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Bowen Disease

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Copyright 1989, 2000, 2011, 2014

NORD is very grateful to Colin A. Morton, MD, Consulting Dermatologist, NHS Forth Valley, Stirling, UK, for assistance in the preparation of this report.

Synonyms of Bowen Disease

Disorder Subdivisions

General Discussion

Bowen disease is a rare skin disorder. Affected individuals develop a slow-growing, reddish scaly patch or plaque on the skin. Sun exposed areas of the skin are most often affected. Bowen disease only affects the outermost layer of the skin (epidermis). Lesions are usually not painful or may not be associated with any symptoms (asymptomatic). In most cases, treatment is highly successful. Bowen disease is considered a pre-cancerous condition, although the risk of developing skin cancer is less than 10 percent. The disorder usually affects older adults. The exact cause of Bowen disease is unknown, although there are identified risk factors such as chronic sun exposure.

Bowen disease was first described in the medical literature by a physician named JT Bowen in 1912. Bowen disease is also known as squamous cell carcinoma in situ, and is generally considered an early, noninvasive form of intraepidermal squamous cell carcinoma. Intraepidermal means that the disease occurs inside the epidermal layer of the skin.

Symptoms

Typically, Bowen disease appears as a slow-growing, persistent reddish-brown patch or plaque of dry, scaly skin. These lesions may be flat or slightly raised. The lesions are normally not associated with any symptoms, but, occasionally, can itch, ooze pus, bleed or become crusted and/or tender. In some cases, the lesions may be warty (verrucous), split open (fissured) or, less often, darkly colored (pigmented). In most cases, there is only one lesion, but in approximately 10-20 percent of individuals multiple lesions may develop usually in more than one area of the body.

Although Bowen disease occurs most often on sun exposed areas of the skin, it can develop anywhere on the body, even areas of the skin that are not usually exposed to the sun. The disorder most often develops on the lower legs. Less commonly, the head, neck, palms, soles and genitals can be affected. The lesions can measure anywhere from a few millimeters to a few centimeters.

Individuals with Bowen disease are at risk of developing skin cancer. The risk is estimated to be less than 10 percent, but can be higher in individuals with a compromised immune system. Early signs of cancerous transformation in Bowen disease include the development of a fleshy nodule or bump in a skin lesion. This nodule may be tender and bleed easily. Ulceration or hardening (induration) of a skin lesion also indicates malignant transformation.

Bowen disease is classified as an early, noninvasive form of squamous cell carcinoma, a type of skin cancer that most often occurs on sun damage areas of the skin. Squamous cell carcinoma is the second most common form of skin cancer.

Causes

The exact cause of Bowen disease is unknown. Chronic sun exposure and aging are believed to be two major risk factors for developing the disorder. Individuals with fair skin and individuals who spend a lot of time outdoors in the sun are at a greater risk of developing Bowen disease. Individuals who take drugs to suppress the immune system (usually taken to treat an immune system disorder) are also at a greater risk than the general population of developing Bowen disease.

Individuals who have cutaneous human papillomavirus (HPV) infection are at risk of developing Bowen disease. Human papilloma viruses are a group of more than 150 related viruses, some of which can cause cancer. HPV 16, 18, 34, and 48 have caused Bowen disease at genital sites. HPV 16 is most commonly associated with the development of Bowen disease. HPV 16 is also the cause of some cases of cervical cancer. Less often, HPV types 2, 16, 34, and 35 are associated with Bowen disease in areas of the body other than the genitals.

Chronic exposure to arsenic appears to be a risk factor for the development of Bowen disease as well. Arsenic is a tasteless, colorless metal element. Arsenic has many uses in manufacturing and other commercial uses. According to the medical literature, chronic exposure to arsenic can cause Bowen disease, approximately 10 years or so after initial exposure. In the past, arsenic was known to have contaminated well water and was once used in various medical preparations. Arsenic exposure occurs far less often today than it did in the past.

Affected Populations

The exact incidence of Bowen disease in the general population is unknown. Bowen disease is most frequently diagnosed in Caucasian individuals over the age of 60, although can occur in individuals much younger. It is believed to occur more frequently in males than females.

Related Disorders

Symptoms of the following disorders can be similar to those of Bowen disease. Comparisons may be useful for a differential diagnosis.

There are numerous different skin disorders that can resemble Bowen disease and the disorder is often mistaken for psoriasis or eczema. Additional skin disorders that can resemble Bowen disease include superficial basal cell carcinoma, actinic keratosis, seborrheic keratosis, lichen planus, tinea corporis and extramammary Paget's disease. (For more information on these disorders, choose the specific disorder name as your search term in the Rare Disease Database.)

