Dermatology: Practical and Conceptual Case Report | Dermatol Pract Concept 2016;6(2):2 5 DERMATOLOGY PRACTICAL & CONCEPTUAL www.derm101.com Introduction Bowen’s disease (BD) is an in situ variant of cutaneous squa- mous cell carcinoma (SCC). Etiological factors for BD include ultraviolet radiation (solar, iatrogenic and sunbeds) [1,2], radiotherapy, carcinogens (arsenic), immunosuppression [3,4], and infection with human papillomavirus [5-7]. Histo- pathologically it is characterized by abnormal and pleomor- phic keratinocytes that in the precursor stages involve only the lower part of the epidermis and, in time, with progression to BD, the full thickness of it. Dermatoscopic criteria have been described for pigmented and non-pigmented BD, actinic keratosis (AK) and superficial forms of SCC [8-10]. A recent study has focused on a progression model of actinic keratosis and intraepidermal carcinomas to invasive carcinoma but was limited to facial cases [10]. Case report A 44-year-old male presented with a 10-year history of a 35x25 cm erythematous hyperkeratotic, crusted, and ulcer- Development of poorly differentiated invasive squamous cell carcinoma in giant Bowen’s disease: a case report with dermatoscopy Bengu Nisa Akay1, Aysenur Maden1, Oguzhan Kocak2, Seher Bostanci1, Ayşe Boyvat1, Pelin Kocyigit1, Aylin Okcu Heper3 1 Ankara University Faculty of Medicine, Department of Dermatology, Ankara, Turkey 2 Kutahya Evliya Celebi State Hospital, Department of Dermatology, Kutahya, Turkey 3 Ankara University Faculty of Medicine, Department of Pathology, Ankara, Turkey Key words: Bowen’s disease squamous cell carcinoma, dermatoscopy, dermoscopy Citation: Akay BN, Maden A, Kocak O, Bostanci S, Boyvat A, Kocyigit P, Okcu Heper A. Development of poorly differentiated invasive squamous cell carcinoma in giant Bowen’s disease: a case report with dermatoscopy. Dermatol Pract Concept 2016;6(2):2. doi: 10.5826/ dpc.0602a02 Received: August 24, 2015; Accepted: January 12, 2016; Published: April 30, 2016 Copyright: ©2016 Akay et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Funding: None. Competing interests: The authors have no conflicts of interest to disclose. All authors have contributed significantly to this publication. Corresponding author: Aysenur Maden, MD, Ankara University Faculty of Medicine, Department of Dermatology, Ankara, Turkey. Tel. +90 (312) 5082231; Fax. +90 (312) 3108636. E-mail: abotsali@hotmail.com Bowen’s disease (BD) is an in situ form of squamous cell carcinoma (SCC), often occurring in the chronically UV-damaged skin of elderly people. The risk of progression of BD to invasive SCC var- ies between 3% and 5%, and one-third of invasive tumors may metastasize. Herein we discuss the dermatoscopic findings of a case of giant Bowen’s disease, which progressed to poorly differentiated invasive SCC. ABSTRACT mailto:abotsali@hotmail.com 6 Case Report | Dermatol Pract Concept 2016;6(2):2 whole lesion. Multiple skin biopsies from the peripheral and central parts were taken. Central parts were histopathologi- cally consistent with poorly differentiated invasive SCC, while peripheral parts were compatible with BD (Figure 4). There were no cytopathic changes compatible with human papil- lomavirus infection. The patient’s arsenic level was found to be elevated on blood analysis. Thoracic-abdominal-pelvic computed tomography did not show any metastasis. The lesion was totally excised with safe surgical margins and split-thickness skin grafting was performed in the plastic surgery department. Discussion Bowen’s disease (BD) can be considered a low-grade form of SCC, with the majority of studies reporting the risk of pro- gression to SCC at 3-5% [12,13]. Despite the low incidence of malignant progression, BD has significant consequences since ated plaque on his abdominal region (Figure 1). The patient did not report previous history of trauma, immunosuppres- sion or radiotherapy to the lesion site. Dermatoscopic examination of the central part of the lesion with DermLite Pro HR (3Gen, LLC, San Juan Capist- rano, CA, USA) mounted on an iPhone 5S®, revealed white circles and a vascular pattern, observed as large coiled ves- sels in linear arrangement intermingled with ulcerations and adherent fibrin crusts (Figure 2). The peripheral parts showed a linear arrangement of gray dots and coiled vessels without prominent white circles and ulceration (Figure 3). The diameter of the coiled vessels on the central part was larger in size than seen in the periphery. In