Erythroplasia of Queyrat

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]

Synonyms and keywords: EQ

Overview

Erythroplasia of Queyrat was named after Louis Queyrat, a French dermatologist who was head of the dermatology service of l'Hôpital Ricord, a venereal hospital in Paris, now Hôpital Cochin. The pathogenesis of Erythroplasia of Queyrat is characterized as a precancerous lesion of squamous cell carcinoma in situ of the glans penis and inner prepuce. Erythroplasia of Queyrat is most commonly observed among white male patients aged 40 years old and older. The most common risk factor in the development of Erythroplasia of Queyrat is an uncircumcised penis. The mainstay of therapy for Erythroplasia of Queyrat is imiquimod or 5-fluorouracil for several weeks to months.

Historical Perspective

• Erythroplasia of Queyrat was named after Louis Queyrat, a French dermatologist who was head of the dermatology service of l'Hôpital Ricord, a venereal hospital in Paris, now Hôpital Cochin.

Tarnovsky originally described erythroplasia of Queyrat in 1891, but it was Queyrat who coined the term erythroplasia in 1911.

[Disease name] was first discovered by [name of scientist], a [nationality + occupation], in [year]/during/following [event].

The association between [important risk factor/cause] and [disease name] was made in/during [year/event].

In [year], [scientist] was the first to discover the association between [risk factor] and the development of [disease name].

In [year], [gene] mutations were first implicated in the pathogenesis of [disease name].

There have been several outbreaks of [disease name], including -----.

In [year], [diagnostic test/therapy] was developed by [scientist] to treat/diagnose [disease name].

Classification

Erythroplasia of Queyrat is classified as a precancerous lesion and the earliest stage of squamous cell cancer of the penis known as Carcinoma in Situ (CIS). This is also known as stage 0 of penile cancer. In this stage, the cancer cells are found only in the top layers of skin. They have not yet grown into the deeper tissues.

Depending on where the CIS is on the penis, doctors may use other names for the disease.

• CIS of the glans or prepuce is called Erythroplasia of Queyrat, presents as erythroplakia.
• CIS on the shaft of the penis (or other parts of the genitals) is called Bowen disease, presents as leukoplakia.

About 95% of penile cancers start in flat skin cells called squamous cells. Squamous cell carcinoma can start anywhere on the penis. Most of these cancers start on the prepuce or foreskin (in men who have not been circumcised) or on the glans. These tumors tend to grow slowly. If they're found at an early stage, they can usually be cured.

Squamous Cell Carcinoma of Penis may be classified according to Jackson's Staging System into number subtypes/groups: I, II, III, and IV.

Jackson's Staging System for Squamous Cell Carcinoma of Penis

Stage Description

I Confined to glans of prepuce

II Invasion into shaft or corpora

III Operable inguinal lymph node metastasis

IV Tumor invades adjacent structures; inoperable inguinal lymph node metastasis

Histopathological Features

• Low-grade (I-II), well-differentiated lesions show a thickened, hyperkeratotic, and papillomatous epidermis, with downward fingerlike projection of atypical squamous cells that often appear as concentrically arranged nests of cells surrounding keratin accumulations (keratin pearls).
• More poorly differentiated SCC (III-IV) shows little or no keratinization, increased nuclear pleomorphism, is hyperchromatic, and deeper invasion and may have areas of necrosis or superinfection.

Pathophysiology

• The pathogenesis of Erythroplasia of Queyrat is characterized by squamous cell carcinoma in situ of the glans penis

It is a premalignant dermatosis that usually occurs on the glans penis and appears as a well-marginated erythematous velvety patch or plaque.

Analogous to Bowen's disease (BD), infiltration, nodularity or ulceration often suggest the possibility of conversion to an invasive squamous cell carcinoma. Transformation of EQ into an invasive SCC is more common than in BD, with an incidence varying from 10%to 33%. This difference could be related to the mucosal location of the disease. If the penile submucosa is invaded, the rate of involvement of regional lymph nodes is about 20%. Clinically, the presence of ulceration and/or papillary lesions usually corresponds to progression into an invasive carcinoma.

The exact pathogenesis of [disease name] is not fully understood.

OR

It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].

OR

[Pathogen name] is usually transmitted via the [transmission route] route to the human host.

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Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.

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[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].

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The progression to [disease name] usually involves the [molecular pathway].

OR

The pathophysiology of [disease/malignancy] depends on the histological subtype.

Causes

Besides old age and lack of circumcision, Erythroplasia of Queyrat has been linked to various factors including:

• Chronic irritation from retained secretions under the foreskin
• Poor hygiene
• Smegma
• Genital herpes simplex
• Heat, friction
• Trauma
• Human Papilloma Virus (HPV) infection, types 16, 18, 31, 33.

