Practice Nursing - Managing Bowen's disease in primary care settings

Abstract

Bowens disease is a rare condition, considered a precursor to squamous cell carcinoma. Because of its similarity to several other skin conditions, accurate diagnosis can be challenging. There are multiple risk factors and several treatment options. This article has hoped to give an overview of risk factors, signs and symptoms, treatment, and complications with the aim of giving nurses and non-medical prescribers increased confidence in recognising this disease.

Bowen's disease is a rare condition thought to affect approximately 15 per 100,000 people in the UK each year. Because of the similarity with other skin conditions, diagnosis can be challenging for clinicians.

Bowen's disease, also called squamous cell carcinoma (SCC) in situ is regarded as a precursor to skin cancer. Because of the similarity with other skin conditions, diagnosis can be challenging for clinicians. This article aims to give an overview of the condition and will discuss risk factors, recognition, diagnosis, treatment, and complications. It is hoped that the information provided will give nurses and non-medical prescribers more confidence in recognising this disease with the aim of getting patients an earlier diagnosis, reducing the risk of complications, and improving quality of life.

Prevalence

Bowen's disease is a rare condition thought to affect approximately 15 per 100,000 people in the UK each year (GP Notebook 2020). Although the condition can affect both males and females, approximately three quarters of cases occur in women, commonly in their 60's with a higher occurrence in those with pale skin (Tidy 2021). The condition is rare in patients with darker pigmented skin colouration.

Risk factors

Potential causes have been extensively studied but, in some patients, there may be no obvious cause. The following have been cited as the most likely to be associated:

Long term exposure to the sun and use of sunbeds

Patients with impaired immunity are also at increased risk. This includes those undergoing treatment for cancer, those who have had an organ transplant and/ or are on medication to prevent organ rejection and those with HIV or AIDS. (Willacy 2023)

Patients who have had an organ transplant receiving immunosuppressive drugs such as azathioprine or cyclosporin are at increased risk because it is thought these drugs may activate different pathways resulting in induction and promotion of skin malignancies. (Palaniappan and Karthikeyan 2022)

Skin damage or scarring of the skin caused by a previous injury may also be associated with increased risk of development Bowen's disease at the site of the original injury.

Genetic factors, exposure to chemical carcinogens, and to x-ray radiation are also indicated.

Other risk factors are shown in table 1.

Table 1: Additional risk factors for Bowen's disease

Risk factor	Additional information
A history of non-melanoma skin cancer (e.g., Basal cell or squamous cell carcinoma)	Patients who have had Bowens disease are also at a greater risk of developing a non-melanoma skin cancer
Exposure to arsenic	Bowens disease arises after several decades
HPV virus	Several subtypes of HPV virus such as HPV 16,18,31,32,33,35, 54, 58m 61,62, and 73 have been detected. There is a strong association with HPV 16 and vulvar Bowens disease
Autoimmune disease	Can occur in patients with chronic systemic lupus erythematosus (SLE) or lupus vulgaris

Pathophysiology

The condition arises as a result of the growth of cancerous cells in the outer layer of the skin (epidermis) and is a malignant tumour of the keratinocytes (Kaidas 2023).

Signs and symptoms

Bowen's disease can affect anywhere on the body but is most commonly seen at sites regularly exposed to the sun such as the face, arms, neck, hands, scalp, and lower legs. In the UK, three quarter of cases occur on the leg below the knee (Tidy 2021). Less common sites are shown in table 2. The patient typically develops an asymptomatic slowly enlarging, patch of red-looking skin at the affected site. Typically, the lesion is a well-demarcated, erythematous, scaly patch or plaque, with some degree of redness, ranging from pink to bright salmon-red erythema (Palaniappan and Karthikeyan 2022). Some patients may experience itching and the lesion may feel sore and, in some cases, will bleed. Most people affected will have only one patch of Bowen's disease, but approximately 10 to 20% of affected individuals will have multiple lesions (Palaniappan and Karthikeyan 2022). A delay in diagnosis of Bowen's disease is often a problem when the lesion is asymptomatic and any early skin changes may be subtle and overlap with clinical features seen in many other skin conditions (Kaidas 2023). See below for similar disorders.

Table 2: Less common sites of Bowen's disease (Sirka et al 2021)

Site of Bowens disease	Additional information
SCC in aitu of the penis (Erythroplasia of Queryat) (EQ)	Risk factors include poor hygiene, uncircumcised penis, phimosis, tobacco usage. Sexual partners of men with EQ should be assessed evaluated as they are prone to develop preinvasive and invasive cancer of the cervix or anus.
Genital region	Can be pigmented in this area
Nails	Can be pigmented and may mimic melanoma
Inner thigh	Very rare site
Perianal	Very rare slow growing type, affecting both males and females and has a high incidence of recurrence
Nipple	Can present with raised scaly itchy plaques, or as an eczema like lesion with crusting, ulceration and bleeding

Diagnosis

This can be confirmed with history and clinical assessment (remove as per reviewer (by those with the knowledge to do so,) but in many cases a biopsy will be needed. Dermoscopy may also be useful as a diagnostic tool but may not be available in some clinic settings. When assessing lesions, the weighted 7-point checklist can be used to help determine the need for referral. This is as follows: (NICE 2022)

Major features of the lesion (2 points for each)

Change in size

Irregular shape

Irregular colour

Minor features of the lesion (1 point for each)

Largest diameter 7mm or more

Inflammation

Oozing

Change in sensation (including itch)

Differential diagnosis

There are several conditions which share similarity to Bowen's disease, (remove as per review as per reviewer some more common than others). These include: (Stollery 2013)

Superficial basal cell carcinoma: This is often a slow growing solitary lesion. There is usually no pain or itching but there is no response to topical treatments. Although similar to BD, superficial basal cell carcinoma differs as it is more prone to form on the trunk and its edges are slightly raised (Chen et al 2023)

Psoriasis: Can be widespread, but commonly affects the extensor surfaces of the elbows and knees. Plaques are associated with scales which can have a silvery white appearance and well demarcated edges and differs from Bowen's disease in that lesions have a distinctive rich red colour (Willacy 2021).

