Dermatology: Practical and Conceptual 244 Letter | Dermatol Pract Concept 2018;8(3):18 DERMATOLOGY PRACTICAL & CONCEPTUAL www.derm101.com Giant Clear Cell Acanthoma with Dermatoscopic White Lines Omid Zargari1, Seyyede Zeinab Azimi2, Siamak Geranmayeh3 1 Dermatology, Dana Clinic, Rasht, Iran 2 Department of Dermatology, Guilan University of Medical Sciences, Rasht, Iran 3 Sina Pathobiology Laboratory, Rasht, Iran Key words: clear cell acanthoma, dermatoscopy, epidermal tumor Citation: Zargari O, Azimi SZ, Geranmayeh S. Giant clear cell acanthoma with dermatoscopic white lines. Dermatol Pract Concept. 2018;8(3):244-246. DOI: https://doi.org/10.5826/dpc.0803a18 Received: December 9, 2017; Accepted: February 2, 2018; Published: July 31, 2018 Copyright: ©2018 Zargari 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: Seyyede Zeinab Azimi, MD, Department of Dermatology, Guilan University of Medical Sciences, Razi Hospital, Rasht, Iran, 41448. Email: sz.azimi@yahoo.com. Introduction Clear cell acanthoma (CCA) was first described by Degos in 1962 [1] and has been referred to as Degos’ acanthoma. It is a benign epidermal tumor that commonly emerges as a soli- tary dome-shaped lesion on the legs with no gender predilec- tion [1]. However, eruptive forms and involvements of other areas such as inguinal region, scrotum, vermilion mucosa, scalp, palm, nipple, and hallux have been reported [2,3]. Case Presentation A 73-year-old Caucasian man presented with an asymptom- atic strawberry-like plaque on his right ankle, reportedly present for several years. The plaque was approximately 4 cm in diameter, colored red and purple, and surrounded by a fine collarette of scale (Figure 1). There was no history of pigmented skin changes or melanoma among his family members. Past medical history was notable for ischemic heart disease, diabetes mellitus, and hypertension. His regu- lar medications included aspirin, warfarin, metformin, and atorvastatin. Dermatoscopic examination revealed a variegated red/ purple-colored lesion with curved white lines and with dot vessels in a serpiginous (“string of pearls”) pattern (Fig- ure 2). There were no colors to indicate melanin (black, Figure 1. An asymptomatic erythematous vascular plaque on right ankle present for several years. [Copyright: ©2018 Zargari et al.] zenaidavega Inserted Text Skin Research Center, Department of Dermatology, Razi Hospital, School of Medicine, Guilan University of Medical Sciences, Rasht, Iran zenaidavega Cross-Out Letter | Dermatol Pract Concept 2018;8(3):18 245 tumor, a more recent hypothesis sug- gests that CCA is a reactive inflamma- tory dermatosis [5]. The higher inci- dence of CCA in lower limbs of elderly patients favors a reactive nature, prob- ably induced by stasis dermatitis [6]. Shalin et al demonstrated that the concurrence of CCA with syringofibro- adenomatous changes also suggests that both of these entities may share deriva- tion from the eccrine apparatus [7]. The clinical variants of CCA include giant, polypoid or pedunculated, pigmented, eruptive, atypical, and cystic patterns [8]. The giant type of CCA measures more than 40 mm. Until now, there are only 5 reports of giant CCA in the Eng- lish literature, these being located on the foot, buttock, and perineum. Due to the variable clinical presentations of CCA, it frequently is mistaken for several other lesions, including Bow- en’s disease, squamous cell carcinoma, malignant melanoma, Kaposi sarcoma, and angiosarcoma [9,10]. Indeed, the diagnosis