Dermatology: Practical and Conceptual Observation | Dermatol Pract Concept 2018;8(1):9 43 DERMATOLOGY PRACTICAL & CONCEPTUAL www.derm101.com Introduction Verrucous hemangioma is an uncommon congenital vascular malformation described by Imperial and Helwig as a separate entity in 1967 [1]. It usually presents at birth or in early child- hood, with the commonest site being the lower extremities. The initial lesion presents as a reddish macular area resem- bling a vascular stain. Gradually with the growth of child, the lesions increase in size, spread locally and become verrucous. The lesions are usually scattered but linear, serpiginous and reticular patterns can be seen rarely [2]. The linear arrange- ment of these lesions usually reflects genetic mosaicism or dermatomal distribution [3]. Verrucous hemangioma does not involute spontaneously, rather, incomplete excision can result in regrowth [3]. Clinically, VH is a close mimicker of other vascular lesions like angiokeratoma, infantile hemangioma and venous or lymphatic malformations. Thus, dermoscopic and microscopic evaluation aids in confirming the clinical diagnosis. Case Report A 13-year-old female presented with purplish, warty skin lesions over the inner surface of the left foot. Her mother stated that these lesions were noticed in early infancy, and with age they enlarged, increased in number, and became irregular on the surface. There was no history of any trauma or bleeding from these lesions. Cutaneous inspection revealed well-defined erythematous to violaceous plaques and nodules with verrucous surfaces arranged in a linear array over the medial aspect of the left foot that were tender on palpation (Figure 1a, b). No limb length discrepancy was noted. Sys- temic examination did not reveal any abnormalities, nor did laboratory investigations. Linear verrucous hemangioma—a rare case and dermoscopic clues to diagnosis Aditi Dhanta1, Payal Chauhan1, Dilip Meena1, Neirita Hazarika1 1 Department of Dermatology, Venereology & Leprology, All India Institute of Medical Sciences, Rishikesh, India Key words: verrucous hemangioma, vascular malformation, dermoscopy Citation: Dhanta A, Chauhan P, Meena D, Hazarika N. Linear verrucous hemangioma—a rare case and dermoscopic clues to diagnosis. Dermatol Pract Concept. 2018;8(1):43-47. DOI: https://doi.org/10.5826/dpc.0801a09 Received: June 26, 2017; Accepted: November 21; Published: January 31, 2018 Copyright: ©2018 Dhanta 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: Payal Chauhan, MD, Department of Dermatology, Venereology & Leprology, All India Institute of Medical Sciences, Rishikesh, India. Email: chauhanpayal89@gmail.com Verrucous hemangioma (VH) is a rare, congenital and localized vascular malformation, which usu- ally presents as warty, bluish, vascular papules, plaques, or nodules, mainly on the lower extremities. Linear presentation of the disease is rare. A deep biopsy is necessary to confirm the clinical diagnosis by histopathological examination, with dermoscopy acting as a useful tool for evaluating the precise vascular structure. Here, we report on a 13-year-old female child with linear VH presenting over her foot since infancy and dermoscopic findings of VH along with the clinical-pathologic features. ABSTRACT 44 Observation | Dermatol Pract Concept 2018;8(1):9 a parakeratotic epidermis. Small and thin-walled capillaries lined by flattened endothelial cells were seen predominantly in the dermis (papillary and deep dermis) reaching up to the dermosubcutaneous junction. A few capillaries were dilated and filled with fresh fibrin thrombi (Figure 2a-c). These features, thus, confirmed the diagnosis of verrucous hem- angioma. The patient was referred to plastic surgery, where serial excisions were planned to remove the whole lesion. Discussion In 1937, Halter introduced the term “verrucous haeman- gioma” which is an uncommon vascular malformation. It was first described as a separate entity and distinguished The dermoscopic features were different depending on the type and site of the lesions. The most striking feature was a prominent bluish background. Hyperkeratosis and bluish lacunae were observed in most of the lesions but they were most prominent in verrucous plaques (Figure 