Introduction: Bowen’s disease is a squamous cell carcinoma in situ, the most common malignancy of the nail unit. Presenting more frequently in the fingernails, common risk factors include ionizing radiation, oral exposure to arsenic or pesticides, dyskeratosis congenita, and quite commonly diverse subtypes of HPV. We report the first case of multiple periungual pigmented Bowen’s disease in a pediatric patient. Case Presentation: A healthy 13-year-old boy presented with a 9-month history of a pigmented erythematous patch on the proximal nail fold of his 3rd right finger without associated symptoms. A punch biopsy was taken, and the diagnosis of Bowen’s disease was made. The patient received photodynamic therapy and three cycles of imiquimod without response, and two new lesions appeared on the first and second right fingers. Surgical removal was performed on all lesions. A polymerase chain reaction detected an HPV type 16. Discussion/Conclusion: Multiple periungual Bowen’s disease is rare, with the most frequent risk factors being HPV infection and chronic immunosuppression. Less than 10% of the cases present as longitudinal melanonychia. To date, there are no previous reports of multiple pigmented periungual Bowen’s disease. HPV-induced Bowen’s disease is usually present in adults aged between 22 and 89 years as persistent verrucae. In this case, koilocytosis and the fact that all lesions appeared on the right hand are suggestive of HPV infection.

Established Facts

  • Multiple periungual Bowen’s disease is rare, with the most frequent risk factors being HPV infection and chronic immunosuppression.

  • Less than 10% of the cases present as longitudinal melanonychia.

Novel Insights

  • There are no previous reports of multiple pigmented periungual Bowen’s disease.

Bowen’s disease is a squamous cell carcinoma in situ, the most common malignancy of the nail unit. Presenting more frequently in the fingernails, clinical features include onycholysis, hyperkeratosis, paronychia, ulceration, nail plate destruction, leukonychia, erythronychia, and longitudinal melanonychia. Common risk factors include ionizing radiation, oral exposure to arsenic or pesticides, dyskeratosis congenita, and quite commonly diverse subtypes of HPV [1]. While rare, multiple non-pigmented periungual Bowen’s disease and multiple pigmented Bowen’s disease elsewhere in the body have been reported. We report the first case of multiple periungual pigmented Bowen’s disease in a pediatric patient.

A healthy 13-year-old boy presented with a 9-month history of a pigmented erythematous patch on the proximal nail fold of his 3rd right finger without associated symptoms (Fig. 1a). A punch biopsy was taken, showing an acanthotic epidermis with atypical keratinocytes, with large, pleomorphic, hyperchromatic nuclei (Fig. 2). With these findings, the diagnosis of Bowen’s disease was made. The patient received photodynamic therapy and three cycles of imiquimod without response and developed longitudinal melanonychia over the course of 9 months, besides spread of the initial lesion (Fig. 1b). In the same time lapse, two new lesions appeared on the first and second right fingers (Fig. 3). Removal of the nail unit and skin grafting of the 3rd right finger were performed, and wide excision with secondary intention healing of the lesions of the 1st and 2nd right fingers (proximal nail fold). The biopsy of all lesions showed the same changes, along with koilocytosis. A polymerase chain reaction detected an HPV type 16. During the first clinical examination, concomitant wart lesion was observed in the genital area. The patient has been followed for 6 years with no recurrence.

Fig. 1.

a A pigmented erythematous patch in the proximal nail fold of the right middle finger. b Clinical presentation 20 months after showing lack of improvement and longitudinal melanonychia and nail dystrophy.

Fig. 1.

a A pigmented erythematous patch in the proximal nail fold of the right middle finger. b Clinical presentation 20 months after showing lack of improvement and longitudinal melanonychia and nail dystrophy.

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Fig. 2.

Acanthotic epidermis with dyskeratotic and atypical keratinocytes in all layers showing large, hyperchromatic, pleomorphic nuclei. Plenty melanin granules are also seen. H&E stain, ×10.

Fig. 2.

Acanthotic epidermis with dyskeratotic and atypical keratinocytes in all layers showing large, hyperchromatic, pleomorphic nuclei. Plenty melanin granules are also seen. H&E stain, ×10.

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Fig. 3.

Additional lesions on the first and second right finger.

Fig. 3.

Additional lesions on the first and second right finger.

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Multiple periungual Bowen’s disease is rare, with the most frequent risk factors being HPV infection and chronic immunosuppression [2]. Less than 10% of the cases present as longitudinal melanonychia [3]. To date, there are no previous reports of multiple pigmented periungual Bowen’s disease. Given the lack of treatment guidelines, proposed therapeutic options include imiquimod, photodynamic therapy, wide-margin excision, Mohs surgery, and radiotherapy [3, 4].

HPV-induced Bowen’s disease is usually present in adults aged between 22 and 89 years as persistent verrucae [5]. Several HPV subtypes have been identified, with type 16 being present in 74% of the cases [1]. In this case, koilocytosis and the fact that all lesions appeared on the right hand are suggestive of HPV infection.

This case presented multiple pigmented periungual lesions and longitudinal melanonychia. Continuous surveillance is warranted to detect new or recurrent lesions.

This research complies with the guidelines for human studies welfare regulations and was conducted ethically in accordance with the World Medical Association Declaration of Helsinki. Written informed consent was obtained from the parent of the patient for publication of this case report and any accompanying images. Ethical approval is not required for this study in accordance with local or national guidelines.

The authors have no conflicts of interest to disclose.

No funding was received for the preparation of this manuscript.

Guillermo Antonio Guerrero-González: writing of the original draft, writing, and bibliographic review. Nilton Gioia Di Chiacchio, Francisco Macedo Paschoal, and Nilton Di Chiacchio: idea conception, article editing, and revision of final manuscript. All authors agreed to submit to the current journal, gave final approval of the version to be published, and accepted to be accountable for all aspects of the work.

The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request. Data are not publicly available due to ethical reasons. Further inquiries can be directed to the corresponding author.

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