Peyronie's Disease
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Peyronie's disease (PD) is a benign, acquired penile
connective tissue disease Connective tissue diseases (also termed connective tissue disorders, or collagen vascular diseases), are medical conditions that affect connective tissue. Connective tissues protect, support, and provide structure for the body's other tissues a ...
characterized by the occurrence of fibrotic plaques within the tunica albuginea — the dense elastic covering of the corpora cavernosa. The plaques cause abnormal curvature,
pain Pain is a distressing feeling often caused by intense or damaging Stimulus (physiology), stimuli. The International Association for the Study of Pain defines pain as "an unpleasant sense, sensory and emotional experience associated with, or res ...
, penile deformities (e.g., narrowing or indentation), and usually
erectile dysfunction Erectile dysfunction (ED), also referred to as impotence, is a form of sexual dysfunction in males characterized by the persistent or recurring inability to achieve or maintain a Human penis, penile erection with sufficient rigidity and durat ...
, particularly during
erection An erection (clinically: penile erection or penile tumescence) is a Physiology, physiological phenomenon in which the penis becomes firm, engorged, and enlarged. Penile erection is the result of a complex interaction of psychological, neural, ...
. The condition typically leads to significant sexual and psychological effects, including difficulty with penetration and lowered self-esteem or evasiveness. Peyronie's disease is most often seen in middle-aged and older men with a median age of onset between 55 and 60 years, although it has also been noted in younger individuals and adolescents. While the
etiology Etiology (; alternatively spelled aetiology or ætiology) is the study of causation or origination. The word is derived from the Greek word ''()'', meaning "giving a reason for" (). More completely, etiology is the study of the causes, origins ...
of PD is still a mystery, the leading hypothesis is that it arises from dysregulated wound healing in response to chronic microtrauma of the erect penis. This triggers a cascade of profibrotic molecular pathways — most notably overexpression of transforming growth factor-beta 1 (TGF-β1) — that end in
fibroblast A fibroblast is a type of cell (biology), biological cell typically with a spindle shape that synthesizes the extracellular matrix and collagen, produces the structural framework (Stroma (tissue), stroma) for animal Tissue (biology), tissues, and ...
proliferation,
myofibroblast A myofibroblast is a cell phenotype that was first described as being in a state between a fibroblast and a smooth muscle cell. Structure Myofibroblasts are contractile web-like fusiform cells that are identifiable by their expression of α-s ...
differentiation, and overproduction of
type I collagen Type I collagen is the most abundant collagen of the human body, consisting of around 90% of the body's total collagen in vertebrates. Due to this, it is also the most abundant protein type found in all vertebrates. Type I forms large, eosinop ...
. Genetic predisposition is supported by family clustering and linkage with systemic fibrosing disorders such as Dupuytren's contracture. Risk factors include age, penile injury,
diabetes mellitus Diabetes mellitus, commonly known as diabetes, is a group of common endocrine diseases characterized by sustained hyperglycemia, high blood sugar levels. Diabetes is due to either the pancreas not producing enough of the hormone insulin, or th ...
, and
cigarette smoking Tobacco smoking is the practice of burning tobacco and ingesting the resulting smoke. The smoke may be inhaled, as is done with cigarettes, or released from the mouth, as is generally done with pipes and cigars. The practice is believed to hav ...
. The prevalence of PD has been projected at 3% to 9% among the general population of men increasing with age and comorbidities such as
erectile dysfunction Erectile dysfunction (ED), also referred to as impotence, is a form of sexual dysfunction in males characterized by the persistent or recurring inability to achieve or maintain a Human penis, penile erection with sufficient rigidity and durat ...
or
connective tissue disease Connective tissue diseases (also termed connective tissue disorders, or collagen vascular diseases), are medical conditions that affect connective tissue. Connective tissues protect, support, and provide structure for the body's other tissues a ...
. While PD is neither infectious nor malignant, it can have disastrous implications on
sexual health Sexual and reproductive health (SRH) is a field of research, health care, and social activism that explores the health of an individual's reproductive system and sexual well-being during all stages of their life. Sexual and reproductive healt ...
and quality of life. It is diagnosed mainly on the clinical presentation supplemented by penile ultrasonography if necessary. Treatment depends on the phase and severity of the disease with conservative measures (e.g., oral therapy, traction, intralesional injection) in the milder and stable forms to surgical intervention for the advanced or stable ones. The condition is named for French surgeon François Gigot de la Peyronie, who in 1743 described the condition. It is estimated to affect 1–20% of men. The condition becomes more common with age.


