Verrucous Hemangioma

Nur Camelia

, Syamsul Arifin

, Yosep Ferdinand Rahmat Sugianto

, Diah Adriani Malik

Yan Wisnu Prajoko

, Meira Dewi Kusuma Astuti

Departement of Dermatovenereology, Faculty of Medicine, Diponegoro University / Dr. Kariadi General Hospital

Departement of Surgery, Faculty of Medicine, Diponegoro University / Dr. Kariadi General Hospital

Departement of Anatomical Pathology, Faculty of Medicine, Diponegoro University / Dr. Kariadi General Hospital

Keywords: Verrucous hemangioma, vascular proliferation, excision

Abstract: Verrucous Hemangioma (VH) is a rare congenital vascular malformation consisted of capillary or cavernous

hemangioma affecting dermis and subcutaneous tissue. VH usually presents at birth or early childhood,

often unilateral and localized on the lower extremity. The clinical presentation shows hyperkeratotic plaques

and nodules, bluish-purple, and partly confluent. Early diagnosis and treatment are pivotal for a satisfactory

cosmetic result. A 28-year-old male presented with an asymptomatic rough lump on the left leg which

gradually enlarged, thickened and became rougher through time. The lesion appeared since birth and was

flat with red-bluish color, Dermatologic examination showed hyperkeratotic plaques and nodules, black-

grey colored, confluent with defined margins, measured 10 x 4 cm on lower left leg. Histopathologic

examination revealed epidermis hyperkeratosis, verrucous growth, acanthosis, elongated rete ridges, and

blood vessels proliferation in the dermis. The patient was treated with cryosurgery, and propranolol tablets 2

x 10 mg and subsequently referred for surgical excision. Diagnosis of VH is hallmarked by hyperkeratotic

nodules and confirmed by skin biopsy. Excision is one of the recommended treatment whenever possible.

The patient was treated with broad and deep excision due to the high recurrence of VH. After four months

of evaluation, the lesion showed satisfactory healing without signs of recurrences. The prognosis was quo

ad vitam ad bonam, ad sanam and ad cosmetikam dubia ad bonam. We have reported a patient with VH

treated by surgical excision, which showed satisfactory healing without signs of recurrences.

1 INTRODUCTION

Verrucous hemangioma (VH) is a rare congenital

vascular malformation, consisting of a proliferation

of dilated blood vessels of different sizes that occupy

the dermis and subcutaneous tissue. The epidermis of

the affected area shows a robust proliferative reaction

that presents as a warty appearance (fatani et al,

2016).

Verrucous hemangioma is rare, and only a few

cases have been reported (Nupur et al, 2014).

The

exact incidence is difficult to determine as it has been

referred to by many different names in the past (Laun

et al, 2019).

Verrucous hemangioma has been

reported under various names in the literature until

1967, including unilateral verrucous hemangioma,

hemangioma unilateralis neviforme, nevus vascularis

unius lateris, nevus angiokeratoticus, keratotic

hemangioma, nevus keratoangiomatosus, and papular

angiokeratoma (Fatani et al, 2016; Laun et al, 2019).

Verrucous hemangioma usually presents at

birth or in early childhood and then gradually

progresses in size with age (Laun et al, 2019; Dhanta

et al, 2018). It often unilateral and localized on the

lower extremity (Fatani et al, 2016; Dhanta et al,

2018 ; Sandhu et al, 2016).

The clinical presentations

are hyperkeratotic plaques and nodules, bluish-

purple, and partly confluent. The initial lesions

present as flat red or bluish lesions that slowly

enlarge and become verrucous (Dhanta et al, 2018 ;

Sandhu et al, 2016; Oppermann et al, 2018).

The

lesions are usually scattered but linear, serpiginous

and reticular patterns can be seen rarely. The linear

arrangement of these lesions usually reflects genetic

mosaicism or dermatomal distribution (Dhanta et al,

2018).

Verrucous hemangioma linear is a rarer

presentation, according to our literature search, only

10 cases have been reported until 2016 (Sandhu et

al, 2016).

394

Camelia, N., Ariﬁn, S., Sugianto, Y., Malik, D., Prajoko, Y. and Astuti, M.

Verrucous Hemangioma.

DOI: 10.5220/0009989903940398

In Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease (ICTROMI 2019), pages 394-398

ISBN: 978-989-758-469-5

A skin biopsy is needed to confirm the clinical

diagnosis (Fatani et al, 2016).

Verrucous

hemangioma, histologically characterized by dilated

capillaries and large cavernous spaces, lined by

endothelium. These dilated spaces extend into the

reticular dermis and subcutaneous fat. The overlying

epidermis shows reactive hyperplasia with marked

acanthosis, hyperkeratosis, and papillomatosis

(Bindhuja et al, 2013).