1c). The periph- ery of the plaque showed well-defined dark blue lacunae characteristic of vascular lesions(Figure 1d). Doppler sonography of the lower limb (arterial system) showed diffuse irregular echogenicity in the subcutaneous plane of the affected area of the left foot. At some places, minimum vascularity was observed and these sonographic features were suggestive of hemangioma. A 4 mm punch biopsy was taken for histopathological evaluation. Microscopy revealed elongation of rete ridges, thick parakeratosis and dense infiltration of eosinophils in A B C D Figure 1. (a) Erythematous to violaceous plaques with verrucous surface (black circle) arranged in a linear array over the medial aspect of the left foot. (b) Satellite plaques (black arrow) arranged linearly over the dorsum of foot. (c) Dermoscopy of verrucous lesions showing the prominent hyperkeratosis over bluish background (black circle) along with the reddish blue lacunae (black arrow) indicating the underlying dilated vascular channels. (d) Peripheral areas of the lesion showing the bluish lacunae (black arrow) characteristic of vascular lesions cor- relating with the vascular channels seen in histopathology. [Copyright: ©2018 Dhanta et al.] Observation | Dermatol Pract Concept 2018;8(1):9 45 hyperkeratosis seen in the verrucous lesions over a bluish background, which again favored a vascular etiology. VH in its pre-verrucous state may be indistinguishable from infantile hemangioma, venous or lymphatic malforma- tion and angiokeratoma. Dermoscopy of infantile heman- gioma has been reported to exhibit a polymorphous pattern of vascular structures with or without red linear and red dilated vessels [14]. The absence of a bluish component in hemangioma can help distinguish it from VH in early stages. As mentioned by Osio et al. [14], the color of the hemangioma can help classify infantile hemangioma with the superficial type showing a bright reddish color, and the superficial and deep type depicting a dark red color. We would like to believe that a prominent bluish component in VH is seen due to the depth of the vascular involvement. Angiokeratoma has been described as having three patterns in dermoscopy with dark lacunae and whitish veil in all three, peripheral erythema as a second pattern and hemorrhagic crust as a third [15]. Although it is difficult to distinguish between angiokeratoma and VH on the basis of dermoscopy the presence of reddish- blue lacunae without whitish veil, as seen in our case points more towards VH. More studies are needed to differentiate angiokeratoma from VH concretely. In its mature phase, the clinical and dermoscopic differen- tial diagnoses of VH include pigmented lesions like pigmented basal cell carcinoma, verrucous epidermal nevus and sebor- roheic keratosis in smaller lesions. Blue or dark lacunae are rarely seen with dermoscopic examination of non-vascular lesions. Complete absence of a pigment network which is a highly specific dermoscopic feature of melanocytic lesions, helps in differentiating the two. The absence of leaf-like and spoke-wheel pigmentation, arborizing vessels, and erosions separates pigmented basal cell carcinoma from VH. Verrucous epidermal nevus (VEN) shows a large brown circle represented by oval or round structures with a hyperchromic brown edge surrounding a hypochromic area. In VEN dermoscopic pattern is brown in color given its superficial nature and never blue, which helps in differentiating it from VH [16]. Seborrheic keratosis shows milia-like cysts, comedo-like openings, and fissuring from angiokeratoma and its other variants by Imperial and Helwig in 1967 [1]. VH usually presents at birth or in early childhood and then gradually progresses in size with age. The initial presen- tation is a reddish macular area of a vascular anomaly resem- bling a ‘‘port-wine’’ stain. Recurrent episodes of bleeding and infection result in the characteristic bluish-black color along with the development of a verrucous, hyperkeratotic surface [4]. Localized or scattered lesions on the unilateral lower extremity are the most common presentation. However lin- ear, serpiginous, or reticular pattern can also be seen, though uncommonly [2]. In our literature search, we could find only eight reported