Surgical perspective on perianal Bowen's disease: A rare case report

Abstract

Introduction and importance

Bowen's Disease (BD) stands out as a dermatologic entity known for its rarity and diagnostic intricacies. While BD is recognized for its diverse clinical presentations, its occurrence in the perianal region is particularly exceptional. Our case contributes to the limited body of knowledge regarding perianal BD, shedding light on its distinctive characteristics and guiding clinicians in navigating the intricacies associated with this uncommon presentation.

Case presentation

We present the case of a 67-year-old female who sought medical attention for persistent itching in the perianal region. A thorough examination revealed a solitary BD lesion, a notable rarity in this anatomical site. Considering the distinctive characteristics and location, surgical excision was chosen as the preferred treatment strategy. The postoperative course was straightforward, yielding favorable aesthetic outcomes and no recurrence.

Clinical discussion

The clinical discussion explores the unique challenges associated with perianal BD, emphasizing the rarity of its presentation and its potential to mimic other dermatologic conditions. Factors contributing to the diagnosis, including clinical indicators and risk factors, are scrutinized. Furthermore, the discussion delves into the evolving landscape of diagnostic tools and treatment modalities, especially relevant in the context of perianal BD.

Conclusion

This case illuminates the rarity of BD in the perianal region, serving as a valuable addition to the limited body of knowledge on this unusual presentation. By unraveling the complexities associated with perianal BD, this report contributes to a deeper understanding of the disease and provides insights that can guide clinicians in navigating similar cases.

Highlights

• •The report discusses a rare case of Perianal Bowen's Disease, detailing its atypical presentation and surgical treatment.
• •Offering insights for surgeons, the article emphasizes unique features in perianal Bowen's Disease, enhancing understanding of diverse clinical presentations.
• •Navigating complex diagnostic challenges, the report addresses multifactorial causes and risk factors in Bowen's Disease.
• •Highlighting the importance of tailored approaches, the article discusses the choice of surgical excision for effective management in less common areas.
• •Recognizing ongoing advancements, the report underscores evolving diagnostic tools and therapies, promising improved patient care in Bowen's Disease.

1. Introduction

Bowen's disease (BD) refers to an in-situ squamous cell carcinoma (SCC) originating in the epidermis [1,2]. Primary causal factors for BD involve exposure to ultraviolet light, immunosuppression, and infections with Human Papilloma Virus (HPV) [1]. BD frequently occurs in photo-exposed areas of skin, yet it can also affect other areas [2,3]. Histopathology stands as the gold standard diagnostic method for confirming the diagnosis [1,4]. The therapeutic options currently accessible encompass topical chemotherapy, surgical approaches, light-based treatments, and destructive therapies [3,5]. Treatment considerations rely on various factors such as the site, size, immune status, patient's age, aesthetic outcomes, and other relevant factors [1].

Our case report delves into the unique presentation of Bowen's Disease in the perianal region in a 67-year-old female, who underwent surgical excision for treatment. Through this report, we aim to share this uncommon experience with surgeons, highlighting the distinctive features of the presentation and engaging in a discourse on the role of surgery within the spectrum of therapeutic modalities. This work has been reported in line with the SCARE 2023 criteria [6].

2. Case presentation

This concerns a 67-year-old patient with a history of hypertension and well-controlled type 2 diabetes under treatment. She underwent an extended colpo-hysterectomy for cervical cancer via the midline approach 2 years ago. She presented to our outpatient clinic with itching in the perianal region persisting for 5 months, without any other associated functional symptoms for which she has not received prior treatment. Physical examination revealed an erythematous perianal plaque, slightly infiltrated, covered with scales and crusts, measuring 3*2 cm in its largest dimensions. It is located at the 4 o'clock position in the gynecological position (Fig. 1). Rectal examination and examination of the vulvar region are normal. The scalp, oral mucosa, palms, and soles exhibited no abnormalities. The rest of the physical examination is unremarkable. Laboratory results are within normal limits.

Fig. 1.

Preoperative image of the perianal lesion.

