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| CASE REPORT |
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| Year : 2020 | Volume
: 7
| Issue : 2 | Page : 102-105 |
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Pigmented Bowen’s disease over photo protected site in an Indian male: a rare case report
Ishan Agrawal, Debasmita Behera, Ipsita Debata, Maitreyee Panda
Department of DVL, Institute of Medical Sciences and SUM Hospital, S‘O’A University, Bhubaneswar, Odisha, India
| Date of Submission | 04-Jan-2020 |
| Date of Decision | 26-Apr-2020 |
| Date of Acceptance | 11-Jul-2020 |
| Date of Web Publication | 03-Dec-2020 |
Correspondence Address:
MD Ipsita Debata
Associate Professor, Department of DVL, IMS & SUM Hospital, Kalinga Nagar, Bhubaneswar, Odisha 751003
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/Pigmentinternational.Pigmentinternational_
Bowen’s disease is a relatively common, yet under-recognized form of squamous cell carcinoma in situ. Pigmented BD (Pigmented BD) is a rare subtype of BD characterized by a hyperpigmented appearance, accounting for only 2% of all reported cases of BD. A 56 year old male presented with a scaly violaceous plaque over the scrotal skin with a dermoscopic picture of structure-less areas and bluish-brown dots, black globules with diffuse light violaceous pigmentation. Biopsy showed full thickness keratinocyte atypia with mitosis at all levels and pigment incontinence. We report this case because of the atypical presentation of BD, supported by dermoscopy and histopathology findings. The rarity of such a clinical presentation signifies the need of a high index of clinical suspicion in such cases.
Keywords: Dermoscopy of skin malignancy, histopathology, immunohistochemistry, pigmented BD, squamous cell carcinoma
How to cite this article:
Agrawal I, Behera D, Debata I, Panda M. Pigmented Bowen’s disease over photo protected site in an Indian male: a rare case report. Pigment Int 2020;7:102-5 |
| Introduction | |  |
Bowen’s disease is a relatively common, yet under-recognized form of squamous cell carcinoma in situ. It usually presents as a well demarcated, slow growing erythematous patch or plaque with a scaly or crusted surface mostly over the sun-exposed part of the body. There is a peak incidence in the sixth to eighth decade of life. It is very common in the Caucasians with an incidence of about 1.42/1000.[1] Pigmented BD is a rare subtype accounting for only 2% of all reported BD cases.[2],[3] We are reporting a case of pigmented variant of BD in type IV Fitzpatrick skin type with a challenging diagnosis. Dermoscopy and histopathological study helped in arriving at the diagnosis. Extensive literature search revealed very few case reports of this rare solitary pigmented variant of BD in the Indian population.
Case Report
A 56 yr old, non-smoker male presented to the dermatology outpatient department with complaints of a localized, raised itchy lesion on the lateral aspect of left scrotal skin. The lesion showed gradual increase in size over the last 3 months. On clinical examination, there was a solitary sharply demarcated, scaly hyperpigmented to violaceous plaque, of size 3 cm x 2 cm, on the lateral side of the left scrotum [Figure 1]. It was non-tender and there was no local rise of temperature. Regional lymph nodes were non-palpable. Patient denied any history of trauma, radiation exposure or any chemical application over the affected area prior to the appearance of the lesion. Patient had no evidence of pre-existing cutaneous neoplasia. All the routine laboratory investigations were within normal limit. | Figure 1 Single well-defined hyperpigmented to violacous plaque of size 3 × 2 cm associated with scaling.
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Lesion was examined using a dermoscope DermLite DL3 (3Gen, Dana point, CA,USA) with 10X magnification equipped with Iphone 7 in non-polarised mode without any interface medium. On dermoscopy, we saw an erythematous to violaceous scaly plaque with sharply defined edge, focal hypopigmented structureless areas, patchy area of homogenous bluish-brown dots, red dots, black globules with bluish hue and few comedo-like openings [Figure 2]. The dermoscopic findings gave a hint of malignant findings in favour of BD. | Figure 2 Well-defined violaceous plaque with multiple structureless areas (red arrow), bluish-brown dots (green arrow), black globules (blue arrow), red dots arranged in linear fashion (yellow arrow), comedo-like opening (black arrow) and bluish-white veil at periphery of plaque (white arrow) (10x, non-polarised, no interface medium).
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Lesional biopsy with a 4 mm punch was done and sent for histopathological evaluation with the differential diagnoses of superficial spreading melanoma, extra-mammary Paget’s disease, BD.
Histopathological examination of the tissue specimen showed full thickness keratinocyte atypia with mitosis at all levels, cells showed loss of polarity with dyskeratosis and occasional multinucleated keratinocytes. The red dots and brown globules seen on dermoscopy were reflected by the histopathological presence of blood vessels in superficial dermis and prominent pigment incontinence, respectively. Additionally, there was presence of acute and chronic infiltrate around dermo-epidermal junction. Histopathological study confirmed it to be BD of pigmented variant [Figure 3] and [Figure 4]. | Figure 3 Full epidermal thickness showing keratinocyte atypia (blue arrow), prominent melanin incontinence (yellow arrow) and dilated blood vessels (green arrow) (H and E staining, 40x).
