Genital Haemangioma with Ulceration: A Case Report

Dr. Ch. Saritha

DDVL

Consultant Dermatologist

Vishwa Skin Clinic

Siddipet, Telangana

Introduction

Haemangioma are benign proliferative tumour, often referred to as vascular malformations. It can be classified based on the location and depth of the tumour, as well as the types of blood vessels that are involved.1 It often breaks out at birth and develops later on in childhood.2 The most common type of haemangioma is the "capillary haemangioma," which is made up of small blood vessels called capillaries. These haemangioma are also known as "strawberry marks" because of their bright red colour. Capillary haemangioma typically occur in infants and young children and tend to disappear on their own over time.2 Another type of haemangioma is the "cavernous haemangioma," which is made up of larger blood vessels called cavernous vessels often located in internal organs such as the liver, brain or spinal cord.2

Genital haemangioma with ulceration is a rare condition that occurs when a benign tumour build up of blood vessels on the genitals, develops an ulcer or a break in the surface layer of the skin or mucous membrane. This can happen when a haemangioma becomes large or is located in an area that is subject to trauma or pressure. Genital haemangioma can vary in size, shape, location and it can be either superficial or deep. They can appear as a single lump or multiple lumps and they can be red or purple in color.1,3, 4 It is a relatively rare condition, but it can affect both males and females, with a higher incidence in females. They may be asymptomatic or associated with pain, bleeding and infection. The cause of genital haemangioma is not well understood, but it is thought to be a congenital disorder of abnormal proliferation of blood vessels. Ulceration is a complication that can occur due to the size and location of the haemangioma.1, 4 It shows characteristics similar to a neoplasm in true sense that it is consist of epithelial cells which is continuously being dividing and spreading while its multiplication there are new vessel channel that is being formed.5 It typically consist of three phases which includes emergence at birth followed by its proliferation for approximately 6 months and then slowly regresses towards the involution period.3 In its growing phase elevated levels of pro-angiogenic factors mainly vascular endothelial growth factors (VEGF) and the fibroblast growth factors (FGF) are observed.6

There is an elevated levels of mast cell observed which is indirectly linked with new vessel generation via heparin synthesis.5 Early diagnosis and appropriate management are essential to prevent complications and improve quality of life.

Symptoms of a genital haemangioma with ulceration can include raised, red or purplish lump on the genitals, pain or discomfort in the area of the lump, bleeding or discharge from the ulcerated area, swelling or tenderness in the affected area.

Hereby we are presenting a case of 3-months-old child who has a genital haemangioma with ulceration and he was treated successfully.

Case Presentation

A 3 months old baby was presented to our department with complaints of red, raised lesions over the genitalia. No similar ulceration could be detected anywhere else in the body. The baby was then treated with mild steroid with antibiotics topically over the ulcer for a week along with topical timolol drops with one drop twice daily application for 15 days then gradually increased the dose to 5 -6 drops daily twice till the whole haemangioma got cleared. After the therapy the lesions slowly turned pale from pink and eventually disappeared.

Before treatment After treatment

Diagnosis

The diagnosis of genital haemangioma is typically made based on the physical appearance of the lesion and confirmed by ultrasound, echographic or magnetic resonance imaging (MRI).1 In few severe and unsure cases biopsy are performed.6 In few cases angiography is done to examine the presence of any bruits or any deep involvement of any organs.2 On physical examination solitary lesions near the scrotum was found. Other examination carried out include identifying the type of haemangioma and its stage, location, underlying complications and ulceration (present or absent).6 Based on the evaluation made tailored treatment modalities have been selected like for proliferating lesions steroids have been proved to be best choice and non proliferating lesions can be treated by observation and care.2

Treatment

The management of genital haemangioma depends on the type, location, size and symptoms. Observation and monitoring of the present condition is one such option that can be implemented to ensure that it is not spreading or damaging the body. The treatment options include topical therapy and oral therapy. The other treatment options include surgical excision, cryotherapy, sclerotherapy and laser therapy. Steroid injection can be used to reduce inflammation and also shrink the size of the haemangioma.2

Oral therapy

Corticosteroids : Prednisolone is the preferred drug of choice in this class of drugs. Main action exhibited by it is repressing effect on section of vascular endothelial growth factor A (VEGF-A). Steroids have been proven to be effective in early stages of haemangioma and should be taken until any deflation is observed or cessation of the lesion. On chronic use many side effects are associated like cushingoid faces and in rare case adrenal insufficiency was observed.6

Propranolol: It’s a safe, efficient and good alternative to traditionally used drugs; it is a non-selective betaadrenergic receptor blocker. It acts by blocking the receptor and thereby counteracting the release of nitric oxide which is a vasodilator resulting in vasoconstriction of the capillaries adjoining in the haemangioma. Down regulation of pro-angiogenic factors are seen. It can also be used in treating ulcerated and painful cases. Relapse of the condition is observed in little case after completion of the therapy. Negative inotropic and also chronotropic effects has been observed on the heart as it is beta blocker.3,6

Topical Therapy

Timolol: It is non-selective beta-blocker and has successfully emerged as an alternative to systemic corticosteroids in treating haemangioma. It has been used in localized, superficial and non-ulcerative cases. It cannot penetrate deeply hence cannot be used in deep haemangioma. 1 drop usually contains 0.25mg of the drug. It is found to works better in proliferative phase rather than involution phase. No major and significant side effect reported yet and no systemic drugs needed.4, 6

Imiquimod: It is basically an immune response modifier useful in superficial healing with added antiangiogenic action. Some inflammatory changes were seen as a side effect.6

Systemic drugs like interferon alpha, vincristine and cyclophosphamide are reserved for life threatening cases.2,6

Surgical excision of the haemangioma, which can be done under local or general anaesthesia. Here significant loss of tissue is a major issue observed.2

Embolotherapy, which deals with embolization that is blocking of the vessel by some foreign substance.2

Cryotherapy, which involves freezing the haemangioma using liquid nitrogen and often associated with scaring.2

Sclerotherapy, which involves injecting a solution into the haemangioma to shrink it.2 Sodium tetradecyl sulfate, sodium morrhuate, sodium psylliate are few secreting agents used.2

Steroid injection, used to reduce inflammation in the area and also to shrink the size of the haemangioma.