Early diagnosis and treatment are pivotal for a

satisfactory cosmetic result. The treatment of choice

for VH is surgical excision. Various therapeutic

options such as cryotherapy, ultrasonography,

electrocautery, NdYAG laser, and laser pulse-dye

can be considered as additional therapy, especially

for smaller lesions and when excision is not possible

(Laun et al, 2019; Sandhu et al, 2016; Prabhakar et

al, 2015).

This case is reported to increase our

understanding of making an accurate diagnosis of

VH and choosing an appropriate treatment.

2 CASE

A 28-year-old Indonesian man presented to the

outpatient Dermatovenereology Departement Dr.

Kariadi Hospital Semarang with complaints of

asymptomatic rough lump on the left leg, which

gradually enlarged, thickened and became rougher

through time. These lesions had been present since

birth, and the initial lesion was flat with red-bluish

color. There was no history of any trauma or

bleeding from these lesions. The patient gave a

history of about 14 years ago that he had been taken

by his mother for treatment, and some of the lesions

had been excised. There was a recurrence of the

lesion after several years. The clinical notes and

histopathological reports of the previous excision

were not available.

On physical examination, the patient was

composmentis. Height 168 cm, weight 67 kg, blood

pressure: 120/70 mmHg, heart rate: 84 beats/minute,

respiratory rate: 22 breaths/minute, and axillar

temperature: 36,7

C. There were no enlarged lymph

nodes in the inguinal or in the area around the lesion.

On clinical examination showed hyperkeratotic

plaques and nodules in a linear pattern, black-grey

colored, confluent with defined margins, measured

10 x 4 cm on the lower left leg (Figure 1.A).

Figure. 1. A. Hyperkeratotic plaques and nodules in a linear pattern, black-grey colored, confluent with defined margins on

the lower left leg. B. 1. Hyperkeratosis, 2. Parakeratosis (Hematoxylin & eosin, x40). C. 1. Acanthosis, 2. The proliferation

of blood vessels in the superficial dermis (Hematoxylin & eosin, x10). D. Elongated rete ridge (Hematoxylin & eosin, x10).

Verrucous Hemangioma

395

The routine laboratory and coagulation factors

examination result were standard. The lesion was

biopsied and confirmed to be a verrucous

hemangioma. Histopathological examination

showed the epidermis in the form of a stratified

squamous cell epithelium, keratinized,

hyperkeratosis, parakeratosis, verrucous growth,

elongated rete ridge, acanthosis containing a

proliferation of partially dilated blood vessels lined

with endothelial cells, with lumen containing

erythrocytes surrounded by epidermal papillae. In

the superficial dermis, there was the varying size of

blood vessel proliferation, skin adnexa accompanied

by a mild distribution of interstitial

lymphohistiocytic. No malignancy sign found

(Figure 1. B-E).

Previously this case had been treated with

cryotherapy, propranolol tablets 2x10 mg, and

retinoid acid 0,1% cream applied twice daily. After

ten days of evaluation post, cryotherapy showed that

the lesion had a mild regression. Propanolol tablets

and retinoid acid 0,1% cream were still continued.

However, after three months of evaluation, the

lesions recurred (Figure 2. A-B). Due to recurrence,

the patient was referred to the surgery department

for excision. All the lesions were excised with a 1

cm margin (Figure 2.C-F).

Figure. 2. A. The lesions immediately following the cryotherapy procedure. B. Recurrence of the lesion after three months

post cryotherapy (black arrow). C. The resected margin was 1 cm away from the lesions. D. The lesions were completely

excised. E. Postoperative photography following surgical excision. F. After four months of evaluation, the lesion showed

satisfactory healing without signs of recurrences.

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3 DISCUSSION

The term VH is defined by Imperial and Helwig in

1967, which means that congenital localized

vascular malformations. (fatani et al, 2016; (Dhanta

et al, 2018; Prabhakar et al, 2015).

In 1996, The

International Society for the Study of Vascular

Anomalies classified vascular anomalies into

vascular malformations, and vascular tumors.

(J.Bindhuja et al,2013 Diagnosis of VH is

established based on clinical features and

histopathological examination. Prabhakar et al,

2015;Nargis et al,2017).

Verrucous hemangioma presents on the lower

extremity in 95% of cases and typically is unilateral

(Laun et al, 2019;Singh et al,2017) It may also

involve unusual anatomic locations such as the

abdomen, arm, and glans penis. Although VH

almost invariably presents at birth or in early

childhood, it may appear later on, even in adult life

(Sandhu et al, 2016; Singh et al,2017) In our case,

the lesion appeared from birth and located on the

lower left leg.

