Synonyms: penile fibrosis, induratio penis plastica
This a disorder of penile connective tissue, first brought to widespread medical attention by François Gigot de la Peyronie in 1743. Fibrous plaque formation occurs in the corpus cavernosum's tunica albuginea. There is inflammatory thickening with fibrin deposition, increased collagen production, decreased quantity of elastic fibres and subsequent fibrosis ± calcification. This leads to penile angulation or an hourglass-like deformity with distal flaccidity. It usually affects only the erect penis. Sexual intercourse can become painfully difficult or impossible. It is thought to occur (but nobody really knows) as a result of one-off or repeated penile vascular trauma causing leakage and immunological reaction in the relatively avascular tunica albuginea. Genetic susceptibility is thought to play a role.Studies suggest a link with low testosterone levels.A congenital form has also been described.
Exact figures for prevalence and incidence are hard to come by as, due to embarrassment, many men may not seek help. The incidence of the congenital form has been reported variously as <1% and between 4-10% without hypospadias.Based on the current literature, the prevalence seems to be 3-9% with an average age of onset in the fifth life decade.
One study reported a subset of patients presenting in adolescence. These tended to have a higher number of plaques and an increased incidence of raised HbA1c levels.
Purportedly, Dupuytren's contracture, diabetes mellitus, hypertension, lipid abnormalities, ischaemic cardiopathy, erectile dysfunction, smoking and excessive consumption of alcohol. However, the pathophysiology remains unclear.
When Peyronie's disease (PD) first presents, it tends to be with penile pain during erections, penile angulation (this can be seen in some cases in a flaccid penis), palpable fibrous plaque at the site of angulation and erectile dysfunction. This inflammatory phase settles in 18-24 months, to be followed by a fibrotic phase characterised by plaque formation, angulation and calcification. One study, using a validated mental health questionnaire, has shown that 48% of men with PD have mild or moderate depression.
By Peyronie, via Wikimedia Commons
Physical examination should include measuring the penis dorsally from base to the tip of the glans. Plaque size and angulation should be measured while the penis is erect. Angulation can be measured by means of a photograph taken at home, a vacuum pump or by intra-cavernosal injection of a vasoactive agent. Changes in girth are often self-reported by the patient. The hands should be examined for evidence of Dupuytren's contracture.
There are validated assessment tools to measure erectile dysfunction, such as the International Index of Erectile Function (IIEF).
- Prevailing guidelines do not recommend ultrasonography in routine clinical practice, as this has been found to be inaccurate and operator-dependent.
- Duplex ultrasonography is occasionally used to detect associated vascular abnormalities.
Studies of the natural history of the disease suggest that it is a self-limiting condition which goes through an active, scarring phase followed by a mature quiescent phase. This makes the interpretation of pharmaceutical trials difficult to interpret. Most pharmacotherapy reduces scarring and is therefore most effective during the active phase. Before embarking on intra-lesional or surgical approaches, several months should therefore be allowed to pass to allow resolution to occur. The role of conservative therapy is controversial and evidence-based guidelines are needed. Whilst there is no gold standard approach, many professionals in the field find that a combined approach, using oral therapy, intralesional treatments and minimally invasive surgical techniques is the best approach for patients with mild-to-moderate symptoms requiring treatment.
Psychosexual difficulties are an unsurprising byproduct of the condition and referral to a psychologist/counsellor/psychiatrist skilled in this area may significantly reduce the burden of the disease on the patient.
- External penile traction is a new technique that is currently being evaluated. Initial data seem promising in terms of improving penile length and reducing deformity.
- Vacuum devices act in a similar manner.
- Oral: drugs which have anecdotally been tried for this condition include para-aminobenzoate, tocopherol (vitamin E), colchicine, tamoxifen, propoleum, acetyl-L-carnitine and pentoxifylline. Few have shown any consistent effect in placebo-controlled trials and prevailing European guidelines no longer recommend the use of vitamin E, tamoxifen, acetyl-L-carnitine or pentoxifylline. Para-aminobenzoate may decrease plaque size and curvature and may reduce erectile pain and inhibit disease progression. Only para-aminobenzoate is licensed in the UK for the treatment of PD.
- Topical: verapamil has been reported to have some success in improving penile curvature and plaque size after prolonged use (nine months) but evidence that it penetrates into the deeper layers of the tunica albuginea is lacking.
- Intralesional: several agents have been tried, including verapamil, interferon and clostridial collagenase. None has a particularly strong evidence base but studies have reported some improvement in such parameters as pain, plaque size and angle of deviation. In the USA, the FDA approved clostridial collagenase as a treatment in 2013. Steroids are now thought best avoided, as there is evidence that they can increase inflammation.