The biopsy of the lesion revealed an acantho-papillomatous and hyperkeratotic epidermis. It demonstrates architectural disorganization associated with maturation disturbances. The Malpighian cells exhibit moderately atypical nuclei displaying mitotic figures (Fig. 2). The basement membrane is intact. The dermis is inflammatory without evidence of infiltration. Upon consideration of clinical features and histopathological findings, we established the diagnosis of Bowen's disease.

Fig. 2.

Marked nuclear atypia and mitotic fig. (HE × 40).

In the face of the singular lesion and the accessibility of the site to surgery, a surgical treatment was chosen. She underwent surgical excision under spinal anesthesia in the gynecological position. The excision was performed approximately 1 cm from the lesion and in healthy tissue (Fig. 3). The anal sphincter was spared from excision, and the loss of cutaneous and subcutaneous substance was closed layer by layer, resulting in a good aesthetic outcome (Fig. 4). The postoperative course was uneventful. The patient was discharged on the first postoperative day. The results of the anatomopathological examination confirmed the diagnosis of Bowen's disease. Follow-up of the patient for 2 years showed no functional complaints or recurrence.

Fig. 3.

Image of the operative specimen.

Fig. 4.

Image of the aesthetic result after excision.

3. Discussion

Bowen's Disease (BD), initially documented in 1912 by John Templeton Bowen, is characterized by a squamous cell carcinoma (SCC) in situ [1,7]. However, it took 27 years from the initial discovery for Bowen's Disease (BD) to be documented in the perianal region [1]. Bowen's Disease (BD) is an uncommon lesion characterized by a gradual growth pattern and a tendency to maintain the integrity of the basal membrane of the epidermis [2]. While it has the potential to affect any part of the human body, Bowen's Disease (BD) typically manifests in sun-exposed areas [1,8]. Nevertheless, non-exposed regions may also be involved, as exemplified by its presentation in the anogenital region, as observed in our case [2,3]. It is more frequently observed in females within the age range of 20 to 45 years [9].

The etiology of BD is complex, involving multiple factors [1,4]. Risk factors encompass Caucasian race, fair skin, individuals sensitive to sunlight, and an elevation in overall occupational and recreational sun exposure [1,4]. Prolonged exposure to ultraviolet light radiation leads to DNA damage and immunosuppression, facilitating the clonal expansion of underlying p53 mutations [1]. Bowen's Disease (BD) resulting from arsenic exposure is recognized to develop several decades later [1,4]. Previous studies have indicated that HPV infection could be a potential risk factor for Bowen's disease, as in the case of our patient with a history of treated cervical cancer [1,9].

The morphological characteristics of Bowen's Disease (BD) vary depending on the age of the lesion, the site of origin, and the extent of keratinization [1]. In areas where keratinization is lacking, the lesions appear erythematous and velvety [1]. However, this erythema is concealed by scaling in lesions that occur over keratinized epithelium [1,8]. Typically, lesions are solitary, with multiple occurrences observed in 10 %–20 % of affected individuals [1,8]. Classical lesions of Bowen's Disease (BD) are usually asymptomatic, but larger lesions can be pruritic [1]. BD commonly manifests as a slowly growing, well-demarcated, erythematous, scaly patch or plaque [1]. Perianal Bowen's Disease is more frequently observed in women, and individuals with this condition may experience mild symptoms like itching or a burning sensation, as evident in our patient [1,3]. Within the anogenital region, the average size at the time of biopsy is approximately 1.3 cm² [1]. The risk of invasive carcinoma in this condition ranges from 2 % to 6 % [1,4].

The progression to invasive squamous cell carcinoma (SCC) is attributed to the destruction of the basement membrane facilitated by metalloproteinases [1]. This advancement to invasive carcinomas is more prevalent in elderly individuals and those with compromised immune systems [9]. Clinical indicators suggestive of malignant transformation include the presence of ulceration, bleeding, and the formation of nodules [1].