In the early phase of evolution, the lesions are

non-keratotic, soft, blue/red plaques, and clearly

demarcated. Gradually the lesions become

increasingly hyperkeratotic and verrucous (Nargis et

al,2017;Moss et al,2010). The lesions may vary in

size from roughly 0,5 to 8 cm in diameter and maybe

single or grouped. (Laun et al, 2019)

Verrucous

hemangioma in its mature phase presents as

hardened, hyperkeratotic plaques or nodules with a

brownish to bluish-black appearance. This is often

accompanied by a history of tenderness and/or

bleeding following minimal trauma.(Vijayan et

al,2016) The linear form of VH is rare, and only a

few cases have been reported. It is not known

whether linear lesions actually follow Blaschko’s

lines or the linear arrangement represents genetic

mosaicism. (Nupur et al, 2014) In our case, the

lesion showed in a linear pattern, and there was no

history of any trauma or bleeding from these lesions.

Histologically, VH shows with hyperkeratosis,

variable epidermal acanthosis, and papillary

telangiectasias overlying a deep capillary or

cavernous hemangioma. The abnormal proliferating

vessels are situated in the dermis and hypodermis.

The hemangiomatous component mostly comprises

dilated capillaries and wider cavernous,

endothelium-lined, blood-filled spaces.

Inflammatory cells, fibrosis, and hemosiderin may

exist in the upper dermis. (fatani et al, 2016;

J.Bindhuja et al,2013; Moss et al,2010). Typical

histopathological features were observed in our case

also. Immunohistochemical staining with endothelial

markers like CD 31, CD 34 and GLUT1 may be

done for confirmation, but the diagnosis can be

made by light microscopic features alone.

(J.Bindhuja et al,2013;(Vijayan et al,2016)

The differential diagnosis with angiokeratoma

can be excluded. The histologic appearance closely

resembles angiokeratoma, as both lesions show

vascular spaces beneath a papillomatous and

hyperkeratotic epidermis. However, in contrast to

angiokeratoma, the vascular spaces in VH also

involve the lower dermis and subcutaneous tissues.

(Oppermann et al, 2018;.Naveen et al,2016)

Verrucous epidermal nevus (VEN) also can be

excluded because histologically, the hallmark

finding of VEN is hyperkeratosis, acanthosis, and

papillomatosis. In VEN, there are no abnormal

proliferation of blood vessels (Das et al, 2015)

Verrucous hemangioma should be identified,

diagnosed, and treated as early as possible to limit

the extent of resection. Because of the risk of

recurrence, resection should encompass the deep

portions of the lesion with usually a 1 cm margin of

excision. If the lesion is small (<2 cm), cryosurgery,

electrocautery, or laser therapy can be used, but

resection is the primary treatment. These additional

therapies can be used in combination with resection

for extensive lesions to further assist in reducing the

risk of recurrence. (Laun et al, 2019)

In our case, a

propanolol tablet was given for three months, but

after that, there was a recurrence. Propranolol is the

treatment of choice for troublesome haemangiomas.

Other studies that have employed oral propranolol

therapy would not recommend using it on other

vascular anomalies. Oral propranolol is more

effective in hemangioma infantile than in an

adult.(Dimaguila et al,2017)

Surgical excision is one of the recommended

treatment whenever possible, and 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%, especially when the lesions are more

significant than 2 cm in diameter (Dhanta et al,

2018) The patient was treated with broad and deep

excision. After evaluation for four months, the lesion

showed satisfactory healing without signs of

recurrences.

The prognosis for VH is excellent, with

recurrence being low when adequate surgical

margins are utilized and if in combination with

additional therapies. If inadequate wide excision is

performed, recurrence can exceed 30%.(Laun et al,

2019) The prognosis of this case was quo ad vitam

Verrucous Hemangioma

397

ad bonam, quo ad sanam, and quo ad cosmeticam

dubia ad bonam.

4 CONCLUSION

We have reported a case of verrucous hemangioma

in a 28-years old male. The diagnosis was made on

the basis of anamnesis, clinical examination, and

histopathological examination. On the anamnesis,

the patient complained of asymptomatic rough lump

on the left leg, which gradually enlarged, thickened,

and became rougher through time. The lesion

appeared since birth and was flat with red-bluish

color. The clinical examination, we found

hyperkeratotic plaques and nodules in a linear

pattern, black-grey colored, confluent with defined

margins, measured 10 x 4 cm on lower left leg.

Histopathological examination confirmed the

diagnosis of verrucous hemangioma. The patient

was treated with broad and deep surgical excision,

which showed satisfactory healing without signs of

recurrences. The prognosis of this case was quo ad

vitam ad bonam, quo ad sanam dubia ad bonam, and

quo ad cosmeticam dubia ad bonam.

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