Bowen Disease and Erythroplasia of Queyrat

Bowen disease and erythroplasia of Queyrat are 2 related entities that describe squamous cell carcinoma Squamous cell carcinoma Cutaneous squamous cell carcinoma (cSCC) is caused by malignant proliferation of atypical keratinocytes. This condition is the 2nd most common skin malignancy and usually affects sun-exposed areas of fair-skinned patients. The cancer presents as a firm, erythematous, keratotic plaque or papule. Squamous Cell Carcinoma (SCC) in situ of the skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin. Bowen disease usually presents in sun-exposed areas (e.g., face and forearms) as a red, scaly skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin lesion. The lesion can occur in other areas as well. The genital region, particularly the penile shaft, is also affected. When the glans penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis is involved, the lesion is called erythroplasia of Queyrat, with uncircumcised males being at high risk. Both genital lesions have HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomaviridae: HPV infection as a risk factor. Pathologic findings show full-thickness atypia without basement membrane invasion. Both entities may progress to invasive SCC. Thus, local destruction via surgical excision or topical chemotherapy should be performed.

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Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

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Bowen Disease

  • Squamous cell carcinoma (SCC) in situ affecting any area of the body:
    • Represents a thin skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin cancer that is localized to the epidermis
    • 3%–5% of cases progress to invasive SCC.
  • Annual incidence among white individuals is 14.9 cases per 100,000.
  • Risk factors: 
    • Sun exposure
    • Immunosuppression
    • Fair complexion
    • Smoking
    • Arsenic exposure 
    • HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomaviridae: HPV infection (type 16 is the most common)
  • Clinical presentation: 
    • Erythematous, scaly, well-demarcated patch/plaque 
    • +/- Ulcerations
    • +/- Crusting
    • Sun-exposed areas (e.g., face, neck, and forearms)
    • Can affect genital region (e.g., penile shaft)
    • Grows slowly but can present as invasive SCC
    • Usually asymptomatic
  • Diagnosis: 
    • Skin biopsy: gold standard test
    • Pathology:
      • Shows full-thickness atypia without basement membrane invasion
      • Large hyperchromatic nuclei with numerous mitoses
  • Treatment: 
    • Local excision
    • Topical chemotherapy creams: 
      • Fluorouracil
      • Imiquimod

Erythroplasia of Queyrat

  • SCC in situ (Bowen disease) of the glans penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis
  • Incidence is < 1 per 100,000 males.
  • Risk factors:
    • Lack of circumcision
    • Chronic irritation (e.g., urine)
    • HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomaviridae: HPV infection: specifically high-risk HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomaviridae: HPV subtypes 16 and 18
    • Smoking
    • Immunosuppression
  • Clinical presentation: 
    • Red, well-defined velvety plaque over the glans or foreskin
    • +/- Ulcerations
    • +/- Bleeding
    • +/- Pruritus
  • Diagnosis: skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin biopsy (similar to Bowen disease)
  • Treatment: 
    • Local excision
    • Topical chemotherapy creams: 
      • Fluorouracil
      • Imiquimod
Erythroplasia of queyrat

Erythroplasia of Queyrat

Image: “Figure 1” by João Roberto Antônio et al. License: Public Domain

Differential Diagnosis

  • Cutaneous squamous cell carcinoma Squamous cell carcinoma Cutaneous squamous cell carcinoma (cSCC) is caused by malignant proliferation of atypical keratinocytes. This condition is the 2nd most common skin malignancy and usually affects sun-exposed areas of fair-skinned patients. The cancer presents as a firm, erythematous, keratotic plaque or papule. Squamous Cell Carcinoma (cSCC): the 2nd most common skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin malignancy, caused by malignant proliferation of atypical keratinocytes. This condition usually affects sun-exposed areas of fair-skinned patients. The cancer presents as a firm, erythematous, keratotic plaque or papule. Histopathologic examination provides the diagnosis. Surgical excision is the mainstay of treatment.
  • Penile cancer Penile cancer Malignant lesions of the penis arise from the squamous epithelium of the glans, prepuce, or penile shaft. Penile cancer is rare in the United States, but there is a higher prevalence in lower socioeconomic regions. The most common histologic subtype is squamous cell carcinoma. Penile Cancer: malignant lesions of the penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis arise from the squamous epithelium Epithelium The epithelium is a complex of specialized cellular organizations arranged into sheets and lining cavities and covering the surfaces of the body. The cells exhibit polarity, having an apical and a basal pole. Structures important for the epithelial integrity and function involve the basement membrane, the semipermeable sheet on which the cells rest, and interdigitations, as well as cellular junctions. Surface Epithelium of the glans, prepuce, or penile shaft. The most common histologic subtype is SCC. Both Bowen disease and erythroplasia of Queyrat are considered thin, noninvasive skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin cancers, but these conditions can progress to invasive SCC. Uncircumcised men are at highest risk, along with those with HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomaviridae: HPV infections. Diagnosis is a combination of clinical findings and tissue biopsy. Treatment approach depends on cancer stage and can include local topical therapy and multimodal surgical/radiation/chemotherapy.
  • Bowenoid papulosis Bowenoid papulosis Bowenoid papulosis is a sexually transmitted condition induced by HPV infection, which facilitates keratinocyte neoplastic transformation. On skin biopsy, Bowenoid papulosis manifests as low-grade dysplasia. Affected individuals present with genital papules of a red-to-brown color that are often asymptomatic. Bowenoid Papulosis: a sexually transmitted condition induced by HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomaviridae: HPV infection, which facilitates keratinocyte neoplastic transformation. On skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin biopsy, Bowenoid papulosis Bowenoid papulosis Bowenoid papulosis is a sexually transmitted condition induced by HPV infection, which facilitates keratinocyte neoplastic transformation. On skin biopsy, Bowenoid papulosis manifests as low-grade dysplasia. Affected individuals present with genital papules of a red-to-brown color that are often asymptomatic. Bowenoid Papulosis manifests as low-grade dysplasia. Presenting features include genital papules of a red to brown color that are often asymptomatic. Most cases resolve spontaneously. Lesions should be followed up due to the risk of transformation to invasive SCC. Cryosurgery, excision, or topical therapy may be used for persistent bowenoid papulosis.

References

  1. Bolognia, JL, Schaffer, JV, Cerroni, L. (2018). Actinic Keratosis, Basal Cell Carcinoma, and Squamous Cell Carcinoma. Dermatology, 4e. Edinburgh Elsevier.
  2. Curti, BD, Leachman, S, Urba, WJ. (2018). Cancer of the skin. In Jameson, J, et al. (Eds.). Harrison’s Principles of Internal Medicine, 20e. McGraw-Hill.
  3. Porten, SP, Presti, Jr., J.C. (2020). Genital tumors. In McAninch, JW, Lue, TF (Eds.). Smith & Tanagho’s General Urology, 19e. McGraw-Hill.

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