addition, red and white structureless areas were distributed throughout the Figure 1. Clinical image of the patient showed a 35x25 cm ery- thematous hyperkeratotic, crusted, and ulcerated plaque on the ab- dominal region. [Copyright: ©2016 Akay et al.] Figure 2. Dermatoscopy of the central region. (A) Linear arrange- ment of coiled vessels becoming convoluted and larger in size (blue arrows) intermingled with ulcer- ation and adherent fibrin crusts. (B) White circles (blue arrows). [Copyright: ©2016 Akay et al.] Figure 3. Dermatoscopy the peripheral region. (A, B) White and pink structureless areas, white lines (green circle) and a linear ar- rangement of gray dots and coiled vessels (blue arrows). No ulcer- ation or white circles are seen. [Copyright: ©2016 Akay et al.] Figure 4. (A) Histopathology of the peripheral part which is con- sistent with BD: An acanthotic epidermis shows full-thickness epidermal replacement by crowd- ed keratinocytes that demonstrate disordered dyspolarity, lack of maturation, atypical mitotic fig- ures and nuclear pleomorphism with hyperchromasia. A band-like lymphocytic infiltrate is appar- ent in the papillary dermis. (B) Histopathology of the central re- gion shows invasive part of tumor which extends as invasive broad tongues into the dermis. This part of the tumor composed of pleo- morphic atypical epithelioid cells with high nucleocytoplasmic ratio and frequent mitosis. Squamous differentiation features, such as squamous pearls or single cell kera- tinization, are not obvious. Foci of necrosis can be noted at the right part of the figure. [Copyright: ©2016 Akay et al.] Case Report | Dermatol Pract Concept 2016;6(2):2 7 in the infundibular epidermis, which in effect is invasion of adnexa and can be a feature of well-differentiated SCC. The white circles observed in our case were poorly formed and sparse which may be associated with the poor differentiation status. In the present case, the periphery of the lesion cor- responding to BD showed gray to brownish dots and coiled vessels arranged in lines, while the central part corresponding to invasive SCC revealed white circles, large coiled vessels arranged in lines and ulceration. The diameter of the coiled vessels on the central part was larger in size than seen on the periphery, similar to the progression model of Zalaudek et al [10]. In addition, white circles and ulceration were not observed on the parts where there was BD. Rosendahl et al., found the positive predictive value of white circles as 92% in SCC when compared with BD [14]. In conclusion, we have presented the first description of dermatoscopic findings of poorly differentiated invasive SCC developing on a huge BD. Dermatoscopy may help to differentiate intraepidermal carcinoma from invasive SCC, especially when confronted with white circles and coiled vessels with large diameter. This in turn may help clinicians not only to diagnose in situ or invasive lesions but also to improve the selection of lesions requiring biopsy or excision for definitive histopathologic diagnosis. References 1. Kossard S, Rosen R. Cutaneous Bowen’s disease. An analysis of 1001 cases according to age, sex, and site. J Am Acad Dermatol 1992; 27:406–10. PMID: 1401276. 2. Reizner GT, Chuang TY, Elpern DJ, Stone JL, Farmer ER. Bowen’s disease (squamous cell carcinoma in situ) in Kauai, Hawaii. A population-based incidence report. J Am Acad Dermatol 1994; 31:596–600. PMID: 8089285 3. Drake AL, Walling HW. Variations in presentation of squamous cell carcinoma in situ (Bowen’s disease) in immunocompromised patients. J Am Acad Dermatol 2008; 59:68–71. PMID: 18440666. DOI: 10.1016/j.jaad.2008.03.028 4. Moloney FJ, Comber H, O’Lorcain P, et al. A population-based study of skin cancer incidence and prevalence in renal transplant recipients. Br J Dermatol 2006; 154:498–504. PMID: 16445782. DOI: 10.1111/j.1365-2133.2005.07021.x 5. Kettler AH, Rutledge M, Tschen JA, Buffone G. Detection of human papillomavirus in nongenital Bowen’s disease by in situ DNA hybridization. Arch Dermatol 1990; 126:777–81. PMID: 2161202. DOI: 10.1001/archderm.1990.01670300077011 6. Collina G, Rossi E, Bettelli S et al. Detection of human papillo- mavirus in extragenital Bowen’s disease using in situ hybridiza- tion and polymerase chain reaction. Am J Dermatopathol 1995; 17:236–41. PMID: 8599431. 