cases of linear verrucous hemangioma, making our patient the ninth [1,2,5-10]. Apart from the clinical picture, confirmation of diagno- sis is made with histopathology. Characteristic microscopic features of VH are irregular acanthosis and hyperkerato- sis in the epidermis. The abnormal proliferating vascular channels are located in the dermis and hypodermis, which differentiates it from angiokeratomas, where the lesion is limited to the papillary dermis. Currently, no specific immunohistochemical marker exists to diagnose VH. In one study, positivity for glucose transporter protein 1 (GLUT1), a determinant expressed by infantile hemangioma, was seen in 7 of 11 VH lesions [11]. The dermoscopic features reported in the literature include an alveolar appearance with numerous small, oval to polygonal elements surrounded by slightly darker pigmenta- tion. Different shades of blue, including light blue, indigo blue, dark bluish black and a bluish white veil have been described [12,13]. Well-defined dark lacunae were seen in the periphery in our case, which is characteristic of vascular lesions and further correlated well with the vascular chan- nels seen on histopathology. Dermoscopy of the late lesions showed more prominent hyperkeratosis along with the bluish lacunae indicating the underlying dilated vascular channels (DermLite II Hybrid M [3Gen, San Juan Capistrano, CA, USA]; 10 × magnification). In our case we could not find a significant alveolar pattern, but all the other features were seen. The highlight of our case was the expected dominant A B C Figure 2. (a) Scanning view showing hyperkeratosis, elongation of rete ridges, and vascular dilatations in the papillary dermis extending to subcutaneous tissue (H&E, 4x). (b) Hyperkeratosis, papillomatosis, acanthosis, and dilated blood vessels in the dermis (H&E, x10). (c) Small and thin-walled dilated capillaries seen in dermis lined by flattened endothelial cells (black arrow) with fibrin thrombi present in some of the vessels (H&E, x40). [Copyright: ©2018 Dhanta et al.] 46 Observation | Dermatol Pract Concept 2018;8(1):9 2. Hayashi H, Shimizu T, Nakamura H, Shimizu H. Linear ver- rucous haemangioma on the abdomen. Acta Dermatol Venereol. 2004;84(1):79-80. 3. Calduch L, Ortega C, Navarro V, Martinez E, Molina I, Jorda E. Verrucous haemangioma: report of two cases and review of the literature. Pediatr Dermatol. 2000;17(3):213-217. 4. Wang G, Li C, Gao T. Verrucous haemangioma. Int J Dermatol. 2004;43(10):745-746. 5. Klein JA, Barr RJ. Verrucous hemangioma. Pediatr Dermatol. 1985;2:191-193. 6. Jain VK, Aggarwal K, Jain S. Linear verrucous hemangioma on the leg. Indian J Dermatol Venereol Leprol. 2008;749(6):656–658. 7. Nupur P, Savant SS, Kumar P, Hassan S. Linear verrucous heman- gioma. Indian Dermatol Online J. 2014;5(suppl 2):S136–S137. 8. Kaliyadan F, Dharmaratnam AD, Jayasree MG, Sreekanth G. Lin- ear verrucous hemangioma. Dermatol Online J. 2009;15(11):7. 9. Srinivas SM, Mukherjee SS. Linear verrucous hemangioma in a child - A rare case report. Indian J Paediatr Dermatol. 2015;16:227-229. 10. Naveen KN, Pai VV, Athanikar SB, Athanikar VS, Rai V. Bluish red verrucous lesions on the leg. Cutis. 2015;95(3):E12-4. 11. Tennant LB, Mulliken JB, Perez-Atayde AR, Kozakewich HP. Verrucous haemangioma revisited. Pediatr Dermatol. 2006; 23(3):208-215. 12. Popadić M. Dermoscopic diagnosis of a rare, congenital vascular tumor: Verrucous haemangioma. J Dermatol. 2012; 39(12):1049- 1050. 13. Prabhakar V, Kaliyadan F. A case of verrucous haemangioma and its dermoscopic features. Indian Dermatol Online J. 2015; 6, Suppl S1:56-58. 14. Oiso N, Kawada A. The dermoscopic features in infantile heman- gioma. Pediatr Dermatol. 2011; 28(5):591-593. 15. JH, Kim MR, Lee SH, Lee SE, Lee SH. Dermoscopy: a useful tool for diagnosis of angiokeratoma. Ann Dermatol. 2012;24(4): 468-471. 16. Carbotti M, Coppola R, Graziano A, et al. Dermoscopy of ver- rucous epidermal nevus: large brown circles as a novel features for diagnosis. Int J Dermatol. 