|Year : 2021 | Volume
| Issue : 1 | Page : 50-53
Verrucous hemangioma: A rare and distinct entity
Farhan Asif Siddiqui1, Jawad Al-Khalaf1, Yusef Al-Marzooq1, Kimy Khan2
1 Department of Laboratory and Blood Bank, King Fahad Hospital, Hofuf, Kingdom of Saudi Arabia
2 Department of Dermatology, Al Ahsa Hospital, Hofuf, Kingdom of Saudi Arabia
|Date of Submission||04-Aug-2020|
|Date of Decision||06-Aug-2020|
|Date of Acceptance||25-Sep-2020|
|Date of Web Publication||13-Jan-2021|
Dr. Farhan Asif Siddiqui
Department of Laboratory and Blood Bank, King Fahad Hospital, Hofuf
Kingdom of Saudi Arabia
Source of Support: None, Conflict of Interest: None
Verrucous hemangioma (VH) is an uncommon congenital vascular lesion that presents at birth or in early childhood. The clinical and histopathological resemblance to angiokeratoma and infantile hemangioma raises the question of it being a variant or a separate distinct entity. The authors take this opportunity of reporting a case of VH, occurring in a 14-year-old female for its rarity and the possibility of recurrence, if not diagnosed correctly and treated by wide excision followed by skin grafting.
Keywords: Congenital, hemangioma, vascular, verrucous
|How to cite this article:|
Siddiqui FA, Al-Khalaf J, Al-Marzooq Y, Khan K. Verrucous hemangioma: A rare and distinct entity. Curr Med Issues 2021;19:50-3
| Introduction|| |
Verrucous hemangioma (VH) is a rare congenital vascular lesion present since birth or very early in life, with lower extremities being the most common site. It needs to be differentiated from other vascular lesions sharing similar clinical and histopathological features, due to its different therapeutic approach. Histopathological examination of a deep biopsy is essential for confirmation of the diagnosis of VH based on its involvement of the deep dermis and subcutaneous tissue.
| Case Report|| |
A 14-year-old girl presented with the chief complaints of the hyperpigmented raised lesion over the lower one-third of her right leg, measuring approximately 3.5 cm × 2 cm. The lesion was present since birth and developed a verrucous surface gradually over the years. There was no history of any bleeding, ulceration, or infection over that lesion. Local examination showed a well-circumscribed, verrucous, and hyperkeratotic plaque with a diameter of 3.5 cm × 2 cm with no limb length discrepancy. General and systemic examinations were unremarkable. Routine blood counts and routine biochemical tests were within the normal limits.
Complete excision of the hyperpigmented lesion till the subcutaneous tissue was performed followed by the closure in layers with full-thickness skin graft harvested from the lower abdomen. The excised tissue was sent for histopathological examination. On gross examination, the specimen was a single firm, gray-tan skin ellipse with attached variable amount of subcutaneous adipose tissue, measuring 6.8 cm × 2.7 cm × 1.9 cm, and showing central papulonodular skin lesion spanning 3.5 cm × 2 cm. Serial sectioning revealed a variegated, polypoid, and gray-brown lesion.
Microscopic sections revealed skin fragment showing a verrucous epidermis overlying a benign vascular lesion. The epidermis was verrucous, featuring papillomatosis, acanthosis, hyperkeratosis, focal parakeratosis, and focal ulceration. The vascular lesion, composed of small to medium-sized venulocapillary blood vessels, extending to subcutaneous adipose tissue, filled with RBCs, embedded in a fibrous stroma, with variable mixed inflammatory cell infiltrate [Figure 1],[Figure 2],[Figure 3]. A diagnosis of VH was rendered.
