Practice Essentials
First described by the American dermatologist John T. Bowen in 1912, Bowen disease is a squamous cell carcinoma (SCC) in situ with the potential for significant lateral spread. [1] Larger lesions can reach several centimeters in diameter.
The image below depicts Bowen disease of the right temple.
Signs and symptoms
Patients often present with an asymptomatic, slowly enlarging, erythematous, well-demarcated scaly patch or plaque. It may occur anywhere on the mucocutaneous surface. A delay in diagnosis of Bowen disease often is encountered because the lesion is asymptomatic; early skin changes may be subtle and overlap with clinical features seen in many conditions, such as tinea corporis, nummular eczema, seborrheic keratosis, [2, 3] Paget disease, superficial basal cell carcinoma, actinic keratosis, and psoriasis. A classic clinical history is presentation of a non–steroid-responsive dermatosis.
Bowen disease presents as a single lesion in two thirds of cases. Lesions may appear on sun-exposed or covered skin. The head, neck, and extremities are the most commonly affected anatomic locations in men, while the lower limbs and cheeks are most commonly affected in women. [4] Lesions, as shown in the images below, vary in size from a few millimeters to several centimeters in diameter.
A sharply demarcated, irregular border usually is present. Lesions are erythematous, scaly patches or plaques that may become hyperkeratotic, crusted, fissured, or ulcerated. Rarely, the lesions are pigmented, especially in the genital region and the nails. [5] Lesions in these locations may simulate melanoma. [6] Bowen disease also may occur on mucous membranes. When it arises on the glans penis, it is referred to as erythroplasia of Queyrat and presents as an erythematous, moist, velvety or smooth plaque, as demonstrated in the image below.
Diagnostics
Also see Histologic Findings.
Skin biopsy
Perform a shave or punch biopsy to confirm the diagnosis of Bowen disease. Incorporating follicular structures in the biopsy material is helpful, as is sampling many areas of larger lesions to exclude evidence of invasion amounting to a cutaneous squamous cell carcinoma. These procedures are typically performed in an office setting with the patient under local anesthesia. Pathologic analyses are best completed by dermatopathologists.
Complete skin examination
Perform a total body skin examination on patients with Bowen disease on sun-exposed skin. Studies indicate a higher incidence of nonmelanoma skin cancers may exist in these patients.
Management
Please see Treatment, Guidelines, and Medication.
Pathophysiology
Bowen disease is a form of intraepidermal carcinoma, a malignant tumor of keratinocytes. Bowen disease may ultimately progress to an invasive squamous cell carcinoma.
Etiology
Bowen disease may arise de novo or from a preexisting actinic keratosis. The etiology is most likely multifactorial.Note the following:
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Chronic UV radiation exposure: The age and sun-exposed body distribution of Bowen disease suggests the importance of chronic sun damage as a factor in the carcinogenesis of Bowen disease. [7]
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Arsenic exposure: The literature supports an association between Bowen disease and arsenic exposure, often occurring after a time lag of 10 years. The main sources of arsenic exposure include Fowler solution, a medication formerly used to treat psoriasis; Gay solution, a medication formerly used to treat asthma; contaminated well water; and certain pesticides. [8, 9]
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Immunosuppression: Immunosuppressed patients with Bowen disease are more likely to have multiple tumors and more aggressive tumors. [14]
Other possible causes include genetic factors, trauma, other chemical carcinogens, and x-ray radiation.
Epidemiology
US frequency
Because no national health databases collect the numbers of nonmelanoma skin cancers and because of regional differences in incidence rates, estimating the frequency of Bowen disease is difficult. In 1991, a study from Minnesota reported the annual average rate of Bowen disease as 14.9 cases per 100,000 Whites. [15] In 1994, a study from Hawaii reported a rate 10 times that, 142 cases per 100,000 Whites. [16]
Race-, sex-, and age-related information
Bowen disease is most commonly reported in sun-exposed sites of Whites. Bowen disease rarely occurs in patients with darker-pigmented skin; if it does, it usually affects nonexposed sites. [17]
The ratio of Bowen disease is approximately equal between males and females. Bowen disease is more commonly found on the head and neck of men and on the lower limbs and cheeks of women. [7]
Bowen disease occurs in adulthood, with the highest incidence in patients older than 60 years. [18]
Prognosis
The prognosis for Bowen disease is favorable. The majority of studies report a risk of progression to invasive SCC at 3-5%. According to a retrospective case series with multiple potential biases, of those that become invasive SCC, one third may metastasize. The risk of invasive carcinoma is estimated to be higher for genital Bowen disease or erythroplasia of Queyrat at 10%. [19]
Much controversy surrounds whether Bowen disease is associated with internal malignancies. [20, 21, 22, 23, 24] Many early papers reported such an association in 15-70% of cases. Some later reports supported an association of internal malignancies with Bowen disease that was associated with arsenic ingestion but not with Bowen disease from other causes. [8] In 1989, a meta-analysis of 12 studies showed no significant association. The most recent population-based cohort study of 1147 Bowen disease patients in Denmark demonstrated no statistically significant increased risk of internal cancers. Currently, Bowen disease is not believed to be a paraneoplastic condition. [24]
Patient Education
Patients with a history of Bowen disease should be counseled on safe sun behavior, to include avoiding the sun when it is most intense (between 10 am and 4 pm), wearing hats and other sun protective clothing, and using a dual-spectrum sunscreen with an SPF of 30 or higher.
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Squamous cell carcinoma in situ, Bowen disease. Courtesy of Hon Pak, MD.
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Bowen disease right temple.
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Erythroplasia of Queyrat, squamous cell carcinoma in situ of the glans penis.
