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 many affected individuals unless treated. Symptoms may be chronic or, as in 10-15% of cases, spontaneously resolved. 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 fiber may also appear in patches of various sizes on the penis (plaques). Formation of the plaques limit the elasticity of the penis and can cause pain and curvature upon erection. In some cases, symptoms may eventually lead to impotence. 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 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 in a permanently 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.
Chromosomes, which are present in the nucleus of human cells, carry the genetic information for each individual. Human body cells normally have 46 chromosomes. Pairs of human chromosomes are numbered from 1 through 22 and the sex chromosomes are designated X and Y. Males have one X and one Y chromosome and females have two X chromosomes. Each chromosome has a short arm designated "p" and a long arm designated "q". Chromosomes are further sub-divided into many bands that are numbered. For example, "chromosome 11p13" refers to band 13 on the short arm of chromosome 11. The numbered bands specify the location of the thousands of genes that are present on each chromosome.
Genetic diseases are determined by the combination of genes for a particular trait that are on the chromosomes received from the father and the mother. All individuals carry 4-5 abnormal genes. Parents who are close relatives (consanguineous) have a higher chance than unrelated parents to both carry the same abnormal gene, which increases the risk to have children with a recessive genetic disorder.
Dominant genetic disorders occur when only a single copy of an abnormal gene is necessary for the appearance of the disease. The abnormal gene can be inherited from either 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% for each pregnancy regardless of the sex of the resulting child.
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.
Diagnosis A medical history and physical examination are usually sufficient for the diagnosis. The plaque formed can usually be felt upon examination and may be found on the upper (dorsal) or lower (ventral) side of the shaft of the penis.
Treatment
In some cases, 10-15% of treatment of Peyronie's disease may not be required since symptoms may resolve spontaneously over a period averaging from 8 to 12 months. In other cases, the condition may persist and become disabling.
Conservative treatment protocols or progress include vitamin E, or colchicine, or paraaminobenzoic acid. Unfortunally, no long-term controlled studies with oral drug placebos have shown any benefits. Leading clinical researchers in this field now prescribe intralesional interferon therapy with verapamil and interferon-alpha 2b injections.
Radiation therapy is contraindicated in cases that fail to respond to drug treatment. Surgery to correct the curvature of the penis may be effective, although side effects such as erectile dysfunction may develop.
Oral medication and injected 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 may include phicating tissue on the side of the penis opposite the plaque to correct bending, incising or excising the plaque and grafting skin from other parts of the body or from other sources to cover the area, and in some cases implanting a prosthesis to 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:
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
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 have 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.
McKusick VA, ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Entry No: 171000; Last Update: 8/24/98.
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 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 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.
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.
Trost LW, Gur S, Hellstrom WJ. Pharmacological management of Peyronie's disease. Drugs. 2007; 67(4);527-45.
FROM THE INTERNET Peyronie's Disease. National Kidney and Urologic Diseases Information Clearinghouse. December 2003. 5pp. www.niddk.nih.gov/kudiseases/pubs/peyronie/index.htm
Laumann A. Peyronie Disease. emedicine. Last Updated: May 14, 2003. 21pp. www.emedicine.com/derm/topic851.htm
Henegar C. Peyronie syndrome. orphanet. January 2004. 1p. www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=2870
Henegar C. Clinical signs of Peyronie disease. orphanet. nd. 1p. www.orpha.net/data/patho/GB/peyronie-signs.pdf
Treatment of Peyronie's Disease at Mayo Clinic in Rochester. Mayo Clinic. nd. 2pp. www.mayoclinic.org/peyronies-rst/
Peyronie's Disease. University of Maryland Medicine. Last reviewed on May 16,2003. 2pp. www.umm.edu/urology-info/peyroni.htm
American Urological Association Foundation 1000 Corporate Blvd. Linthicum, MD 21090 USA Tel: (410)689-3700 Fax: (410)689-3800 Tel: (866)746-4282 Email: auafoundation@auafoundation.org Internet: http://www.auafoundation.org
NIH/National Kidney and Urologic Diseases Information Clearinghouse 3 Information Way Bethesda, MD 20892-3580 Tel: (800)891-5390 Email: nkudic@info.niddk.nih.gov Internet: http://kidney.niddk.nih.gov/
Association of Peyronie's Disease Advocates (APDA) PO Box 62865 Colorado Springs, CO 80962-2865 USA Email: info@peyroniesassociation.org Internet: http://www.peyroniesassociation.org
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