Erythroplasia of Queyrat (Bowen Disease of the Glans Penis)

Background

Erythroplasia of Queyrat (EQ) is an in situ squamous cell carcinoma of the penis.^[1] The glans and prepuce are most commonly involved.^[2] Erythroplasia of Queyrat is seen almost exclusively in uncircumcised men. Progression to invasive carcinoma may occur, and spontaneous regression is unlikely.^{[3, 4]}

Erythroplasia of Queyrat was originally described by Tarnovsky in 1891 and later appreciated as a penile disease by Fournier and Darier in 1893. Studies by Queyrat in 1911 allowed erythroplasia of Queyrat to be accepted as a distinct entity. In 1933, Sulzberger and Satenstein recognized erythroplasia of Queyrat as a form of carcinoma in situ.^[5]

Some references equate erythroplasia of Queyrat with the term Bowen disease of the glans penis”.^[6] The term erythroplasia of Queyrat is used for squamous cell carcinoma in situ (SCCIS) of the mucocutaneous epithelium of the penis.^[1]

Some references extend use of the term erythroplasia of Queyrat to also describe SCCIS of the labia minora, vestibule, vulva, labia majora, conjunctivae, buccal mucosa, and anal mucosa.^{[1, 4, 7]}

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Etiology

The etiology of erythroplasia of Queyrat remains unclear. The following have been proposed to contribute to the development and progression of erythroplasia of Queyrat^{[1, 4, 6, 8, 9, 10, 11]} :

• Lack of circumcision
• Chronic irritation, inflammation, or infection: Includes urine, smegma, trauma, herpes simplex viral infection, bacteria, heat, friction, trauma
• Zoon balanitis
• Human papillomavirus infection (HPV) types 8 and 16: In 2010, however, Nasca et al failed to detect HPV in lesions of 11 patients with erythroplasia of Queyrat
• Immunosuppression (including HIV infection)
• UV light
• Phimosis
• Multiple sexual partners
• Smoking
• Chronic underlying diseases (lichen sclerosis, lichen planus)
• Social/cultural habits, hygiene, religious practices

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Epidemiology

Frequency

Erythroplasia of Queyrat is a rarely reported disorder. It makes up less than 1% of malignancies in males.^[6]

Age

Erythroplasia of Queyrat is a disease of middle-aged to elderly males.^[8] It has been described in males ranging from age 20-80 years.^[12]

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Prognosis

The cure rate for erythroplasia of Queyrat is high if lesions are identified and treated early.

If urethral involvement is noted, treatment may be both more challenging and lead to higher recurrence rates.^[6]

Transformation to invasive carcinoma is possible within erythroplasia of Queyrat lesions. Graham and Helwig reported 10% of erythroplasia of Queyrat cases progressing to malignant disease.^[12] Others report progression rates as high as 33%.^[4] Cases of erythroplasia of Queyrat metastatic to local lymph nodes have also been reported.^[13]

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History

Patients with erythroplasia of Queyrat (EQ) typically present with solitary or multiple, often times nonhealing, lesions on the glans penis and/or adjacent mucosal epithelium.^[1]

Presenting symptoms can vary and may include the following:

• Redness
• Crusting
• Scaling
• Ulceration
• Bleeding
• Pain
• Itching
• Dysuria
• Penile discharge
• Difficulty retracting the foreskin

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Physical Examination

Single or multiple, nontender, slightly raised, red papules and plaques on the glans penis and/or adjacent mucosal epithelium are seen in erythroplasia of Queyrat; the inner surface of the foreskin or coronal sulcus may be involved.^[1] The plaques may appear smooth, velvety, scaly, crusty, or verrucous. Note the image below.

The patient is usually uncircumcised.^[14]

There may also be involvement or the urethral meatus.^[9]

View Image

Erythroplasia of Queyrat. Courtesy of Hon Pak, MD.

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Complications

Dermatological and urological complications may occur.

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Laboratory Studies

The diagnosis of erythroplasia of Queyrat (EQ) is made via histological examination. Specifically, biopsy should be performed on any areas with signs of bleeding, ulceration, or papillomatous change.^[6] Additionally, biopsy should be performed on therapy-resistant lesions.^[1] The following diagnostic procedures may be useful in excluding other infectious processes:

• Tzanck preparation
• Bacterial/viral/fungal culture
• Potassium hydroxide examination
• Gram stain

Early invasive disease should be evaluated for with several biopsies as needed.^[6] Failure to carefully evaluate any patient, especially uncircumcised patients, presenting with a subacute or chronic balanitis is a potential medicolegal pitfall. The threshold for performing skin biopsy of any lesion should be very low. In addition, a failure to diagnose erythroplasia of Queyrat expediently can easily result in disease that progresses to frank squamous cell carcinoma of the penis.

Some authors have also called for optical coherence tomography in conjunction with skin biopsy.^[15]

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Histologic Findings

Histologic findings include the following^{[14, 9]} :

• Epidermal acanthosis, parakeratosis
• Partial- or full-thickness epidermal atypia
• Possible dyskeratosis
• Possible lymphohistiocytic dermal infiltrate

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Medical Care

Cases of erythroplasia of Queyrat (EQ) have been treated with the following:

• 5-Fluorouracil^{[16, 17]}  - Limited success
• Imiquimod^{[18, 19, 20, 21, 22]}  - Variable response with limited data on long-term efficacy

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Surgical Care

Surgical treatments for erythroplasia of Queyrat include the following^[23] :

• Mohs micrographic surgery^{[1, 24, 25, 26]} : Five-year cure rate up to 90%^[1]
• Surgical excision^[4] : Recurrence rate of 2% for total glansectomy^[9] ; penis preserving strategies recommended for small lesions^{[6, 9]} ; may include partial glansectomy and circumcision with skin grafting^[6]
• Cryotherapy
• Electrodesiccation and curettage
• Radiation
• Carbon dioxide laser ablation^{[27, 28, 29]}
• Nd:YAG laser ablation^[30]
• Photodynamic therapy with aminolevulinic acid: Multiple treatments may be required for clearance^[31]
• Photodynamic therapy with methyl-aminolevulinate: Multiple treatments may be required for clearance^[32] ; studies have shown up to 83% of patients with clinical remission,^{[33, 34]} but rates as low as 27% reported (2011)^[25] ; may lead to preservation of function and good cosmesis^[25] ; may be used in cases of recurrence or if surgery not desired^[25] ; adverse effects include redness, burning, pain, swelling, dysuria, ulceration, blistering, and pigment changes^{[25, 33]}

Circumcision is recommended.^[6]

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Long-Term Monitoring

Close follow-up is recommended for patients treated medically or surgically.^[35]

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Medication Summary

Several case reports describe limited success in treating selected superficial lesions of erythroplasia of Queyrat (Bowen disease of the glans penis) with topical 5-fluorouracil 5% cream.

Several case reports and series describe successful treatment of noninvasive erythroplasia of Queyrat (Bowen disease of the glans penis) with topical imiquimod 5% cream.

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