Peyronie Disease

Basics

Description

Acquired disorder characterized by fibrosis and plaque formation of the tunica albuginea leading to curvature shortening, penile deformity, and painful erections (1,2)[C].
Named after 18th-century French physician Francois de la Peyronie (1)[C]
The most common plaque location is on the dorsal aspect of the penis followed by lateral then ventral (1)[C].
Curvature can range from mild to severe and may preclude sexual intercourse and cause psychological distress.
Synonyms: acquired penile curvature, penile induration

Epidemiology

Incidence

Estimated incidence of 2 " 8% of men of all ages (1)[C]
Up to 9% men >40 years old (2)[C]
Mean age of diagnosis: 53 years in White males (1)[C]

Prevalence
2 " 9% (1)[C]

Etiology and Pathophysiology

Traumatic (1)[C]
Theory: Microtrauma in men with genetic predisposition leads to inflammation of the tunica albuginea then collagen deposition and eventually fibrosis in the form of a plaque (1)[C].
Acute phase
- 6 " 18 months of acute inflammation eventually leading to fibrosis and plaque formation
- Painful erections that resolve spontaneously
- Angulation in flux: 12% improve, 40% remain stable, 48% worsen (2)[C],(3)[B]
Chronic phase
- Stable plaque formation and angulation (1)[C]
- Pain resolves

Risk Factors

Genetic predisposition/HLA-B7
Trauma (1)[C]

Commonly Associated Conditions

Dupuytren contracture
Erectile dysfunction (ED)

Diagnosis

History

History of trauma
Duration and onset of symptoms
Pain with erections and intercourse
Degree of curvature
Ability to penetrate
ED
Bother
- Peyronie 's Disease Questionnaire (PDQ)

Physical Exam

Genitourinary (GU) exam focusing on penile plaque size and location
Photograph or artificial erection to assess degree of curvature
Examine hands for Dupuytren contracture.

Differential Diagnosis

Penile fracture
Cancer (rare)

Diagnostic Tests & Interpretation

Initial Tests (lab, imaging)

No tests necessary for diagnosis if medical management is preferred.
Preoperative assessment if surgery planned.
- Penile ultrasound (US) with Doppler to assess vascular status for ED

Follow-up tests & special considerations
In office, artificial erection by cavernosal injection to quantify degree of curvature if planning surgery
Test Interpretation
Validated questionnaire

PDQ will assess degree of bother

Treatment

General Measures

There are a variety of medical and surgical options for treatment. The paucity of randomized controlled trials (RCTs) makes drawing conclusions difficult (1,2)[C].
Theoretically, medical management will be more helpful for patients in the acute phase. Surgical treatment should be reserved for patients in the chronic phase when the fibrosis has stabilized and if the deformity significantly limits or bothers sexual intercourse (1)[C].

Medication

Oral therapy
- Colchicine (1,2)[C]
  - Inhibits collagen deposition, activates collagenase, and decreases inflammation
  - 0.6 mg PO t.i.d.
  - Side effects: GI upset, weakness, rare agranulocytosis
  - May be some benefit but larger RCTs needed (2)[C]
- Pentoxifylline
  - 400 mg PO t.i.d.
  - Noncontrolled, nonrandomized data support its use.
  - Large RCTs needed (2)[C]
- Vitamin E (1,2)[C]
  - Antioxidant effects
  - 800 " 1,200 IU divided doses for 3 " 6 months
  - Side effects: GI upset, bleeding, cardiovascular events
  - No evidence to support benefit
  - One small study found vitamin E and colchicine in combination is beneficial for mild curvature.
- Potaba (1,2)[C]
  - No evidence, not recommended(1,2)[C]
  - 3 mg PO q6h (24 tablets daily)
  - Used to treat Dupuytren contracture
  - Side effects: GI upset, hypoglycemia
  - Not well tolerated
Intralesional injection therapy
- Localized therapy minimizes systemic side effects while delivering drug directly to plaque.
- Verapamil (1,2)[C]
  - Plaque remodeling: Calcium blockage prevents collagen transport.
  - 6 " 12 injections (10 mg/10 mL) given once every 2 " 4 weeks
  - Nonrandomized, noncontrolled data shows reasonable efficacy with minimal side effects (1,2)[C].
- Interferon-α (1,2)[C]
  - Decrease collagen production
  - 0.5 million units biweekly for 6 weeks
  - Data is mixed but may provide some benefit (2)[C].
  - Significant flulike symptoms
- Steroids (1,2)[C]
  - No evidence to support its use (1,2)[C].
  - Local side effects include pain and bruising.
- Purified clostridial collagenase (Xiaflex)
  - Enzyme degrades collagen.
  - Only FDA-approved treatment
  - Four treatment cycles each consisting of two injections of 10,000 units 2 " 3 days apart and "penile modeling "
  - Side effects: ecchymosis, hematoma, pain, and rarely corporal rupture (4)[A]

