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Erythroplasia of Queyrat is a squamous-cell carcinoma of the glans penis (head of the penis) or inner prepuce (foreskin) in males, and the vulvae in females. It mainly occurs in uncircumcised males, over the age of 40. Erythroplasia of Queyrat may also occur on the anal mucosa or the oral mucosa.
Some sources state that this condition is synonymous with Bowen's disease, however generally speaking Bowen's disease refers to carcinoma in situ of any location on the skin such as the lower leg. Like Bowen's disease, erythroplasia of Queyrat is associated with human papillomavirus 16 and is a precursor for invasive squamous-cell carcinoma.
It is named for French dermatologist Louis Queyrat (1856-1933), who was head of the dermatology service of l'Hôpital Ricord, a venereal hospital in Paris, now Hôpital Cochin.
Although often the terms "erythroplasia" and "erythroplakia" are used synonymously, some sources distinguish them, stating that the latter is maccular (flat) while the former is papular (bumpy).
Erythroplakia of the genital mucosae is often referred to as erythroplasia of Queyrat.
The most common areas in the mouth where erythroplakia is found are the floor of the mouth, buccal vestibule, the tongue, and the soft palate. It appears as a red macule or plaque with well-demarcated borders. The texture is characterized as soft and velvety. An adjacent area of leukoplakia may be found along with the erythroplakia.
Erythroplasia may also occur on the laryngeal mucosa, or the anal mucosa.
Symptoms can include:
- First signs – small red erosions on the glans
- Redness of the foreskin
- Redness of the penis
- Other rashes on the head of the penis
- Foul smelling discharge
- Painful foreskin and penis
Erythroplakia (or erythroplasia) is a clinical term to describe any erythematous (red) area on a mucous membrane, that cannot be attributed to any other pathology.
The term erythroplasia was coined by Louis Queyrat to describe a precancerous red lesion of the penis. This gave rise to the term erythoplasia of Queyrat. Depending upon the context, this term may refer specifically to carcinoma in situ of the glans penis or vulva appearing as a red patch, or may be used as a synonym of erythroplasia on other mucous membrane or transitional sites.
It mainly affects the glans penis (the head of the penis), although uncommonly it may present on the mucous membranes of the larynx, and rarely, the mouth, or the anus.
Erythroplakia is analogous to the term leukoplakia which describes white patches. Together, these are the 2 traditionally accepted types of premalignant lesion in the mouth, When a lesion contains both red and white areas, the term "speckled leukoplakia" or "eyrthroleukoplakia" is used.
Although erythroplakia is much less common than leukoplakia, erythroplakia carries a significantly higher risk of containing dysplasia or carcinoma in situ, and of eventually transforming into invasive squamous cell carcinoma (a type of oral cancer).
The term Vulvar intraepithelial neoplasia (VIN) refers to particular changes that can occur in the skin that covers the vulva. VIN is not cancer, and in some women it disappears without treatment. If the changes become more severe, there is a chance that cancer might develop after many years, and so it is referred to as a precancerous condition.
Recurrent bouts of balanitis may cause scarring of the preputial orifice; the reduced elasticity may lead to pathologic phimosis.
Medically speaking, the term denotes a squamous intraepithelial lesion of the vulva that shows dysplasia with varying degrees of atypia. The epithelial basement membrane is intact and the lesion is thus not invasive but has invasive potential.
The terminology of VIN evolved over several decades. In 1989 the Committee on Terminology, International Society for the Study of Vulvar Disease (ISSVD) replaced older terminology such as vulvar , Bowen's disease, and Kraurosis vulvae by a new classification system for "Epithelial Vulvar Disease":
- Nonneoplastic epithelial disorders of vulva and mucosa:
- Lichen sclerosus
- Squamous hyperplasia
- Other dermatoses
- Mixed neoplastic and nonneoplastic disorders
- Intraepithelial neoplasia
- Squamous vulvar intraepithelial neoplasia (VIN)
- VIN I, mildest form
- VIN II, intermediate
- VIN III, most severe form including carcinoma in situ of the vulva
- Non-squamous intraepithelial neoplasia
- Extramammary Paget's disease
- Tumors of melanocytes, noninvasive
- Invasive disease (vulvar carcinoma)
The ISSVD further revised this classification in 2004, replacing the three-grade system with a single-grade system in which only the high-grade disease is classified as VIN.