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The patient was referred to the surgical oncology department where the excisional biopsy was done and subsequent histopathology and immunohistochemistry with p63 positivity, further established the diagnosis.
| Discussion | | |
BD is an in-situ squamous cell carcinoma with a characteristic slow-growing erythematous, non-pigmented, sharply demarcated, scaly asymptomatic plaque. Anatomical site of disease gives a clue about the etiological factors which include chronic exposure to UV radiation, trauma, long-standing exposure to arsenic, immunosuppressed states and viral infection.
Pigmented BD is a distinct variant uncommonly found over the anogenital region of young and immunosuppressed adults. The pathogenesis behind pigmented nature of disease is melanocyte proliferation and melanin production due to the cytokines secreted by neoplastic cells. There is presence of abundant melanin pigment in the cytoplasm of atypical keratinocytes, primarily in the basal layer due to increased function and size of melanocytes.[4]
Pigmented BD presents as a dark pigmented plaque with either verrucous or hyperkeratotic surface. Over anogenital area, it is usually caused by high risk strain of Human Papilloma Virus such as HPV 16. Though in our patient HPV association could not be ruled out because of lack of provision for virological study.[5]
In the pigmented form of BD, we see dermoscopic patterns with a linear arrangement of brown dots, coiled vessels, brown-black globules with diffuse grey to brown pigmentation.[4],[6],[7] These amorphous brownish (epidermal melanin) or greyish pigment (dermal melanin) structures are most sensitive on dermoscopy. The aforementioned findings of brown dots, coiled or dotted vessels, which maybe present in linear or occasionally glomerular configuration are highly specific.[8]
Certain non-specific dermoscopic findings include comedo like openings, well-defined edges, structures in digital printing form, which are also seen in seborrheic keratosis.[8] Our case showed sensitive and specific findings like patchy homogenous bluish-brown dots, black globules with diffuse light violaceous hue, also, we noted nonspecific features as comedo-like openings and sharp definition of the lesion. Additionally, there is a blue-white veil and focal hypopigmented structureless (due to regression of structures like nets, globules or pigmentation) areas which histopathologically suggest fibrosis.[9]
On histopathology, presence of full thickness keratinocyte atypia, with mitosis, and loss of polarity in keratinocytes, signifies SCC in-situ. Additionally, finding of pigment incontinence indicates a pigmented variant.
Morphological differentials of BD include actinic keratosis, solar lentigo, seborrheic keratosis, melanocytic nevus, pigmented BCC, Bowenoid papulosis and melanoma.[4]
IHC emphasizes on BD as p63 is positive in neoplastic cells. p63 is a member of the p53 gene family. It regulates the proliferation and differentiation of squamous cells and oncogenesis.[10],[11] These characteristics of p63 differentiate the diagnosis of SCC in-situ (BD) from Adenocarcinoma in-situ (Paget’s disease).
| Conclusion | | |
To conclude, clinical suspicion is required to diagnose atypical presentations of BD such as in this case. The presentation in our patient was unusual due to the pigmented variant, lesional location over a sun-protected area, in an immunocompetent male in Indian type IV skin. Pigmented BD should be a differential diagnosis of any long-standing pigmented skin lesion regardless of its site as exemplified by this case. Possibility of a cutaneous malignancy, however remote, should always be considered and ruled out with a skin biopsy.[12] To the best of our knowledge, this is a rare presentation of a pigmented variant of BD in Indian skin type (Type IV Fitzpatrick skin type), and has rarely been reported till date in the present literature.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest
| References | | |
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| 2. | Ragi G, Turner MS, Klein LE, Stoll Jr HL. Pigmented Bowen’s disease and review of 420 Bowen’s disease lesions. J Dermatol Surg Oncol 1988;14:765-9. |
| 3. | Cameron A, Rosendahl C, Tschandl P, Riedl E, Kittler H. Dermatoscopy of pigmented Bowen’s disease. J Am Acad Dermatol 2010;62:597-604. |
| 4. | Ishioka P, Yamada S, Michalany NS, Hirata SH. Dermoscopy of BD: pigmented variant on the penis. An Bras Dermatol 2012;87:482-4. |
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| 8. | Mota AN, Piñeiro-Maceira J, Alves Mde F, Tarazona MJ. Pigmented Bowen’sdisease. An Bras Dermatol 2014;89:825-7 |
| 9. | Bassoli S, Borsari S, Ferrari C et al. Grey-blue regression in melanoma in situ-evaluation on 111 cases. J Skin Cancer. 2011;2011:180980- |
| 10. | lkawa S, Nakagawara A, Ikawa Y. p53 family genes: structural comparison, expression and mutation. Cell Death Differ 1999;6:1154-61. |
| 11. | Mills AA. p63: oncogene or tumor suppressor? Curr Opin Genet Dev 2006 16:38-44. |
| 12. | Verma SB. BD in an Indian female. Clinical and Experimental Dermatology 2009;34:e248-e249. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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