Additional Therapies

Extracorporeal shock wave lithotripsy (ESWL): No data support a benefit (2)[C].
Topical verapamil: No data support its use (1,2)[C].
Penile traction: data lacking (2)[C]
Radiation: should be avoided (1,2)[C]

Surgery/Other Procedures

Indicated in patients with stable disease and a degree of curvature that significantly limits or bothers sexual function
Artificial erections to assess degree of curvature for operative planning
Preoperative penile US with Doppler to assess for vascular status of the penis
Plication (1)[C]
- Candidates: mild to moderate curvature
- Causes penile shortening but not likely to affect erectile function
- Complication: shortening, incomplete straightening
Plaque incision/excision with grafting (1)[C]
- Candidates: moderate to severe curvature with good erectile function
- Graft material: dermis, saphenous vein, tunica vaginalis, cadaveric pericardium, small intestine submucosa, or synthetic materials (Gore-Tex, Dacron)
- Complications: higher rates of ED, loss of sensitivity, infection
Inflatable penile prosthesis (1)[C]
- Candidates: curvature with significant ED
- Modeling: forced manual manipulation of penis over prosthesis
- Complications: infection, erosion, mechanical malfunction

Complementary & Alternative Therapies

See oral agents.

Ongoing Care

Follow-up Recommendations

Patients should be monitored regularly to assess response to therapy.
Patient Monitoring
Significant psychological stress and bother can be present in the male and his partner (1)[C].

Patient Education

Counseling and support groups can be offered.

Prognosis

See "Etiology and Pathophysiology " for the natural history of the disease.
With good urologic management, couples should be able to resume sexually satisfying relationships with local therapy or surgery.

Complications

See individual treatments for details.

References

1.Jordan GH. Peyronie 's disease. In: Wein AJ, Louis R, eds. Campbell-Walsh Urology. 10th ed. Philadelphia, PA: Saunders-Elsevier; 2012.2.Chiles KA, Mulhall JP. Medical Management of Peyonie 's Disease AUA Update Series 2014 Vol 33.3.Berookhim BM, Choi J, Alex B, et al. Deformity stabilization and improvement in men with untreated Peyronie 's disease. BJU Int. 2014;113(1):133 " 136.
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4.Gelbard M, Goldstein I, Hellstrom JW, et al. Clinical efficacy, safety and tolerability of collagenase clostridium histolyticum for the treatment of peyronie disease in 2 large double-blind, randomized, placebo controlled phase 3 studies. J Urol. 2013;190(1):199 " 207.
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Codes

ICD09

607.85 Peyronies disease

ICD10

N48.6 Induration penis plastica

SNOMED

1335005 Induratio penis plastica (disorder)

Clinical Pearls

Peyronie disease (PD) is characterized clinically by penile pain and curvature caused by fibrotic plaque in the tunica albuginea.
An acute active phase of inflammation is followed by a stable chronic phase.
Most of the medical treatments have not been adequately studied with RCTs but are assumed to be more effective early in the disease course.
FDA has recently approved collagenase injections.
Surgical treatment is reserved for severe cases where sexual intercourse is precluded.
PD can be a psychologically distressing disease for the patient and his partner and care should be taken when counseling these patients.

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