Differentiating ((Page name)) from Other Diseases

Erythroplasia of Queyrat must be differentiated from other diseases that cause Squamous cell carcinoma of penis. Differentials include:

• Bowen's Disease
• Bowenoid Papulosis
• Verrucous carcinoma: A verrucous carcinoma growing on the penis is also known as Buschke-Lowenstein tumor. This is an uncommon form of squamous cell cancer that can start in the skin in many areas. This cancer looks a lot like a large genital wart. Verrucous carcinomas tend to grow slowly but can sometimes get very large. They can grow deep into nearby tissue, but they rarely spread to other parts of the body.

Epidemiology and Demographics

In countries where infant circumcision is common, such as Israel and the United States, the incidence of Penile Squamous Cell Carcinoma is less than 1 per 100,000 males, in which it constitutes a small proportion of the total cancers. However, Squamous cell cancer accounts for more than 95% of cases of penile cancer. This represents a significant public health problem in several parts of the world where early circumcision and good genital hygiene are less commonly practiced. Circumcision later in life does not seem to confer protection.

Age

• Erythroplasia of Queyrat is more commonly observed among patients aged 40 years old.

Gender

• Males are affected with Erythroplasia of Queyrat.

Risk Factors

• Most common risk factor in the development of Erythroplasia of Queyrat is uncircumcised penis.

There are no established risk factors for [disease name].

OR

The most potent risk factor in the development of [disease name] is [risk factor 1]. Other risk factors include [risk factor 2], [risk factor 3], and [risk factor 4].

OR

Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

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Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.

Screening

There is insufficient evidence to recommend routine screening for [disease/malignancy].

OR

According to the [guideline name], screening for [disease name] is not recommended.

OR

According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3].

Natural History, Complications, and Prognosis

If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

OR

Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].

OR

Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.

Diagnosis

Diagnostic Study of Choice

The diagnosis of [Erythroplasia of Queyrat] is confirmed with histological examination. at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].

Delays in the diagnosis and treatment of Erythroplasia of Queyrat are common because of two main factors.

• Early penile SCC often has a varying clinical presentation, mimicking benign disorders.
• Patients often tend to disregard minimal genital lesions for a long time before seeking medical attention.

Delay in diagnosis of more than 1 year has been observed in 15% to 20% of patients, the reasons usually being embarrassment, guilt, fear, personal neglect, or ignorance.

OR

The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].

OR

The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].

OR

There are no established criteria for the diagnosis of [disease name].

Symptoms

• Symptoms of Erythroplasia of Queyrat may include the following:

• Red rash on the tip of the penis
• Irritation on the tip of the penis

History and Symptoms

The majority of patients with [disease name] are asymptomatic.

OR

The hallmark of [disease name] is [finding]. A positive history of [finding 1] and [finding 2] is suggestive of [disease name]. The most common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3]. Common symptoms of [disease] include [symptom 1], [symptom 2], and [symptom 3]. Less common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].

Physical Examination

Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].

OR

Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

The presence of [finding(s)] on physical examination is diagnostic of [disease name].

OR

The presence of [finding(s)] on physical examination is highly suggestive of [disease name].

Laboratory Findings

An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].

OR

Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

OR

[Test] is usually normal among patients with [disease name].

OR

Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].

OR

There are no diagnostic laboratory findings associated with [disease name].

Electrocardiogram

There are no ECG findings associated with [disease name].

OR

An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

X-ray

There are no x-ray findings associated with [disease name].

OR

An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with [disease name].

OR

Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

CT scan

There are no CT scan findings associated with [disease name].

OR

[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

MRI

There are no MRI findings associated with [disease name].

OR

[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Other Imaging Findings

There are no other imaging findings associated with [disease name].

OR

[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

There are no other diagnostic studies associated with [disease name].

OR

[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• There are several non-invasive treatment options for erythroplasia of Queyrat such as photodynamic therapy, cryosurgery and applying various kinds of topical agents. The mainstay of therapy for Erythroplasia of Queyrat is imiquimod or 5-fluorouracil for several weeks to months. A therapeutic regimen of 5 per cent 5-fluorouracil cream applied to lesion(s) twice daily for four to five weeks has produced a high cure rate and maintained penile integrity and function.

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR   The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

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Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

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Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Surgery

Surgical excision is the treatment of choice, although some authors have used 5% 5-FU cream with some success. When the lesion is limited to preputial skin, circumcision is recommended. Mohs microscopic surgery is also effective and can be performed for patients with aggressive forms of Erythroplasia of Queyrat.

Surgical intervention is not recommended for the management of [disease name].

OR

Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]

OR

The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].

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The feasibility of surgery depends on the stage of [malignancy] at diagnosis.

OR

Surgery is the mainstay of treatment for [disease or malignancy].

Primary Prevention

There are no established measures for the primary prevention of Erythroplasia of Queyrat. However, circumcision at an early age is thought to prevent its development.

OR

There are no available vaccines against [disease name].

OR

Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

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[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].

Secondary Prevention

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].