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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) (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. Skin: Structure and Functions. 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. Skin: Structure and Functions lesion. The lesion can occur in other areas as well. The genital region, particularly the penile shaft, is also affected. When the glans penis Glans Penis Penis: Anatomy 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. Papillomavirus (HPV) infection as a risk factor. Pathologic findings show full-thickness atypia Atypia Fibrocystic Change without basement membrane Basement membrane A darkly stained mat-like extracellular matrix (ecm) that separates cell layers, such as epithelium from endothelium or a layer of connective tissue. The ecm layer that supports an overlying epithelium or endothelium is called basal lamina. Basement membrane (bm) can be formed by the fusion of either two adjacent basal laminae or a basal lamina with an adjacent reticular lamina of connective tissue. Bm, composed mainly of type IV collagen; glycoprotein laminin; and proteoglycan, provides barriers as well as channels between interacting cell layers. Thin Basement Membrane Nephropathy (TBMN) invasion. Both entities may progress to invasive SCC. Thus, local destruction via surgical excision or topical chemotherapy Chemotherapy Osteosarcoma should be performed.

Last updated: Sep 13, 2022

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Bowen Disease

  • 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) (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. Skin: Structure and Functions cancer that is localized to the epidermis Epidermis The external, nonvascular layer of the skin. It is made up, from within outward, of five layers of epithelium: (1) basal layer (stratum basale epidermidis); (2) spinous layer (stratum spinosum epidermidis); (3) granular layer (stratum granulosum epidermidis); (4) clear layer (stratum lucidum epidermidis); and (5) horny layer (stratum corneum epidermidis). Skin: Structure and Functions
    • 3%–5% of cases progress to invasive SCC.
  • Annual incidence Incidence The number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time. Measures of Disease Frequency among white individuals is 14.9 cases per 100,000.
  • Risk factors: 
    • Sun exposure
    • Immunosuppression
    • Fair complexion
    • Smoking Smoking Willful or deliberate act of inhaling and exhaling smoke from burning substances or agents held by hand. Interstitial Lung Diseases
    • Arsenic Arsenic A shiny gray element with atomic symbol as, atomic number 33, and atomic weight 75. It occurs throughout the universe, mostly in the form of metallic arsenides. Most forms are toxic. According to the fourth annual report on carcinogens, arsenic and certain arsenic compounds have been listed as known carcinogens. Metal Poisoning (Lead, Arsenic, Iron) 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. Papillomavirus (HPV) infection (type 16 is the most common)
  • Clinical presentation: 
    • Erythematous, scaly, well-demarcated patch Patch Nonpalpable lesion > 1 cm in diameter Generalized and Localized Rashes/ plaque Plaque Primary Skin Lesions 
    • +/- Ulcerations
    • +/- Crusting
    • Sun-exposed areas (e.g., face, neck Neck The part of a human or animal body connecting the head to the rest of the body. Peritonsillar Abscess, and forearms)
    • Can affect genital region (e.g., penile shaft)
    • Grows slowly but can present as invasive SCC
    • Usually asymptomatic
  • Diagnosis: 
    • Skin biopsy Skin Biopsy Secondary Skin Lesions: gold standard test
    • Pathology:
      • Shows full-thickness atypia Atypia Fibrocystic Change without basement membrane Basement membrane A darkly stained mat-like extracellular matrix (ecm) that separates cell layers, such as epithelium from endothelium or a layer of connective tissue. The ecm layer that supports an overlying epithelium or endothelium is called basal lamina. Basement membrane (bm) can be formed by the fusion of either two adjacent basal laminae or a basal lamina with an adjacent reticular lamina of connective tissue. Bm, composed mainly of type IV collagen; glycoprotein laminin; and proteoglycan, provides barriers as well as channels between interacting cell layers. Thin Basement Membrane Nephropathy (TBMN) invasion
      • Large hyperchromatic nuclei with numerous mitoses
  • Treatment: 
    • Local excision
    • Topical chemotherapy Chemotherapy Osteosarcoma creams: 
      • Fluorouracil
      • Imiquimod Imiquimod A topically-applied aminoquinoline immune modulator that induces interferon production. It is used in the treatment of external genital and perianal warts, superficial carcinoma, basal cell; and actinic keratosis. Hypertrophic and Keloid Scars