2016;55(6):653-656. without any vascular lacunae, which helps in distinguishing it form VH [17]. The dermoscopic findings of the differential diagnosis of verrucous hemangioma is summarized in Table 1. Dermoscopy can help play an important role in clinching the diagnosis and aiding in management when clinical find- ings alone or an inadequate (superficial) biopsy specimen is misleading. The importance of reaching an accurate diagnosis cannot be overemphasized when planning treatment, given that the treatment of choice for VH is complete surgical excision. Incomplete excision leads to persistence, recurrence and continued enlargement of the lesion. Due to the deeper vascular infiltration, the recurrence rate of VH is 33%, espe- cially when the lesions are larger than 2 cm in diameter [3]. Various other options that have been tried with limited results include ultrasound, cryosurgery and electrocautery, especially for smaller lesions [4,18,19]. Recently, a combination of CO2 and dual pulsed dye laser Nd:YAG has been reported to pro- vide satisfactory response in some cases [20]. Conclusion In this report, we describe clinical, dermoscopic and histo- pathological features of VH in a 13-year-old girl. We empha- size that VH has distinct dermoscopic features and suggests that dermoscopy can contribute significantly to diagnosing such a rare congenital vascular malformation. The interesting fact about this case is rarity of disease more so than the linear presentation along with the presence of both verrucous and pre-verrucous stages. Further, the dermoscopic pattern in both the types of lesions is another highlight of this case. References 1. Wentscher U, Happle R. Linear verrucous hemangioma. J Am Acad Dermatol. 2000;42(3):516-518. TABLE 1. Dermoscopic findings of verrucous hemangioma and its differential diagnosis 1. Verrucous Hemangioma Alveolar appearance with various shadows of bluish small, oval to polygonal elements surrounded by slightly darker pigmentation with well-defined dark lacunae in the periphery. Dominant hyperkeratosis seen in the verrucous lesions [12,13] 2. Infantile Hemangioma Polymorphous pattern of vascular structures with or without red linear and red dilated vessels [14] 3. Angiokeratoma Dark lacunae and whitish veil, peripheral erythema, and hemorrhagic crust in third pattern [15] 4. Pigmented Basal Cell Carcinoma Leaf-like and spoke-wheel pigmentation, arborizing vessels, erosions, blurred lacunae that may look like blue-gray ovoid nests [12] 5. Verrucous Epidermal Nevus Large brown circle seen as oval or round structures with a hyperchromic brown edge surrounding a hypochromic area [16] 6. Seborrheic Keratosis Milia-like cysts, comedo-like openings, fissures and ridges and sharply demarcated border [17] https://www.ncbi.nlm.nih.gov/pubmed/?term=Kaliyadan%252520F%25255BAuthor%25255D&cauthor=true&cauthor_uid=19624993 https://www.ncbi.nlm.nih.gov/pubmed/?term=Dharmaratnam%252520AD%25255BAuthor%25255D&cauthor=true&cauthor_uid=19624993 https://www.ncbi.nlm.nih.gov/pubmed/?term=Jayasree%252520MG%25255BAuthor%25255D&cauthor=true&cauthor_uid=19624993 https://www.ncbi.nlm.nih.gov/pubmed/?term=Sreekanth%252520G%25255BAuthor%25255D&cauthor=true&cauthor_uid=19624993 https://www.ncbi.nlm.nih.gov/pubmed/19624993 https://www.ncbi.nlm.nih.gov/pubmed/?term=Oiso%252520N%25255BAuthor%25255D&cauthor=true&cauthor_uid=21854412 https://www.ncbi.nlm.nih.gov/pubmed/?term=Kawada%252520A%25255BAuthor%25255D&cauthor=true&cauthor_uid=21854412 https://www.ncbi.nlm.nih.gov/pubmed/?term=Kim%252520MR%25255BAuthor%25255D&cauthor=true&cauthor_uid=23197916 https://www.ncbi.nlm.nih.gov/pubmed/?term=Lee%252520SH%25255BAuthor%25255D&cauthor=true&cauthor_uid=23197916 https://www.ncbi.nlm.nih.gov/pubmed/?term=Lee%252520SE%25255BAuthor%25255D&cauthor=true&cauthor_uid=23197916 Observation | Dermatol Pract Concept 2018;8(1):9 47 19. Fatani M, Al Otaibi H, Mohammed M, Hegazy O. Verrucous hemangioma treated with electrocautery. Case Rep Dermatol. 2016;8(2):112-117. 20. Segura Palacios JM, Boixeda P, Rocha J, Alcántara González J, Alonso Castro L, de Daniel Rodríguez C. Laser treatment for verrucous hemangioma. Lasers Med Sci. 2012;27(3):681-684. 17. Braun RP, Rabinovitz HS, Krischer J, et al. Dermoscopy of pig- mented seborrheic keratosis a morphological study. Arch Derm- tol. 2002;138(12):1556-1560. 18. Maejima H, Katsuoka K, Sakai N, Uchinuma E. Verrucous hem- angioma successfully treated using 13-MHz ultrasonography. Eur J Dermatol. 2008;18(5):597.