is rarely made before skin biopsy, and frequently it can be misdiagnosed. Dermatoscopy can improve the accuracy in diagnosing CCA [9,11]. The dermatoscopic pattern of CCA was first described by Blum in 2001 as partly homogenous, symmet- rically or bunch-like arranged pinpoint- like capillaries [12]. CCA usually has a brown, gray, or blue). A biopsy was performed. Histopathological exami- nation showed an acanthotic epidermis with pale-appearing keratinocytes full of a glycogen-rich cytoplasm positive to periodic-acid-Schiff (PAS) staining, consistent with CCA (Figure 3). The lesion was treated with cryotherapy and curettage. In follow-up, the patient had not developed new CCAs after 1 year. Discussion CCA is frequently presented as a soli- tary, slowly growing nodule or plaque on the legs of elderly persons. Its size usually varies from 3 to 20 mm [4]. Perhaps, the original description of Fine and Chernosky is still the most com- prehensive one: “CCA has the stuck on appearance of seborrheic keratosis, the vascular look of pyogenic granuloma, the scale and exudation of an eczema- tous process and advancing rounded border of an epithelioma” [4]. The pathogenesis of CCA remains unknown. There is not significant evi- dence to support a traumatic or drug- induced origin. Although it was primar- ily considered to be a benign epidermal Figure  2. Dermoscopy of the lesion shown in Figure 1 showing a variegated red/purple- colored lesion with curved white lines and with dot vessels in a serpiginous pattern. [Copyright: ©2018 Zargari et al.] Figure 3. Dermatomicrographs of the lesion in Figures 1 and 2. (A) Hematoxylin and eosin (H&E) stain demonstrating pale-appearing cytoplasm of plump keratinocytes with elongat- ed rete ridges. (B) Pale keratinocytes full of a glycogen-rich cytoplasm positive to periodic- acid-Schiff (PAS) staining. (Original magnification: H&E stain ×100, PAS ×40). [Copyright: ©2018 Zargari et al.] unique appearance on dermatoscopy, characterized by red dots, globules, and, sometimes, coiled (glomerular) ves- sels, arranged in a serpiginous pattern. When fully developed, these serpiginous arrangements are strikingly symmetric. However, in some cases the serpiginous vascular pattern is incomplete or partly developed, either showing a forme fruste or a compression artifact but is still clearly recognizable. Dot or coiled (glomerular) vessels can also be a characteristic of inflamma- tory dermatoses, such as psoriasis, pity- riasis lichenoides, and discoid eczema. Nevertheless, in these diseases, the red dots or coiled vessels are uniformly dis- tributed and do not join together to form serpiginous vascular arrays [12- 16]. Other uncommon dermatoscopic features of CCA include the presence of areas of hemorrhage, orange crusts, and a peripheral collarette of translucent scales. A new finding recently described in the literature is the frequent presence of crystalline structures when polarized dermatoscopy is used for the evaluation of CCA [17]. CCA is a benign tumor, and when the diagnosis can be made with con- fidence due to the characteristic ser- piginous vascular pattern, treatment is not indicated for small asymptomatic lesions. If treatment is desired, surgical 246 Letter | Dermatol Pract Concept 2018;8(3):18 7. Shalin SC, Rinaldi C, Horn TD. Clear cell acanthoma with changes of eccrine syringofibroadenoma: reactive change or clue to etiology? J Cutan Pathol. 2013;40(12):1021-1026. doi: 10.1111/cup.12232. 