The accuracy of clinical diagnosis for Bowen's Disease (BD) prior to pathological examination is relatively low [4,5]. BD is most misdiagnosed as Bowenoid papulosis, followed by actinic keratosis, basal cell carcinoma keratosis, seborrheic keratosis, and pigmented nevus [1]. Dermoscopy, a non-invasive tool, is progressively employed in the auxiliary diagnosis of BD [8]. Ultrasound bio microscopy (UBM) and high-frequency ultrasound (HFUS) also hold promise as diagnostic tools for BD. However, a skin biopsy is frequently required to achieve an accurate diagnosis of BD [1]. The epidermis exhibits hyperkeratosis and parakeratosis, along with pronounced acanthosis characterized by the elongation and thickening of rete ridges [1,3]. Keratinocytes display atypia that extends throughout the entire epidermis without breaching the dermo-epidermal junction. In Bowen's Disease (BD), keratinocytes showcase heightened mitotic activity, pleomorphism, and notably large nuclei [1,3]. The concurrent loss of maturity and polarity imparts an appearance to the epidermis described as “windblown” [1,3].

The choice of treatment depends on various factors including tumor size, location, thickness, number of lesions, patient's age, immune status, comorbidities, concurrent medication use, compliance, aesthetic outcomes, availability of equipment, and the preferences of both the patient and the clinician, considering the clinician's expertise [1,4]. Because BD commonly occurs in old individuals, frequently located in regions with poor wound healing, noninvasive treatments are preferred [1,4,7]. Treatment modalities for Bowen's Disease (BD) encompass various approaches, including topical therapies (such as 5-fluorouracil (5-FU) and imiquimod), cryotherapy (cryosurgery), curettage with cautery, photodynamic therapy (PDT), standard surgical excision, Mohs micrographic surgery, laser procedures (including CO2 laser and non-ablative neodymium), radiotherapy, systemic treatments, and combination therapy [1,4,5]. In our case, surgical excision was chosen due to the solitary lesion in an unexposed area, with good potential for healing, and its location spared the anal sphincter. Additionally, several non-invasive modalities are not available at our center. Earlier studies indicated that achieving a surgical margin of 5 mm can result in a complete clearance rate of up to 94.40 % [3,10].

In conclusion, Bowen's Disease poses a diagnostic challenge with varied clinical and morphological presentations [1,3]. Treatment decisions require a nuanced consideration of factors, and while surgical excision remains a common approach, advancements in non-invasive diagnostics and therapies are emerging [3]. A comprehensive and tailored approach, integrating evolving diagnostic tools and therapeutic modalities, is crucial for effective management of Bowen's Disease [1].

4. Conclusion

In conclusion, Bowen's Disease (BD) presents a complex clinical landscape, with diverse manifestations and potential challenges in accurate diagnosis [1,8]. The multifactorial etiology, involving environmental and viral factors, underscores the need for a comprehensive understanding of patient-specific risk factors [4]. Treatment decisions must be individualized, considering factors such as lesion characteristics, patient demographics, and available resources [1,2]. While surgical excision remains a cornerstone, evolving diagnostic tools like dermoscopy and ultrasound hold promise for improved accuracy [3]. Moreover, the expanding array of therapeutic modalities, including non-invasive options, demands a nuanced and patient-centered approach [1,3]. As we navigate the complexities of BD, ongoing research and clinical advancements are essential to refine our diagnostic capabilities and optimize treatment strategies, ultimately enhancing the outcomes and quality of life for individuals affected by this dermatological condition [1,3,4].

Patient consent

Written informed consent was obtained from the patient for the publication of this case report and its accompanying images. A copy of the written consent is available for the Editor-in-Chief of this journal to review upon request.

Grant information

The author(s) declared that no grants were involved in supporting this work.

Declaration of generative AI in scientific writing

AI tools were not used for the elaboration of the manuscript.

Ethical approval

Ethical approval is not applicable/waived at our institution.

Funding

This research did not receive funding from any specific grant provided by public, commercial, or not-for-profit organizations.

Author contribution

Radhwen Zarg El Ayoun and Ahmed Omry contributed to manuscript writing and editing, and data collection;

Hajer Bahi and Amel Changuel contributed to data analysis;

Med Hedi Mannai and Med Bachir Khalifa contributed to conceptualization and supervision;

All authors have read and approved the final manuscript.

Guarantor

Dr. Ahmed Omry

Research registration number

N/A.

Declaration of competing interest

No conflicts of interest.