VIN is subdivided into: (Robbins Pathological Basis of Disease, 9th Ed)
Classic vulvular intraepithelial neoplasia: associated with developing into the warty and basaloid type carcinoma. This is associated with carcinogenic genotypes of HPV and/or HPV persistence factors such as cigarette smoking or immunocompromised states.
Differentiated vulvar intraepithelial neoplasia also known as VIN Simplex: is associated with vulvar dermatoses such as lichen sclerosus. It is associated with atypia of the squamous epithelium.
LS can occur without symptoms. White patches on the LS body area, itching, pain, pain during sex (in genital LS), easier bruising, cracking, tearing and peeling, and hyperkeratosis are common symptoms in both men and women. In women, the condition most commonly occurs on the vulva and around the anus with ivory-white elevations that may be flat and glistening.
In males, the disease may take the form of whitish patches on the foreskin and its narrowing (preputial stenosis), forming an "indurated ring", which can make retraction more difficult or impossible. In addition there can be lesions, white patches or reddening on the glans. In contrast to women, anal involvement is less frequent. Meatal stenosis, making it more difficult or even impossible to urinate, may also occur.
On the non-genital skin, the disease may manifest as porcelain-white spots with small visible plugs inside the orifices of hair follicles or sweat glands on the surface. Thinning of the skin may also occur.
At birth, the inner layer of the foreskin is sealed to the glans penis. The foreskin is usually non-retractable in early childhood, and can be as late as 18.
Medical associations advise not to retract the foreskin of an infant, in order to prevent scarring. Some argue that non-retractability may "be considered normal for males up to and including adolescence." Hill states that full retractability of the foreskin may not be achieved until late childhood or early adulthood. A Danish survey found that the mean age of first foreskin retraction is 10.4 years.
Rickwood, as well as other authors, has suggested that true phimosis is over-diagnosed due to failure to distinguish between normal developmental non-retractability and a pathological condition. Some authors use the terms "physiologic" and "pathologic" to distinguish between these types of phimosis; others use the term "non-retractile foreskin" to distinguish this developmental condition from pathologic phimosis.
In some cases a cause may not be clear, or it may be difficult to distinguish physiological phimosis from pathological if an infant appears to be in pain with urination or has obvious ballooning of the foreskin with urination or apparent discomfort. However, ballooning does not indicate urinary obstruction.
In women a comparable condition is known as "clitoral phimosis" whereby the clitoral hood cannot be retracted, limiting exposure of the glans clitoridis.
Bowenoid papulosis is a cutaneous condition characterized by the presence of pigmented verrucous papules on the body of the penis. They are associated with human papillomavirus, the causative agent of genital warts. The lesions have a typical dysplastic histology and are generally considered benign, although a small percentage will develop malignant characteristics.
It is considered as a pre-malignant condition. Other terms used to describe the condition are: Erythroplasia of Queyrat, Squamous cell carcinoma in situ and Bowen’s disease. The term "Bowenoid papulosis" was coined in 1977 by Kopf and Bart and is named after dermatologist John Templeton Bowen.
The term “intraepithelial neoplasia” defines a premalignant intraepithelial change.
On the vulva it is termed VIN (vulvar or vulval intraepithelial neoplasia); on the penis, PIN (penile intraepithelial neoplasia); and on or around the anus, AIN (anal intraepithelial neoplasia). The terminology has been very confusing and it is now recommended that the terms Bowen’s disease, erythroplasia of Queyrat, and bowenoid papulosis should not be used for lesions in the anogenital area. However, dermatologists still recognize a distinct clinical variant, bowenoid papulosis, characterized by discrete papules in a younger age group and a tendency for spontaneous regression. Additionally, some authorities believe that erythroplasia of Queyrat and Bowen’s disease remain useful terms in men.