When Bowen disease affects the genitals, it may be known as erythroplasia of Queyrat (involving the mucosal inner surface of the glans) in men or as a type of vulval intraepithelial neoplasia in women. Some researchers considered these conditions separate entities that are related to, but distinct from, Bowen disease.

Erythroplasia of Queyrat is also known as Bowen disease of the glans penis, although the latter term actually refers to the condition on the outer keratinized surface of the penis. The glans penis is the sensitive tip of the penis. Affected individuals develop a reddish, velvety or smooth plaque. A wide variety of symptoms can occur including crusting, scaling, ulceration, pain or itching. Bleeding, difficulty or pain when urinating (dysuria), or discharge may also occur. Erythroplasia of Queyrat occurs most often in middle aged men who are not circumscribed. The exact, underlying cause is of this disorder is unknown.

Vulvar intraepithelial neoplasia (VIN) is a general term for a skin condition that affects the vulva of women. Lesions may be bright red and have a velvety appearance. Initially there may be no associated symptoms, but eventually itching or burning sensations may develop. Itching can potentially be severe. The exact cause of VIN is unknown, by many cases are strongly associated with human papillomavirus 16.

Standard Therapies

Diagnosis
A diagnosis of Bowen disease is suspected based upon identification of characteristic symptoms, a detailed patient history and a thorough clinical evaluation. The disorder is easily mistaken for other skin disorders such as eczema or psoriasis and can be overlooked because there may be no associated symptoms. Bowen disease may sometimes first be noticed during a routine skin examination.

Clinical Testing and Work-Up
A diagnosis of Bowen disease may be confirmed by a biopsy of affected tissue. With a biopsy, a sample of affected tissue is removed and studied under a microscope. A biopsy can help to differentiate Bowen disease from other skin disorders with a similar appearance. The sample taken must be deep enough to rule out invasive squamous cell carcinoma.

Treatment
There is no specific, definitive treatment for Bowen disease. Several different therapies may be used all of which have excellent success rates. The specific treatment for an individual case depends upon numerous factors, such as the site of the body affected; the size, thickness and number of the lesion(s); the presence or absence of certain symptoms; an individual’s age and general health; and/or additional elements. Decisions concerning the use of particular drug regimens and/or other treatments should be made by physicians and other members of the health care team in careful consultation with the patient based upon the specifics of his or her case; a thorough discussion of the potential benefits and risks, including possible side effects and long-term effects; patient preference; and other appropriate factors.

A wide variety of treatment options exist for individuals with Bowen disease including topical chemotherapy, cryotherapy, curettage, photodynamic therapy and surgery. Most therapies have an excellent response rate and the prognosis of Bowen disease in most cases is excellent. The response to a particular therapy may vary - what works for one person may be ineffective in another. A treatment plan for Bowen disease will be tailored to a patient based on what is best for his or her individual case.

Some individuals may opt for no treatment (watch and wait). Watch and wait refers to when physicians follow a patient with a slow-growing disorder without giving treatment until progression of the disease occurs. This allows some people to avoid undergoing such therapies for many years. Elderly patients with a slow growing lesion in an area where healing may be poor (e.g., lower leg) are candidates for watch and wait.

Topical chemotherapy involves the application of creams applied directly to the lesion. Two common topical medications used to treat Bowen disease are 5-fluorouracil and imiquimod 5%. These treatments may be used alone or in conjunction with other therapies. 5-fluorouracil works by destroying the abnormal skin cells. Generally, affected individuals apply the cream once or twice daily for at least two weeks if not much longer.

Imiquimod 5% is generally used for lesions on the lower legs, larger lesions and the erythroplasia of Queyrat variant of Bowen disease.

Many individuals with Bowen disease may opt for surgical removal of a lesion. Straightforward surgical removal in which the lesion is cut out and the wound is closed by sutures may be used. Surgical removal of a lesion is highly successful, but will leave a surgical scar.

A specific type of surgery called Mohs micrographic surgery may be recommended for some individuals with Bowen disease, especially those with larger lesions, recurrent lesions on the head and neck or lesions that are located on areas that require as much preservation of the tissue as possible, e.g. around the nail. With this surgery, a surgeon uses a precise technique to remove the diseased tissue one layer at a time. According to the medical literature, Mohs surgery has the highest cure rate of all treatment options.

Cryotherapy may also be used to treat Bowen disease. Cryotherapy is the use of extreme cold to freeze and destroy the tissue and cells of skin lesions. Cryotherapy is a minimally invasive treatment option. With cryotherapy a freezing substance such as liquid nitrogen or argon gas is applied directly to the lesion. Cryotherapy is most effective for single or small lesions.