Signs and symptoms

The most frequent sign of Peyronie's disease is penile curvature acquired on erection. The location and extent of curvature vary with the location and extent of the fibrous plaque of the tunica albuginea. Curvature is the most frequent complaint, but some men complain of penile shortening, penile narrowing (hourglass deformity), or indentations. Palpable plaques can be seen along the shaft, most commonly on the dorsum. Pain with erection is common during the initial, inflammatory phase but usually resolves during the chronic phase with resolution of swelling. Erectile dysfunction (ED) is estimated to affect 30–70% of men with PD, either due to decreased rigidity secondary to anatomic changes or psychogenic causes like distress and performance concern. PD can substantially impair sexual function and quality of life, leading to depression, relationship difficulties, and low
self-esteem Self-esteem is confidence in one's own worth, abilities, or morals. Self-esteem encompasses beliefs about oneself (for example, "I am loved", "I am worthy") as well as emotional states, such as triumph, despair, pride, and shame. Smith and Macki ...
.


Psychosocial

Peyronie's disease can also have psychological effects. While most men will continue to be able to have sexual relations, they are likely to experience some degree of
erectile dysfunction Erectile dysfunction (ED), also referred to as impotence, is a form of sexual dysfunction in males characterized by the persistent or recurring inability to achieve or maintain a Human penis, penile erection with sufficient rigidity and durat ...
. It is not uncommon to exhibit depression or withdrawal from their sexual partners.


Causes and Risk Factors

The
etiology Etiology (; alternatively spelled aetiology or ætiology) is the study of causation or origination. The word is derived from the Greek word ''()'', meaning "giving a reason for" (). More completely, etiology is the study of the causes, origins ...
of Peyronie's disease is multifactorial. The most widely held hypothesis is that recurrent microtrauma to the erect penis (e.g., during intercourse) leads to localized
inflammation Inflammation (from ) is part of the biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. The five cardinal signs are heat, pain, redness, swelling, and loss of function (Latin ''calor'', '' ...
and abnormal
wound healing Wound healing refers to a living organism's replacement of destroyed or damaged tissue by newly produced tissue. In undamaged skin, the epidermis (surface, epithelial layer) and dermis (deeper, connective layer) form a protective barrier again ...
in genetically predisposed men. This pathway initiates a cascade of pro-inflammatory and pro-fibrotic
cytokines Cytokines () are a broad and loose category of small proteins (~5–25 kDa) important in cell signaling. Cytokines are produced by a broad range of cells, including immune cells like macrophages, B cell, B lymphocytes, T cell, T lymphocytes ...
such as transforming growth factor beta 1 (TGF-β1), resulting in abnormal collagen deposition and plaque formation. Risk factors include advancing age, penile trauma, diabetes mellitus, Dupuytren's contracture, and tobacco smoking. Familial aggregation and linkage with other fibrosing disorders suggest a genetic basis. PD is seen most frequently in men over 40 years of age, with a prevalence in the general male population estimated at between 3% and 9%, and with higher prevalence in those with erectile dysfunction.