7. Hama N, Ohtsuka T, Yamazaki S. Detection of mucosal human papilloma virus DNA in bowenoid papulosis, Bowen’s disease and squamous cell carcinoma of the skin. J Dermatol 2006; 33:331–7. PMID: 16700665. DOI: 10.1111/j.1346-8138.2006.00078.x 8. Zalaudek I, Argenziano G, Leinweber B, et al. Dermoscopy of Bowen’s disease. Br J Dermatol 2004; 150:1112–6. PMID: 15214896. DOI: 10.1111/j.1365-2133.2004.05924.x approximately 20% of the tumors that develop into SCC will eventually become metastatic [12]. Therefore, patients with BD should be diagnosed and treated as early as possible. Dermatoscopy is considered a helpful and non-invasive tool for increasing the diagnostic accuracy of BD. In 2004, Zalaudek et al., described dermatoscopic features of BD in 21 cases [8]. They observed a particular type of vascular pattern in BD, named glomerular vessels, a variant form of dotted vessels that are large in size and often grouped and regularly arranged in clusters, mimicking the glomerular apparatus of the kidney. They emphasized that the “glomerular” vessels together with a scaly surface and, in cases of pigmented BD, the additional presence of pigmented small globules and/or homogeneous pigmentation, represent specific dermatoscopic criteria for the diagnosis of BD. In their study glomerular vessels were observed in 100% of the non-pigmented and 80% of the pigmented BD. This special type of tortuous cap- illary was histopathologically correlated to a convolution of grouped, frequently dilated capillaries in the dermal papillae and papillary dermis [8]. In 2010, Cameron et al., described dermatoscopic findings of pigmented BD, and they found that the linear arrangement of brown and/or gray dots and/or coiled vessels were specific clues to pigmented BD [9]. Pink, white or skin-colored structureless areas were found to be the most common finding in their study. Histopathologically, the brown to gray dots in BD may correspond to the presence of melanophages arranged in clusters and diffusely situated in the superficial dermis, and/or to a slightly increased number of pigmented keratinocytes in the basal layer and less fre- quently in suprabasal locations. Dermatoscopy of SCC has been the subject of very recent studies. Zalaudek et al., proposed a progression model of facial AK developing into BD and invasive SCC based on dermato- scopic findings [10]. In this model, those AK that progress to increasing atypia tend to display vessels around follicles that become dotted (or coiled on higher magnification), then as the lesion develops into SCC in situ the dotted/coiled vessels appear to enlarge, become more convoluted and clustered, and the follicles in that area appear to miniaturize and dis- appear eventually forming the discrete whitish, opaque scaly areas. With progression of BD to invasive SCC, looped and/ or linear irregular vessels will appear, and a central mass of keratin forms and ulceration may occur [10]. White circles, keratin, and blood spots were the strongest features associ- ated with the diagnosis of SCC in a recent study by Rosendahl et al [14]. Especially, white circles were useful clues to dif- ferentiate SCC and keratoacanthoma from other raised non- pigmented skin lesions by dermatoscopy. White circle is a new dermatoscopic criterion, represented by white circles centered around a dilated infundibulum filled with a keratin plug that is visible as a yellow or an orange clod on dermatoscopy. White circles correspond to acanthosis and hypergranulosis 8 Case Report | Dermatol Pract Concept 2016;6(2):2 12. Peterka ES, Lynch FW, Goltz RW. An association between Bowen’s disease and internal cancer. Arch Dermatol 1961; 84:623–9. PMID: 14485715. DOI: 10.1001/archderm.1961.01580160087015 13. Kao GF. Carcinoma arising in Bowen’s disease. Arch Derma- tol 1986; 122:1124-26. PMID: 3767398. DOI: 10.1001/arch- derm.1986.01660220042010 14. Rosendahl C, Cameron A, Argenziano G, et al. Dermoscopy of squamous cell carcinoma and keratoacanthoma. Arch Dermatol 2012; 148:1386-92. PMID: 22986634. DOI: 10.1001/archder- matol.2012.2974 9. Cameron A, Rosendahl C, Tschandl P, et al. Dermatoscopy of pigmented Bowen’s disease. J Am Acad Dermatol 2010; 62:597- 604. PMID: 20079953. DOI: 10.1016/j.jaad.2009.06.008 10. Zalaudek I, Giacomel J, Schhmid K, et al. Dermatoscopy of facial actinic keratosis, intraepidermal carcinoma and invasive squamous cell carcinoma: A progression model. J Am Acad Dermatol 2012; 66:589-97. PMID: 21839538. DOI: 10.1016/j. jaad.2011.02.011 11. Cox NH, Eedy DJ, Morton CA. Guidelines for management of Bowen’s disease: 2006 update. Br J Dermatol 2007; 156:11-21. PMID: 17199561. DOI: 10.1111/j.1365-2133.2006.07610.x