Laser therapy, have gained a lot of popularity due to photothermolysis of the target rather than non – selective tissue damage caused due to surgical therapy.2

Pulsed Dye Laser (PDL) is proven efficacious in treating ulcerated haemangioma and causes rapid healing. It also helps in removal of residual dilated capillaries.6

Other lasers used are Nd:YAG laser and KTP (PotassiumTitanyl-Phosphate) laser but found to be less efficient as compared to PDL.

Discussion

Haemangioma being a vascular disorder, which originates usually during birth and involves later in the childhood years. Their occurrence in the genital areas is a rare presentation, which may be painful, distressing or functional impairment to the child and even great concern to their parents. In few instances of haemangioma is subjected to ulceration.3,4 Some harmful consequence associated with severity of diseases include rectal bleeding, haemorrhage, affecting spermatogenic activity, even spreading of lesion and ulceration to lower pelvic areas. Hence proper medication plan is must to prevent further spreading, pain and scarring of the skin. The main etiology associated in the emergence of the condition are unknown, at times it is observed to be originated due to decreased oxygenation and ulceration are thought to be caused due to diaper as it covers 75% of the surface of hemangioma.1 Some cases can be observed and monitored if they are small and asymptomatic, while others may require treatment. The most common treatment options for genital haemangioma include surgical excision, cryotherapy, sclerotherapy, steroid injection and laser therapy. Surgical excision is often the treatment of choice for larger or symptomatic haemangioma and it's done under local or general anaesthesia. Cryotherapy, sclerotherapy and laser therapy are used for smaller haemangioma and those located in sensitive areas. Steroid injection can be used to reduce inflammation and also shrink the size of the haemangioma.2 Sometimes, a combination of medications such as corticosteroids may be used to help shrink the haemangioma and control any associated symptoms.6

Genital haemangioma can lead to serious complications if left untreated. They can cause severe bleeding, infection and scarring, which can result in the need for multiple surgeries and also can affect sexual activity, pregnancy and delivery. Hence, it is important to seek medical attention and to be evaluated and treated by a medical professional specializing in this condition, such as a paediatric surgeon, a dermatologist, a plastic surgeon or an obstetrician/ gynecologist.1, 3

Conclusion

In conclusion, a genital haemangioma with ulceration is a rare condition that can cause significant discomfort and complications if not treated promptly and effectively. It is more prevalent in immature infants, especially girls. It disappears spontaneously and proper management may be necessary to prevent the haemangioma from causing complications such as bleeding, ulceration or pain. Consultation with a specialized practitioner such as a vascular surgeon, oncologist is important to determine the best treatment approach. In addition, patient education is important regarding possible risks, benefits and possible complication of the treatment that will be chosen. Furthermore, doctors should be aware that not all haemangioma require treatment and they should choose the best approach depending on the location, size and symptoms of the haemangioma. Close monitoring is crucial to check for any changes or new symptoms that may occur and prompt follow-up is recommended to ensure the most effective treatment and management of the condition.

Reference

1. Patoulias, Ioannis; Farmakis, Konstantinos; Kaselas, Christos; Patoulias, Dimitrios (2016). Ulcerated Scrotal Hemangioma in an 18-Month-Old Male Patient: A Case Report and Review of the Literature. Case Reports in Urology, 2016(), 1–4. doi:10.1155/2016/9236719

2. Ahuja Tarun, Jaggi Nitin, Kalra Amit, Bansal Kanishka, Sharma Shiv Prasad. Hemangioma: Review of Literature. The Journal of Contemporary Dental Practice, September-October 2013;14(5):1000-1007

3. Christine Tran, Joan Tamburro, Audrey Rhee, Alex Golden, Propranolol for Treatment of Genital Infantile Hemangioma, The Journal of Urology, Volume 195, Issue 3, 2016,Pages 731-737, ISSN 00225347, https://doi.org/10.1016/j. juro.2015.09.069.

4. Mutyala, Priyanka; Bommakanti, Janardhan. Ulcerated Infantile Genital Hemangioma Treated with

Timolol. Indian Journal of Paediatric Dermatology 22(1):p 92-93, Jan–Mar 2021. | DOI: 10.4103/ijpd. IJPD_36_20

5. Gary J. Alter; Guy TrengoveJones; Charles E. H Jr. (1993). Hemangioma of penis and scrotum. , 42(2), 205–208. doi:10.1016/00904295(93)90649-u

6. Sethuraman, Gomathy; Yenamandra, VamsiK; Gupta, Vishal (2014). Management of infantile hemangiomas: Current trends. Journal of Cutaneous and Aesthetic Surgery, 7(2), 75–. doi:10.4103/0974-2077.138324