Erythroplasia of Queyrat

  • SCC in situ (Bowen disease) of the glans penis Glans Penis Penis: Anatomy
  • Incidence Incidence The number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time. Measures of Disease Frequency is < 1 per 100,000 males.
  • Risk factors:
    • Lack of circumcision Circumcision Excision of the prepuce of the penis (foreskin) or part of it. HIV Infection and AIDS
    • 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. Papillomavirus (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. Papillomavirus (HPV) subtypes 16 and 18
    • Smoking Smoking Willful or deliberate act of inhaling and exhaling smoke from burning substances or agents held by hand. Interstitial Lung Diseases
    • Immunosuppression
  • Clinical presentation: 
  • Diagnosis: skin biopsy Skin Biopsy Secondary Skin Lesions (similar to Bowen disease)
  • Treatment: 
    • Local excision
    • Topical chemotherapy Chemotherapy Osteosarcoma creams: 
      • Fluorouracil
      • Imiquimod Imiquimod A topically-applied aminoquinoline immune modulator that induces interferon production. It is used in the treatment of external genital and perianal warts, superficial carcinoma, basal cell; and actinic keratosis. Hypertrophic and Keloid Scars
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 (SCC) (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. Skin: Structure and Functions malignancy Malignancy Hemothorax, caused by malignant proliferation of atypical keratinocytes Keratinocytes Epidermal cells which synthesize keratin and undergo characteristic changes as they move upward from the basal layers of the epidermis to the cornified (horny) layer of the skin. Successive stages of differentiation of the keratinocytes forming the epidermal layers are basal cell, spinous or prickle cell, and the granular cell. Skin: Structure and Functions. This condition usually affects sun-exposed areas of fair-skinned patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship. The cancer presents as a firm, erythematous, keratotic plaque Plaque Primary Skin Lesions or papule Papule Elevated lesion < 1 cm in diameter Generalized and Localized Rashes. 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: Anatomy 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: Histology of the glans, prepuce Prepuce The double-layered skin fold that covers the glans penis, the head of the penis. Penis: Anatomy, 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. Skin: Structure and Functions 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. Papillomavirus (HPV) infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease. Diagnosis is a combination of clinical findings and tissue biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma. Treatment approach depends on cancer stage and can include local topical therapy and multimodal surgical/ radiation Radiation Emission or propagation of acoustic waves (sound), electromagnetic energy waves (such as light; radio waves; gamma rays; or x-rays), or a stream of subatomic particles (such as electrons; neutrons; protons; or alpha particles). Osteosarcoma/ chemotherapy Chemotherapy Osteosarcoma.
  • 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. Papillomavirus (HPV) infection, which facilitates keratinocyte Keratinocyte Epidermal cells which synthesize keratin and undergo characteristic changes as they move upward from the basal layers of the epidermis to the cornified (horny) layer of the skin. Successive stages of differentiation of the keratinocytes forming the epidermal layers are basal cell, spinous or prickle cell, and the granular cell. Erythema Multiforme neoplastic transformation Transformation Change brought about to an organism’s genetic composition by unidirectional transfer (transfection; transduction, genetic; conjugation, genetic, etc.) and incorporation of foreign DNA into prokaryotic or eukaryotic cells by recombination of part or all of that DNA into the cell’s genome. Bacteriology. On skin biopsy Skin Biopsy Secondary Skin Lesions, 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 Transformation Change brought about to an organism’s genetic composition by unidirectional transfer (transfection; transduction, genetic; conjugation, genetic, etc.) and incorporation of foreign DNA into prokaryotic or eukaryotic cells by recombination of part or all of that DNA into the cell’s genome. Bacteriology to invasive SCC. Cryosurgery, excision, or topical therapy may be used for persistent 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.

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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