|Figure 1: Photomicrograph showing verrucous epidermis featuring hyperkeratosis, papillomatosis, and acanthosis along with benign dermal vasoformative lesion comprising of venulocapillary blood vessels (H and E, ×20).|
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|Figure 2: Photomicrograph showing dermal variably sized venulocapillary blood vessels lined by non-descript endothelium, filled with RBCs (H and E, ×100).|
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|Figure 3: Photomicrograph showing the extension of the vascular lesion into the subcutaneous tissue in the form of small lumen capillaries infiltrating the subcutaneous adipose tissue (H and E, ×40).|
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There was no recurrence of lesion post excision after 13 months.
| Discussion|| |
International Society for the Study of Vascular Anomalies categorizes vascular lesions into vascular malformations or vascular tumors. However, there are some vascular lesions where the clinical and histopathologic findings overlap or are not sufficient enough to strictly categorize the lesion as a tumor or a malformation. VH is one such lesion. [Table 1] enlists the clinical presentation and management of some of the cases of VH reported.
VHs are rare, localized vascular lesions. These are usually congenital, occurring at birth or early childhood. The Lower limbs are the most common site of involvement, however, other locations such as the abdomen, arm, and glans penis have also been reported. VHs generally have a unilateral involvement with a linear or serpiginous distribution and appear as well-demarcated discrete to confluent, bluish-red/blackish, warty papules, plaques, or nodules. Size variation is well documented and these often develop satellite lesions. Spontaneous resolution is not reported, and these tend to enlarge, become verrucous and recur after incomplete excision.
Histologically, VH in the early phase may mimic a capillary hemangioma concentrated in the dermis and the subcutis. However, in the latter phase, it shows a verrucous pattern with hyperkeratosis with focal parakeratosis, papillomatosis, irregular acanthosis, and an underlying dermal hemangioma which extends to the subcutaneous tissue.
In 1958, Loria et al. defined it as a distinct entity. Many cases have been reported in the literature with a variety of names; however, it was in 1967, when Imperial and Helwig, introduced the term “VH.”, Despite its name, VH is considered by many authors as a malformation rather than an authentic hemangioma. Many features support this including the age of presentation and the growth. VH is typically seen at birth or early childhood which is synonymous with vascular malformations. Secondly, VH do not have a rapid growth phase as is generally seen with vascular tumors. Trindade et al., challenged this viewpoint by showing WT-1 positivity in VH, suggesting it as a vascular neoplasm, although WT-1 is nonspecific and can be seen in neoplastic as well as nonneoplastic lesions.
Some authors are of the opinion that VH is a type of angiokeratoma as their clinical features are quite similar. However, the histopathological picture is a bit different (mentioned later) and so is their biological behavior. Imperial and Helwig considered VH and angiokeratoma as two different entities based on the histological and biological differences, implying a different prognostic and therapeutic approach.,
Differential diagnosis includes angiokeratoma and infantile hemangioma. Angiokeratomas are telangiectatic and have no involvement of the deep dermal or subcutaneous tissue which is seen in VHs and therefore deep biopsy plays an important role., Although infantile hemangiomas do undergo spontaneous involution, this might take some years. However, infantile hemangiomas can be differentiated based on immunohistochemistry with infantile hemangiomas being positive for hemoglobin ζ chain (HBZ) and VH being negative.
Spontaneous resolution of VH has not been reported. The recommendation is excision while they are still small as they tend to become big and spread along with body growth and may lead to the development of large scars., Local excision or electrocautery are the preferred options for smaller lesions, and wide deep excision for the large lesions, which may require grafting. Laser therapy has also helped partially in some cases. About one-third of the cases show postsurgical recurrence but generally with adequate free margins, recurrences do not occur.
VHs do not respond to physical therapy (diathermocoagulation, cryotherapy and sclerotherapy). It is therefore important to timely and correctly diagnoses VH from both prognostic and therapeutic point of view. Any delay in the diagnosis would lead to undesirable consequences such as large displeasing scars. Unfortunately, in practice, the biopsy specimens are usually superficial and seldom contain subcutis and therefore diagnosis might be missed. It is, therefore, very essential to keep a differential of VH for verrucous lesions occurring in the young population, especially on the lower extremities.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his clinical information and investigation reports to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]