Pathophysiology

Peyronie's disease develops in two phases: the acute (inflammatory) and chronic (fibrotic) phase. During the acute phase, microtrauma induces damage to the
endothelium The endothelium (: endothelia) is a single layer of squamous endothelial cells that line the interior surface of blood vessels and lymphatic vessels. The endothelium forms an interface between circulating blood or lymph in the lumen and the r ...
, fibrin deposition, and immigration of
immune cells White blood cells (scientific name leukocytes), also called immune cells or immunocytes, are cells of the immune system that are involved in protecting the body against both infectious disease and foreign entities. White blood cells are genera ...
into the tunica albuginea.
Fibroblast A fibroblast is a type of cell (biology), biological cell typically with a spindle shape that synthesizes the extracellular matrix and collagen, produces the structural framework (Stroma (tissue), stroma) for animal Tissue (biology), tissues, and ...
proliferation and
myofibroblast A myofibroblast is a cell phenotype that was first described as being in a state between a fibroblast and a smooth muscle cell. Structure Myofibroblasts are contractile web-like fusiform cells that are identifiable by their expression of α-s ...
differentiation are promoted by
cytokines Cytokines () are a broad and loose category of small proteins (~5–25 kDa) important in cell signaling. Cytokines are produced by a broad range of cells, including immune cells like macrophages, B cell, B lymphocytes, T cell, T lymphocytes ...
like TGF-β1, PDGF (
platelet-derived growth factor Platelet-derived growth factor (PDGF) is one among numerous growth factors that regulate cell growth and division. In particular, PDGF plays a significant role in blood vessel formation, the growth of blood vessels from already-existing bloo ...
), and ROS (
reactive oxygen species In chemistry and biology, reactive oxygen species (ROS) are highly Reactivity (chemistry), reactive chemicals formed from diatomic oxygen (), water, and hydrogen peroxide. Some prominent ROS are hydroperoxide (H2O2), superoxide (O2−), hydroxyl ...
). Myofibroblasts overexpress
type I collagen Type I collagen is the most abundant collagen of the human body, consisting of around 90% of the body's total collagen in vertebrates. Due to this, it is also the most abundant protein type found in all vertebrates. Type I forms large, eosinop ...
and
extracellular matrix proteins In biology, the extracellular matrix (ECM), also called intercellular matrix (ICM), is a network consisting of extracellular macromolecules and minerals, such as collagen, enzymes, glycoproteins and hydroxyapatite that provide structural and bi ...
, leading to plaque formation. In the chronic presentation, inflammation is reduced, but the fibrotic plaque is preserved, and partial
calcification Calcification is the accumulation of calcium salts in a body tissue. It normally occurs in the formation of bone, but calcium can be deposited abnormally in soft tissue,Miller, J. D. Cardiovascular calcification: Orbicular origins. ''Nature M ...
occurs in most cases. This causes structural deformity of the penile tissue and inelasticity that impedes normal growth during an erection. Intense
calcification Calcification is the accumulation of calcium salts in a body tissue. It normally occurs in the formation of bone, but calcium can be deposited abnormally in soft tissue,Miller, J. D. Cardiovascular calcification: Orbicular origins. ''Nature M ...
and
fibrosis Fibrosis, also known as fibrotic scarring, is the development of fibrous connective tissue in response to an injury. Fibrosis can be a normal connective tissue deposition or excessive tissue deposition caused by a disease. Repeated injuries, ch ...
impair normal
hemodynamics Hemodynamics or haemodynamics are the dynamics of blood flow. The circulatory system is controlled by homeostatic mechanisms of autoregulation, just as hydraulic circuits are controlled by control systems. The hemodynamic response continuously ...
and penile elasticity of the penis, which usually causes venous leakage and erectile failure.


Diagnosis

Peyronie's disease
diagnosis Diagnosis (: diagnoses) is the identification of the nature and cause of a certain phenomenon. Diagnosis is used in a lot of different academic discipline, disciplines, with variations in the use of logic, analytics, and experience, to determine " ...
is mostly clinical.
Patient history The medical history, case history, or anamnesis (from Greek: ἀνά, ''aná'', "open", and μνήσις, ''mnesis'', "memory") of a patient is a set of information the physicians collect over medical interviews. It involves the patient, and even ...
and
physical examination In a physical examination, medical examination, clinical examination, or medical checkup, a medical practitioner examines a patient for any possible medical signs or symptoms of a Disease, medical condition. It generally consists of a series of ...
are crucial. The most prominent features are acquired penile curvature, palpable plaques, erectile dysfunction, and pain during erections. Penile ultrasonography is the imaging method of choice for plaque location, measurement, and confirmation of
calcification Calcification is the accumulation of calcium salts in a body tissue. It normally occurs in the formation of bone, but calcium can be deposited abnormally in soft tissue,Miller, J. D. Cardiovascular calcification: Orbicular origins. ''Nature M ...
.
Doppler ultrasound Doppler ultrasonography is medical ultrasonography that employs the Doppler effect to perform imaging of the movement of tissues and body fluids (usually blood), and their relative velocity to the probe. By calculating the frequency shift of a ...
can be utilized to assess vascular function, which is useful in the evaluation of associated
erectile dysfunction Erectile dysfunction (ED), also referred to as impotence, is a form of sexual dysfunction in males characterized by the persistent or recurring inability to achieve or maintain a Human penis, penile erection with sufficient rigidity and durat ...
. Imaging also helps to differentiate PD from congenital penile curvature, penile fracture, or neoplastic disease.
MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and rad ...
can be employed in severe ones, although it is generally not required.


