Last updated:
May 12, 2015
Years published: 1987, 1989, 1996, 1998, 2000, 2005, 2009, 2012, 2015
NORD gratefully acknowledges Wayne J. Hellstrom, MD, for updating the content of this report and Jessica Daigle 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 plaque(s) within the 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 3-12% of patients.
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 tissue 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 there appears 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.
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 have prescribed intralesional therapy with verapamil and interferon-alpha 2b injections. Phase 3 trials with intralesional collagenase (Xiaflex manufactured by Auxilium Pharmaceuticals) documented a significant decrease compared to placebo in both degree of penile curvature (-34% vs -18.2%, P<0.0001) and Peyronie’s bother-score end points (-2.8 vs –1.8, P=0.003) when combined with a modeling procedure. Xiaflex was approved by the FDA in 2013 to treat men with Peyronie’s disease. For information, contact: https://www.xiaflex.com/
It is well-documented that gradual expansion of tissue results in the formation of new 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 with promising results.
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 length and 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 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 with penile manual modeling or other ancillary procedures 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
Email: [email protected]
For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
Several non-surgical treatments are prescribed to manage Peyronie’s disease. All of them are considered investigational and have shown various degrees of success. Shock wave therapy has been prescribed as well as a range of oral and topical medications.
A calcium channel-blocking drug, known as verapamil, and alpha 2b interferon have been used to treat individuals with Peyronie’s disease. The drugs are 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
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
Gelbard M, Goldstein I, Hellstrom WJ, McMahon CG, Smith T, Tursi J, Jones N, Kaufman GJ, Carson CC III Clinical efficacy, safety and tolerability of collagenase clostridium hystolyticum for the treatment of Peyroni’s disease in 2 large, double-blind, randomized, placebo-controlled phase III studies. J Urol. 2013 Jul;190(1):199-207. doi: 10.1016/j.juro.2013.01.087. Epub 2013 Jan 31.
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 09/22/2009 https://omim.org/entry/171000 Accessed May 11, 2015.
Peyronie’s Disease. National Kidney and Urologic Diseases Information Clearinghouse. last updated July 23, 2014. https://kidney.niddk.nih.gov/kudiseases/pubs/peyronie/index.aspx Accessed May 11, 2015.
Lizza E. Peyronie Disease.Medscape. Updated: Dec 13, 2013. https://emedicine.medscape.com/article/456574-overview Accessed May 11, 2015.
NORD strives to open new assistance programs as funding allows. If we don’t have a program for you now, please continue to check back with us.
NORD and MedicAlert Foundation have teamed up on a new program to provide protection to rare disease patients in emergency situations.
Learn more https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORD’s mission.
Learn more https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/This first-of-its-kind assistance program is designed for caregivers of a child or adult diagnosed with a rare disorder.
Learn more https://rarediseases.org/patient-assistance-programs/caregiver-respite/Please complete this form to access the requested resource.