Webbed penis, also called penis palmatus or penoscrotal fusion, is an acquired or congenital condition in which the scrotal skin extends onto the ventral penile shaft. The penile shaft is buried in scrotum or tethered to the scrotal midline by a fold or web of skin. The urethra and erectile bodies are usually normal. Webbed penis is usually asymptomatic, but the cosmetic appearance is often unacceptable. This condition may be corrected by surgical techniques.
In the congenital form, the deformity represents an abnormality of the attachment between the penis and the scrotum; the penis, the urethra, and the remainder of the scrotum typically are normal.
Webbed penis may also be acquired (iatrogenic) after circumcision or other penile surgery, resulting from excessive removal of ventral penile skin; the penis can retract into the scrotum, resulting in secondary phimosis (trapped penis).
One procedure for webbed penis is the insertion of a subcutaneous soft silicone implant under the penile skin. The procedure was developed by urologist James J. Elist.
Phimosis is a condition in which the foreskin of the penis cannot be pulled back past the glans. A balloon-like swelling under the foreskin may occur with urination. In teenagers and adults, it may result in pain during an erection, but is otherwise not painful. Those affected are at greater risk of inflammation of the glans, known as balanitis, and other complications.
In young children, it is normal to not be able to pull back the foreskin. In more than 90% of cases, this inability resolves by the age of seven, and in 99% of cases by age 16. Occasionally, phimosis may be caused by an underlying condition such as scarring due to balanitis or balanitis xerotica obliterans. This can typically be diagnosed by seeing scarring of the opening of the foreskin.
Typically, it resolves without treatment by the age of three. Efforts to pull back the foreskin during the early years of a young male’s life should not be attempted. For those in whom the condition does not improve further time can be given or a steroid cream may be used to attempt to loosen the tight skin. If this method, combined with stretching exercises, is not effective, then other treatments such as circumcision may be recommended. A potential complication of phimosis is paraphimosis, where the tight foreskin becomes trapped behind the glans. The word is from the Greek "phimos" (φῑμός), meaning "muzzle".
Buried penis (also known as hidden penis) is a congenital or acquired condition, in which the penis is partially or completely hidden below the surface of the skin. It can lead to obstruction of urinary stream, poor hygiene, soft tissue infection, phimosis, and inhibition of normal sexual function. Congenital causes include maldevelopment of penile shaft skin, whereas acquired conditions include morbid obesity, overlaying abdominal fat, or penile injury. Adults with a buried penis will either live with their condition or undergo weight-loss programs. However, weight-loss programs are slow and often do not "unbury" the penis; furthermore, poor hygiene from pooling of urine can lead to soft tissue infection. Patients will eventually need definitive reconstructive surgery and more urgent surgery if infection is present. Surgeons who manage this condition are either reconstructive urologic surgeons or plastic surgeons.
Distress due to the discomfort and pain of Lichen Sclerosus is normal, as are concerns with self-esteem and sex. Counseling can help.
According to the National Vulvodynia Association, which also supports women with Lichen Sclerosus, vulvo-vaginal conditions can cause feelings of isolation, hopelessness, low self-image, and much more. Some women are unable to continue working or have sexual relations and may be limited in other physical activities. Depression, anxiety, and even anger are all normal responses to the ongoing pain LS patients suffer from.
On the shaft of the penis, Fordyce spots are more visible when the skin is stretched, and may only be noticeable during an erection.
The spots can also appear on the skin of the scrotum.
Oral Fordyce granules appear as rice-like granules, white or yellow-white in color. They are painless papules (small bumps), about 1–3 mm in greatest dimension. The most common site is along the line between the vermilion border and the oral mucosa of the upper lip, or on the buccal mucosa (inside the cheeks) in the commissural region, often bilaterally. They may also occur on the mandibular retromolar pad and tonsillar areas, but any oral surface may be involved. There is no surrounding mucosal change. Some patients will have hundreds of granules while most have only one or two.