Actinic keratosis: Lesions are common on the head and hands and the condition has an increased prevalence in older age. Lesions are usually small and are seen as multiple grey, rough areas, rarely larger than 1mm in diameter and differ in appearance as BD lesions tend to be larger. Occasionally they can progress to squamous cell carcinoma (SCC)

Discoid eczema: This is usually bilateral and occurs on the limbs and presents as excoriated patches of affected skin which may be dry or exudative with oozy papules, blisters, and plaques (Oakley 2020). It differs from BD as typical lesions of discoid eczema are coin-shaped red patches of skin which are extremely itchy (British Association for Dermatology 2019)

Treatment and management

There are several treatment options available. Before deciding on the most appropriate course of treatment, consideration will be given to the location of Bowen's disease, the number of lesions present, their size and thickness, and whether the patient has been previously treated for the same condition, and the treatment used and whether there was any benefit or not. Patients age should also be taken in to consideration as the skin on the lower legs in older people tends to be more fragile and may not heal as well, therefore making some options unsuitable (Macmillan 2021).

Treatment choices include: (Macmillan 2021)

No treatment: Conservative measures and monitoring may be appropriate for patients in poor health and with multiple SCC in situ lesions, especially on the lower legs, where there is a greater risk of long-term ulceration after treatment (Sharma et al 2023). This includes a moisturizer (preferably urea based) and skin surveillance, proceeding to biopsy if SCC is suspected. Please remove this.

Chemotherapy cream: Used once or twice daily for around three to four weeks (sometimes longer). These creams include 5-fluorouracil (5-FU) which can cause skin irritation and redness, making the area look worse initially. If recurrence occurs, treatment may need to be repeated.

Immunotherapy cream: Imiquimod (Aldara) can be used and achieves its effect by working with the bodies immune system to attack cancerous cells. Adverse effects include burning and tenderness at the site being treated and this usually occurs at the start of treatment. Adverse effects include flu like symptoms and shivering.

Cryotherapy

This may be appropriate if the area of affected skin is small. Liquid nitrogen is used to freeze the skin and destroy cancer cells. Some patients may find the procedure painful.

Curettage

This done under local anaesthetic and may be used for small areas. Abnormal cells are scraped away using a curette. Healing usually takes a few weeks and leaves a small scar.

Photodynamic therapy

This involves application of a light sensitive chemical to the patch of Bowen's disease, which is activated by a light source directed at the area, hence destroying cancer cells. This treatment option is useful if a large area is affected.

Surgery

May be used if other treatments have failed or if the area affected is deep in to the tissues.

Other less frequently used treatment options are shown in Table 3.

Table 3: Other treatment options for Bowen's disease (Sharma et al 2023)

Treatment	Additional information
Radiotherapy	This is less commonly used but may be an option if the condition is causing symptoms such as pain or if the problem has recurred after earlier treatment. It is not used on areas blow the knee due to problems with skin healing after treatment.
Laser treatment	Used to treat Bowen's disease affecting the genitals or the fingers or may be an option when other treatments have failed.
MOHS micrographic surgery	This is specialist surgery which aims to remove the cancer but leave as much healthy skin tissue as possible. May be an option when conservation of the tissue is important (e.g., around the eyes or the nails)

Combination therapy

Consider combination therapy if monotherapy with 5-fluorouracil (5%), imiquimod (5%), PDT, laser, cryotherapy, or curettage with cautery fails, and surgery (Sharma et al 2023) is not appropriate or is associated with excessive morbidity (Table 2). Combination options are shown in table 4

Table 4: Combination treatment for Bowens disease (Sharma et al 2023)

Cryotherapy followed by imiquimod

Cryotherapy followed by 5-fluorouracil

Imiquimod followed by CO₂ laser

Laser and 5-fluorouracil

Laser and photodynamic therapy

Simple shaving followed by photodynamic therapy

Follow up

It is advisable that patients have a review of after treatment at 3 months. On examination, the presence of any remaining rough scale would suggest that the lesion has not fully responded to treatment and that more is required, whereas, the presence of smooth skin, (sometimes associated with post-inflammatory hyperpigmentation, especially on the lower legs) suggests that the lesion has responded well to treatment and no further follow up is required (PCDS 2023).

Prognosis and complications

After successful treatment there is a risk of recurrence. Regular review and follow up is advisable so that early intervention can be initiated should this occur. It is estimated that there is a 1 in 10 of recurrence (Tidy 2020). Whilst the prognosis is usually favourable there is a small risk of progression to invasive SCC, estimated to occur in around 3 to 5% of cases, and of these one third may metastasise (Kaidas 2023). The development of invasive carcinoma is more common among elderly people and immunocompromised individuals (Palaniappan and Kartikeyan 2022), and risk is estimated to be higher for genital Bowen disease or erythroplasia of Queyrat at 10% (Kaidas 2023).

Conclusion

Bowen's disease is a rare condition considered to be a precursor to SCC. It is more common in women and in those aged over 60 years of age and with fair or pale skin colour. Because of its similarity to several other skin diseases, accurate diagnosis can be challenging for clinicians. It is hoped that this article has given nurses and non-medical prescribers useful information on the condition its risk factors, treatment, and management, with the aim of increasing confidence in recognising this disease and getting earlier treatment for those affected.