Ultrasonography

On penile ultrasonography, the typical appearance is hyperechoic focal thickening of the tunica albuginea. Due to associated calcifications, the imaging of patients with Peyronie's disease shows acoustic shadowing, as illustrated in figures below. Less common findings, attributed to earlier stages of the disease (still mild fibrosis), are hypoechoic lesions with focal thickening of the paracavernous tissues, echoic focal thickening of the tunica without posterior acoustic shadowing, retractile isoechoic lesions with posterior attenuation of the beam, and focal loss of the continuity of the tunica albuginea. In the Doppler study, increased flow around the plaques can suggest inflammatory activity and the absence of flow can suggest disease stability. Ultrasound is useful for the identification of lesions and to determine their relationship with the neurovascular bundle. Individuals with Peyronie's disease can present with erectile dysfunction, often related to venous leakage, due to insufficient drainage at the site of the plaque. Although plaques are more common on the dorsum of the penis, they can also be seen on the ventral face, lateral face, or septum.Originally copied from:

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File:Ultrasonography of orpus cavernosum calcification.jpg, Transverse ultrasound of the penis, in a ventral view, in the middle portion of the penis. Note the echoic image with posterior acoustic shadowing, corresponding to calcification (arrow), in the left corpus cavernosum.


Management and Treatment

Peyronie's disease treatment is based on stage of disease, degree of curvature, loss of function, and patient choice. Conservative management in the acute phase has the aim of reducing
inflammation Inflammation (from ) is part of the biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. The five cardinal signs are heat, pain, redness, swelling, and loss of function (Latin ''calor'', '' ...
and disease extension. Treatment can be with tablets such as pentoxifylline (an anti-fibrotic phosphodiesterase inhibitor), acetyl-L-carnitine, and
vitamin E Vitamin E is a group of eight compounds related in molecular structure that includes four tocopherols and four tocotrienols. The tocopherols function as fat-soluble antioxidants which may help protect cell membranes from reactive oxygen speci ...
. Mechanical treatments in the form of penile traction therapy (PTT) and vacuum erection devices (VED) are used to correct the curvature due to plaque and preserve length. Intralesional injection therapy is the mainstay of non-surgical therapy, particularly for stable disease.
Verapamil Verapamil, sold under various trade names, is a calcium channel blocker medication used for the treatment of high blood pressure, angina (chest pain from not enough blood flow to the heart), and supraventricular tachycardia. It may also be use ...
and interferon-α2b have intermediate efficacy. Collagenase Clostridium histolyticum (CCH), an enzymatic drug approved by the US FDA, targets plaque collagen and repairs in moderate disease. It is administered in a series of treatment cycles with attendant penile modeling and elongation. Surgical therapy is reserved for fixed patients with severe curvature, or failed medical therapy. Operations include penile plication (shortening the concave portion of the penis), plaque incision or excision with grafting (lengthening the convex portion of the penis), and penile prosthesis implantation in concomitant erectile dysfunction. Reasonable levels of satisfaction after surgery are present, especially with appropriate preoperative counseling.


Epidemiology

It is estimated to affect 1–20% of men. The condition becomes more common with age. The median age at onset of disease is 55–60 years although many cases have been recorded in adolescence and early 20's. The overall prevalence of PD is about 1–20% in men. Rates range from 3.2 percent in a community-based survey of 4432 men (mean age of sample 57.4) to 16 percent among 488 men undergoing evaluation for erectile dysfunction (mean age 52.8). The prevalence of PD among the 4432 men in the community based study who responded by self report positively for palpable plaque, newly occurring angulation or curvature and painful erection was 1.5 percent between the ages of 30 and 39, 3 percent between 40 and 49, 3 percent between 50 and 59, 4 percent between 60 and 69, and 6.5 percent over 70. In 534 men undergoing routine prostate screening for cancer detection (without a specific urologic complaint), the prevalence of PD was 8.9 percent. In this study, the mean age of those with PD was 68.2 years compared with 61.8 years of those without PD.


History

The condition was first described in 1561 in correspondence between
Andreas Vesalius Andries van Wezel (31 December 1514 – 15 October 1564), latinized as Andreas Vesalius (), was an anatomist and physician who wrote '' De Humani Corporis Fabrica Libri Septem'' (''On the fabric of the human body'' ''in seven books''), which is ...
and
Gabriele Falloppio Gabriele Falloppio (1522/23 – 9 October 1562) was an Italian priest and anatomist often known by his Latin name Fallopius. He was one of the most important human anatomy, anatomists and physicians of the sixteenth century, giving his name to t ...
and separately by Gabriele Falloppio. The condition is named after François Gigot de la Peyronie, who described it in 1743.


References


External links


Medscape Urology overview of Peyronie's disease
{{DEFAULTSORT:Peyronie's Disease Dermal and subcutaneous growths Diseases named after discoverers Penis disorders Sexual health