Occasionally, several adjacent glands will coalesce into a larger cauliflower-like cluster similar to sebaceous hyperplasia of the skin. In such an instance, it may be difficult to determine whether or not to diagnose the lesion as sebaceous hyperplasia or sebaceous adenoma. The distinction may be moot because both entities have the same treatment, although the adenoma has a greater growth potential. Sebaceous carcinoma of the oral cavity has been reported, presumably arising from Fordyce granules or hyperplastic foci of sebaceous glands.
In some persons with Fordyce spots, the glands express a thick, chalky discharge when squeezed.
PPSH usually consists of:
- a phallus midway in size between penis and clitoris,
- a chordee tethering it to the perineum,
- a urethral opening usually on the perineum (the hypospadias),
- and an incompletely closed urogenital opening, which resembles a small and shallow vagina.
Testes are often palpable in the scrotum or inguinal canals, and the karyotype is XY. In most cases there are no internal female structures such as a uterus or other Müllerian duct derivatives.
Sebaceous glands are normal structures of the skin but may also be found ectopically in the mouth, where they are referred to as "oral Fordyce granules" or ectopic sebaceous glands.
On the foreskin they are called Tyson's glands, not to be confused with hirsuties coronae glandis.
When they appear on the penis, they are also called "penile sebaceous glands".
When seen as a streak of individual glands along the interface between the skin of the lip and the vermilion border, the terms Fox–Fordyce disease and Fordyce's condition have been used.
Paraphimosis is usually caused by medical professionals or parents who handle the foreskin improperly: The foreskin may be retracted during penile examination, penile cleaning, urethral catheterization, or cystoscopy; if the foreskin is left retracted for a long period, some of the foreskin tissue may become edematous (swollen with fluid), which makes subsequent reduction of the foreskin difficult.
The most common location by far is the gingival margin and other areas of the masticatory oral mucosa, these occur more frequently in the fifth decade of life, and have good prognosis, the treatment of choice for oral VXs is surgical excision, and recurrence is rare.
The condition can affect other organs of body, such as the penis, vulva, and can occur in anal region, nose, the ear, lower extremity, scrotum.
Despite the similarity of name, an epispadias is not a type of hypospadias, and involves a problem with a different set of embryologic processes.
Women can also have this type of congenital malformation. Epispadias of the female may occur when the urethra develops too far anteriorly, exiting in the clitoris or even more forward. For females, this may not cause difficulty in urination but may cause problems with sexual satisfaction. Frequently, the clitoris is bifurcated at the site of urethral exit, and therefore clitoral sensation is less intense during sexual intercourse due to frequent stimulation during urination. However, with proper stimulation, using either manual or positional techniques, clitoral orgasm is definitely possible.
Most cases involve a small and bifid penis, which requires surgical closure soon after birth, often including a reconstruction of the urethra. Where it is part of a larger exstrophy, not only the urethra but also the bladder (bladder exstrophy) or the entire perineum (cloacal exstrophy) are open and exposed on birth, requiring closure.
Procedure for buried penis is the insertion of a subcutaneous soft silicone implant under the penile skin. The procedure was developed by urologist James J. Elist.
It is considered a form of 5-alpha-reductase deficiency involving SRD5A2.
Paraphimosis is an uncommon medical condition in which the foreskin of a penis becomes trapped behind the glans penis, and cannot be "reduced" (pulled back to its normal flaccid position covering the glans). If this condition persists for several hours or there is any sign of a lack of blood flow, paraphimosis should be treated as a medical emergency, as it can result in gangrene.
Sclerosing lymphangitis is a skin condition characterized by a cordlike structure encircling the coronal sulcus of the penis, or running the length of the shaft, that has been attributed to trauma during vigorous sexual play.
Nonvenereal sclerosing lymphangitis is a rare penile lesion consisting of a minimally tender, indurated cord involving the coronal sulcus and occasionally adjacent distal penile skin. This disorder most often occurs after vigorous sexual activity and resolves spontaneously.
Etiology of Sclerosing lymphangitis is unknown but has been postulated to be secondary to thrombosis of lymphatic vessels. Spontaneous recovery can occur anywhere within a couple weeks to several months.
Although it is commonly recommended the patient abstain from any sexual activitiy during the recovery, there is no evidence that this expedites recovery nor that not abstaining worsens the condition.