- Iontophoresis - electromotive drug administration (EMDA): various researchers have used electrical current (up to 5 mA) to deliver charged drug molecules to the affected area - usually a combination of dexamethasone, verapamil and/or orgotein and lidocaine. The evidence base supporting the effectiveness of this method is equivocal. There appear to be no significant adverse effects apart from mild, local erythema.
Careful selection, patient education and discussion of patient expectation help to improve satisfaction post-surgery. Issues to be discussed should include the aims and risks of surgery, the risks of penile shortening, the risk of recurrent curvature, the possibility of knots and stitches being palpable beneath the skin and the possibility that circumcision may need to be performed at the time of surgery (to prevent postoperative phimosis). Surgery should not be attempted until the disease has been stable for at least three months (some authorities recommend this period should be 6-12 months). Various techniques are available:
- Extracorporeal shock wave therapy: the National Institute for Health and Care Excellence (NICE) has produced guidance for professionals and public on the use of this technique in this context. Essentially, whilst there are no significant safety concerns, NICE is not convinced of any proof of efficacy for the procedure; it advises it be used in carefully controlled, well-audited programmes or as part of a research trial, with detailed explanation given to patients during the consenting process. The guidance was published in 2003 but remained current in 2015.
- 'Cold steel' surgery: this surgery should be reserved for patients with significant morbidity who fail to respond to medical therapy. It should be deferred until 12-18 months, after which time changes to plaques and angulation are unlikely. Surgery is the only evidence-based treatment that has shown any effectiveness in the congenital form: it can be performed at any time in adulthood. Options include:
- The Nesbit tuck procedure: normal tunica albuginea is removed from the side of the penile shaft opposite the plaque to straighten and shorten the penis. Potency should be normal and the penile curvature should be less than 60°.
- Tunica plication procedure: this involved plication rather than excision of the unaffected tunica albuginea to straighten the penis. This technique also causes penile shortening.
- Plaque excision and grafting: this is performed to preserve penile length when the curvature is greater than 60°.
- Plaque excision and penile prosthesis insertion: this is useful when severe erectile dysfunction is also a problem.
- One study described a new technique using a new lengthening surgical procedure based on a ventro-dorsal incision of the tunica albuginea, penile prosthesis implantation and double dorsal-ventral patch grafting with porcine small intestinal submucosa.
- Carbon dioxide laser: this has been used to good effect in some cases to thin the plaque.
PD rarely resolves completely. Studies suggest that without treatment, 13% of patients have a diminution or complete resolution of pain with time. One half of the remainder has progressive disease; the other half has static disease. Persisting symptoms may vary from static painless plaque to painful erections with curvature significant enough to prevent intercourse. Because evidence of the effectiveness of treatments from large-scale trials is lacking, the effect of the various therapies on prognosis is unknown.
Further reading and references
Dolmans GH, Werker PM, de Jong IJ, et al; WNT2 Locus Is Involved in Genetic Susceptibility of Peyronie's Disease. J Sex Med. 2012 May9(5):1430-1434. doi: 10.1111/j.1743-6109.2012.02704.x. Epub 2012 Apr 10.
Cavallini G, Biagiotti G, Lo Giudice C; Association between peyronie disease and low serum testosterone levels: detection and therapeutic considerations. J Androl. 2012 May-Jun33(3):381-8. Epub 2011 Jun 30.
Guidelines on Penile Curvature; European Association of Urology (2015)
Hellstrom WJ; Medical management of Peyronie's disease. J Androl. 2009 Jul-Aug30(4):397-405. Epub 2008 Oct 30.
Tal R, Hall MS, Alex B, et al; Peyronie's disease in teenagers. J Sex Med. 2012 Jan9(1):302-8. doi: 10.1111/j.1743-6109.2011.02502.x. Epub 2011 Oct 7.
Chung E, Yan H, De Young L, et al; Penile Doppler sonographic and clinical characteristics in Peyronie's disease and/or erectile dysfunction: an analysis of 1500 men with male sexual dysfunction. BJU Int. 2012 Feb 7. doi: 10.1111/j.1464-410X.2011.10851.x.
Kuehhas FE, Weibl P, Georgi T, et al; Peyronie's Disease: Nonsurgical Therapy Options Rev Urol. 2011
Extra-corporeal shock wave therapy for Peyronie's disease; NICE Interventional Procedure Guidance, December 2003
Rolle L, Ceruti C, Timpano M, et al; A New, Innovative, Lengthening Surgical Procedure for Peyronie's Disease by Penile Prosthesis Implantation with Double Dorsal-Ventral Patch Graft: The "Sliding Technique" J Sex Med. 2012 Mar 16. doi: 10.1111/j.1743-6109.2012.02675.x.
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