8. Ko CJ, Subtil A. Clear (pale) cell acanthosis as an incidental find- ing. J Cutan Pathol. 2009;36(5):573-577. doi: 10.1111/j.1600- 0560.2008.01069.x. 9. Tiodorovic-Zivkovic D, Lallas A, Longo C, Moscarella E, Za- laudek I, Argenziano G. Dermoscopy of clear cell acanthoma. J Am Acad Dermatol. 2015;72(1)(suppl):S47-S49. doi: 10.1016/j. jaad.2014.06.039. 10. Tempark T, Shwayder T. Clear cell acanthoma. Clin Exp Dermatol. 2012;37(8):831-837. doi: 10.1111/j.1365-2230.2012.04428.x. 11. Ardigo M, Buffon RB, Scope A, et al. Comparing in vivo re- flectance confocal microscopy, dermoscopy, and histology of clear-cell acanthoma. Dermatol Surg. 2009;35(6):952-959. doi: 10.1111/j.1524-4725.2009.01162.x. 12. Blum A, Metzler G, Bauer J, Rassner G, Garbe C. The dermato- scopic pattern of clear-cell acanthoma resembles psoriasis vulgar- is. Dermatology. 2001;203(1):50-52. doi: 10.1159/000051703. 13. Pan Y, Chamberlain AJ, Bailey M, Chong AH, Haskett M, Kelly JW. Dermatoscopy aids in the diagnosis of the solitary red scaly patch or plaque-features distinguishing superficial basal cell carcinoma, intraepidermal carcinoma, and psoriasis. J Am Acad Dermatol. 2008;59(2):268-274. doi: 10.1016/j.jaad.2008.05.013. 14. Vázquez-López F, Manjón-Haces JA, Maldonado-Seral C, Raya- Aguado C, Pérez-Oliva N, Marghoob AA. Dermoscopic features of plaque psoriasis and lichen planus: new observations. Derma- tology. 2003;207(2):151-156. doi: 10.1159/000071785. 15. Argenziano G, Zalaudek I, Corona R, et al. Vascular struc- tures in skin tumors: a dermoscopy study. Arch Dermatol. 2004;140(12):1485-1489. doi: 10.1001/archderm.140.12.1485. 16. Bowling J. Diagnostic Dermatology: The Illustrated Guide. Ox- ford: Wiley-Blackwell; 2012. 17. Balagula Y, Braun RP, Rabinovitz HS, et al. The significance of crystalline/chrysalis structures in the diagnosis of mela- nocytic and nonmelanocytic lesions. J Am Acad Dermatol. 2012;67:194 e1–e8. excision may be the treatment of choice for single lesions. However, cryosurgery remains a very useful alternative, espe- cially for multiple lesions [10]. Conclusions Giant forms of CCA are extremely rare and confident clini- cal diagnosis is not always possible. Despite the nonspecific clinical presentation, the giant CCA reported herein had the known specific dermatoscopic feature of dot vessels distrib- uted in a serpiginous arrangement as well as a new feature of curved white lines, not previously reported. References 1. Degos R, Delort J, Civatte J, Poiares Baptista A. Epidermal tumor with an unusual appearance: clear cell acanthoma. Ann Dermatol Syphiligr (Paris). 1962;89:361-371. 2. Posligua A, Mercy K, Sable K, Amin S, Gerami P, Brieva JC. Dis- seminated eruptive clear cell acanthoma: A case report of a rare entity. J Am Acad Dermatol. 2016;74(5):AB51. doi: 10.1016/j. jaad.2016.02.203. 3. Wang SH, Chi CC. Clear cell acanthoma occurring on the hallux: the first case report. J Eur Acad Dermatol Venereol. 2006;20(9):1144-1146. doi: 10.1111/j.1468-3083.2006.01640.x. 4. Fine RM, Chernosky ME. Clinical recognition of clear-cell ac- anthoma (Degos’). Arch Dermatol. 1969;100(5):559-563. doi: 10.1001/archderm.1969.01610290043009. 5. Park SY, Jung JY, Na JI, Byun HJ, Cho KH. A case of polypoid clear cell acanthoma on the nipple. Ann Dermatol. 2010;22(3):337- 340. doi: 10.5021/ad.2010.22.3.337. 6. Zedek DC, Langel DJ, White WL. Clear-cell acanthoma versus acanthosis: a psoriasiform reaction pattern lacking tricholemmal differentiation. Am J Dermatopathol. 2007;29(4):378-384. doi: 10.1097/DAD.0b013e31806f46f2.