Another surgical procedure used to treat individuals with Bowen disease is curettage with cautery/electrocautery. With curettage, the lesion is scraped off the skin. The procedure is usually performed under anesthesia. In some cases, curettage may be followed by cauterization, in which the lesion is burned by an electrical charge. This procedure may need to be repeated in some cases and will often leave a small white scar.

Photodynamic therapy, a procedure in which a drug known as a photosensitizer is used along with a special type light, has been used to treat some individuals with Bowen disease who have large lesions. During photodynamic therapy, the drug is administered to an affected individual and absorbed by the affected cells. A specific wavelength of light is used to activate the drug which binds with oxygen creating a chemical that destroys the affected cells.

In the past, x-ray or radiation therapy (radiotherapy) was often used to treat individuals with Bowen disease, especially individuals who were poor candidates for surgery or who had multiple lesions. Individuals with lesions on the legs are not recommended for radiotherapy because of poor wound healing in that area. Radiotherapy is used less often for the treatment of Bowen disease today than it was in the past.

Prevention
The most important step to take to lower the risk of Bowen disease is to limit or avoid excess exposure to the sun. Protective clothing, sunscreen, avoiding tanning beds and other measures can be taken to lower the risk of developing Bowen disease.

Investigational Therapies

Various case reports in the medical literature discuss the use of laser therapy for the treatment of Bowen disease. In individual patients, laser therapy has been effective in treating the disorder. However, no clinical trials in large populations have been undertaken. In addition, laser therapy may be expensive and have limited availability. More research is necessary to determine the long-term safety, effectiveness and viability of laser therapy as a potential treatment for Bowen disease.

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, in the main, contact:
www.centerwatch.com

For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/

Organizations related to Bowen Disease

Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder.

References

TEXTBOOKS
Rowert-Huber J. Bowen's Disease. In: Managing Skin Cancer, Stockfleth E, Rosen T, Shumack S, eds. Springer-Verlag, Berlin. 2010:23.

Eisen DB, Elgart ML. Bowen Disease. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:97-98.

JOURNAL ARTICLES
Morton CA, Birnie AJ, Eedy DJ. British Association of Dermatologists' guidelines for the management of squamous cell carcinoma in situ (Bowen's disease). British Journal of Dermatology. 2014;170: 245-260. doi: 10.1111/bjd.12766

Lopez N, Meyer-Gonzalez T, Herrera-Acosta E, et al. Photodynamic therapy in the treatment of Bowen's disease. J Dermatolog Treat. 2012;23(6):428-30. http://www.ncbi.nlm.nih.gov/pubmed/21787214

Neubert T, Lehmann P. Bowen's disease - a review of newer treatment options. Ther Clin Risk Manag. 2008;4:1085-1095.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2621408/?tool=pubmed

Lee MM, Wick MM. Bowen's disease. CA: Cancer Journal for Clinicians. 2008;40:237-242. http://onlinelibrary.wiley.com/doi/10.3322/canjclin.40.4.237/pdf

Cox NH, Eedy DJ, Morton CA. Guidelines for management of Bowen's disease: 2006 update. Br J Dermatol. 2007;156:11-21. http://www.ncbi.nlm.nih.gov/pubmed/17199561

Akhdari N, Amal S, Ettalbi S. Bowen disease. CMAJ. 2006;175:740. http://www.cmaj.ca/content/175/7/739.1.long

Navi D, Huntley A. Imiquimod 5 percent cream and the treatment of cutaneous malignancy. Dermatol Online J. 2004;10:4. http://dermatology.cdlib.org/101/reviews/imiquimod/navi.htm

Derancourt C, Mougin C, Chopard LM, et al. Oncogenic human papillomaviruses in extra-genital Bowen disease revealed by in situ hybridization. Ann Dermatol Venereol. 2001;128:715-718. http://www.ncbi.nlm.nih.gov/pubmed/11460032

Morton CA, Whitehurst C, McColl JH, Moore JV, MacKie RM. Photodynamic therapy for large or multiple patches of Bowen disease and basal cell carcinoma. Arch Dermatol. 2001;137:319-324. http://www.ncbi.nlm.nih.gov/pubmed/11255332

INTERNET
Eid MP, Anderson BE. Bowen Disease. Medscape, Updated: Jan 24, 2012. Available at: http://emedicine.medscape.com/article/1100113-overview Accessed Jan 2, 2014.

Egan KM, Maino KL. Erythroplasia of Queyrat. Medscape, Updated: May 31, 2012. Available at: Accessed on: http://emedicine.medscape.com/article/1100317-overview Accessed Jan 2, 2014.

Report last updated: 2014/12/25 00:00:00 GMT+0