JP. Expanding the paradigm for plaque development in Peyronie's disease. Int J Impot Res. 2003;15 Suppl 5:S93-102.

Hellstrom WJ. History, epidemiology, and clinical presentation of Peyronie's disease. Int J Impot Res. 2003;15 Suppl 5:S91-92.

JOURNAL ARTICLES
Trost LW, Gur S, Hellstrom WJ. Pharmacological management of Peyronie's disease. Drugs. 2007; 67(4);527-45.

Mulhall JP, Schiff J, Guhring P. An analysis of the natural history of Peyronie's disease. J Urol. 2006;175:2115-8.

Hauck EW, Hauptmann A, Bschleipfer T, et al. Quesytionable efficacy of extracorporeal shock wave therapy for Peyronie's disease: results of a prospective approach. J Urol. 2004;171:296-99.

Usta MF, Bivalacqua TJ, Sanabria J, et al. Patient and partner satisfaction and long-term results after surgical treatment for Peyronie's disease. Urology. 2003;62:105-09.

Wilkins CJ, Sriprasad S, Sidhu PS. Colour Doppler ultrasound of the penis. Clin Radiol. 2003;58:514-23.

Levine LA, Estrada CR, Storm DW, et al. Peyronie diseases in younger men: characteristics and treatment results. J Androl. 2003;24:27-32.

Kadioglu A, Tefekli A, Erol B, Oktar T, Tunc M, Tellaloglu S. A retrospective review of 307 men with Peyronie's disease. J Urol. 2002;68:1075-9.

Hellstrom WJ, et al. Peyronie's disease: etiology, medical, and surgical therapy. J Androl. 2000;21:347-54.

Montorsi F, et al. Transdermal electromotive multi-drug administration for Peyronies' disease: preliminary results. J Androl. 2000;21:85-90.

Riedl CR, et al. Iontophoresis for treatment of Peyronie's disease. J Urol. 2000;163:95-9.

INTERNET
McKusick VA, ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Entry No: 171000; Last Update:September 22, 2009 http://omim.org/entry/171000 Accessed on:February 1, 2012.

Peyronie's Disease. National Kidney and Urologic Diseases Information Clearinghouse. Last Update:September 2, 2010. http://kidney.niddk.nih.gov/kudiseases/pubs/peyronie/index.aspx Accessed on:February 1, 2012.

Lizza E. Peyronie Disease. eMedicine. Last Update:October 10, 2011.
http://emedicine.medscape.com/article/456574-overview Accessed on:February 1, 2012.

Henegar C. Peyronie syndrome. orphanet. January 2004.
www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=2870 Accessed on:February 1, 2012.

Report last updated: 2012/02/07 00:00:00 GMT+0