Bowen disease

ALSO KNOWN AS: Bowen’s disease, intraepidermal carcinoma, squamous cell carcinoma in situ

RELATED CONDITIONS:Squamous cell carcinoma (SCC), Bowenoid papulosis, erythroplasia of Queyrat

DEFINITION: Bowen disease is a type of skin cancer named after American dermatologist John T. Bowen (1857–1940), who documented it in 1912. Also known as squamous cell carcinoma in situ, it may arise anywhere on the skin or the mucous membranes. Bowen disease of the glans penis is called erythroplasia of Queyrat, named after French dermatologist Louis Queyrat (1856–1933), who first used the term "erythroplasia" (also spelled erythroplakia) in reference to a precancerous red lesion on the penis. Unlike other premalignant cancers, Bowen disease possesses a low potential for progression to invasive squamous cell carcinoma (SCC)—3 to 5 percent—and an even lower potential for metastasis. Another characteristic feature of Bowen disease is its greater propensity to spread over skin than to invade deeper skin layers. Although its malignant potential is low, extensive skin damage can result from delays in treatment.

Risk factors: Repeated exposure to the sun, exposure to the human papillomavirus (HPV), particularly HPV-16, and exposure to arsenic have been implicated as risk factors for Bowen disease. However, more often than not, no can account for disease development.

Etiology and the disease process: Bowen disease originates from the overproliferation of squamous cells, the flat superficial cells that make up the upper layer of the skin (epidermis). The entire epidermal layer is involved in Bowen disease. Triggers of this overproliferation include ultraviolet light from chronic sun exposure and exposure to HPV-16. Both can damage or alter cellular deoxyribonucleic acid (DNA) and the cellular controls regulating cell division. The TP53 gene is particularly susceptible to ultraviolet light damage and has been implicated as one of many possible mechanisms of cancer proliferation apart from Bowen disease. Arsenic has been hypothesized to enhance the carcinogenic properties of ultraviolet irradiation, as it has a propensity to accumulate in the skin as well as the lungs, bladder, kidney, and liver.

Incidence: Bowen disease is most common in Caucasians and people over forty years old and more common in women than in men. Incidence can vary depending on geographical location, increasing in areas with a significant Caucasian population that receive high levels of sun exposure. Rates have been estimated to range from 14.9 (Minnesota, 1991) to 142 (Hawaii, 1994) cases per 100,000 Caucasians.

Symptoms: Bowen disease manifests in an insidious manner and often mimics other diseases before being diagnosed. It often appears as a reddish, nonpigmented, raised, scaly lesion that gradually enlarges. The lesion well demarcated from surrounding normal skin. The most common sites are the head, neck, and extremities, although any part of the skin or mucous membrane may be affected. As the lesion enlarges, it thickens and starts to exhibit crusting, fissures, or ulcers. These lesions are usually solitary unless malignancy is present. Nonetheless, a full skin examination is warranted.

Screening and diagnosis: Bowen disease can easily be mistaken for other skin cancers, such as invasive SCC and basal cell carcinoma. Therefore, a punch or shave biopsy of the lesion is essential to initiate appropriate treatment, irrespective of location. The sample must include the full thickness of the epidermis and, as much as possible, hair structures in deeper layers. Staging of Bowen disease as a squamous cell carcinoma is not applicable unless invasion of the underlying dermis is seen during microscopic examination. Apart from routine skin examinations as part of a regular physical examination, there are no formal screening tests for Bowen disease.

Treatment and therapy: Medical and surgical treatments are available, although the latter are preferred. Medical treatment consists of topical treatment with either 5-fluorouracil (5-FU) or imiquimod 5% cream. 5-FU is used on the lesion after keratolytic therapy (controlled chemical breakdown) or cryotherapy (freezing). Iontophoresis can also be used to deliver the drug by inducing a drug “gradient” within the lesion. In women, 5-FU must not be taken during pregnancy. Radiotherapy using x-rays or grenz rays (low-energy x-rays) is reserved for patients who cannot undergo surgery or those with multiple lesions. Lesions may also be treated with photodynamic therapy, in which a chemical agent increases photosensitivity only within tumor cells. This microenvironment is then exposed to a specific wavelength of light, which causes local toxin and oxygen radical release and immune-system activation, leading to tumor cell death.

There are many surgical options, including excision of both the lesion and a small margin of normal tissue, Mohs micrographic surgery, curettage and electrodesiccation, cryotherapy, and carbon-dioxide laser ablation. Simple excision is preferred for small lesions in nonproblematic areas where deformities can be easily corrected. Mohs micrographic surgery, which is a very precise procedure also used in removing other skin cancers, is more suited to removing larger or recurring lesions and in areas where skin conservation is required for cosmetic reasons or functional preservation (for example, the face or joints). Curettage and electrodesiccation, cryotherapy, and carbon-dioxide laser are also effective treatments but do not provide pathologic specimens to confirm tumor eradication, particularly with deeper-lying lesions.

Prognosis, prevention, and outcomes: The overall prognosis of patients after treatment is excellent. Recurrence is often associated with inadequate removal of the lesion or malignant transformation and spread. Some of the sequelae of treatment include skin deformity, scarring, infection, and underlying damage to blood vessels and nerves.

Prevention focuses on decreasing unnecessary ultraviolet light exposure. This includes patient education regarding sun exposure and measures such as wearing protective clothing and sunscreen with an SPF of at least 15 and avoiding excessive exposure to the sun without proper protection.

Bibliography

“Bowen's Disease.” American Osteopathic College of Dermatology, www.aocd.org/page/BowensDisease. Accessed 12 July 2024.

Fossel, Michael B. Cells, Aging, and Human Disease. New York: Oxford UP, 2004. Print.

Habif, Thomas P., et al. Skin Disease: Diagnosis and Treatment. 3rd ed. Philadelphia: Saunders, 2011. Print.

Kirkham, Nigel. "Tumors and Cysts of the Epidermis." Lever's Histopathology of the Skin. Ed. David E. Elder et al. 10th ed. Philadelphia: Lippincott, 2009. 791–850. Print.

McCally, Michael, ed. Life Support: The Environment and Human Health. Cambridge: MIT P, 2002. Print.

Morton, C. A., A. J. Birnie, and D. J. Eedy. "British Association of Dermatologists' Guidelines for the Management of Squamous Cell Carcinoma In Situ (Bowen's Disease), 2014." British Journal of Dermatology 170.2 (2014): 245–60. Print.

Regan, Thomas D., and Naomi Lawrence. "Bowen's Disease and Erythroplasia of Queyrat." Treatment of Skin Disease: Comprehensive Therapeutic Strategies. Ed. Mark G. Lebwohl et al. 4th ed. Philadelphia: Saunders, 2014. 106–7. Print.

Reizner, George T., et al. "Bowen's Disease (Squamous Cell Carcinoma In Situ) in Kauai, Hawaii: A Population-Based Incidence Report." Journal of the American Academy of Dermatology 31.4 (1994): 596–600. Print.

Westers-Attema, Annet, et al. "Bowen's Disease: A Six-Year Retrospective Study with Emphasis on Resection Margins." Acta Dermato-Venereologica 94.4 (2014): 431–35. Print.