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Treatment Protocols for Angiolymphoid Hyperplasia

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Angiolymphoid hyperplasia is a low-grade vascular tumor with no definitive treatment modalities. This article explains different treatments for this condition.

Written byDr. Asha. C

Medically reviewed byDr. Kaushal Bhavsar

Published At March 13, 2023
Reviewed AtMarch 13, 2023

What Is Angiolymphoid Hyperplasia?

Angiolymphoid hyperplasia is an uncommon benign microvascular proliferation tumor. It is also known as epithelioid hemangioma. The exact cause of this disorder is not clear. However, inflammatory processes such as allergic reactions, autoimmune disorders, or trauma are considered possible causes. The occurrence of angiolymphoid hyperplasia is very rare, but it is more reported in Asians. Middle-aged and young women are more commonly affected. It usually presents with smooth-surfaced red to brown solitary or multiple papules or nodules, mostly on the head and neck region; most nodules are localized near the ear. Rare cases have been reported to appear in other sites of the body, like the extremities, trunk, genitalia, lips, and oral mucosa. The main aim of the treatment is to attend to cosmetic issues and to provide relief for the symptoms. Many treatments were tried, but none of them provided successful results.

What Is the Cause of Angiolymphoid Hyperplasia?

The exact cause of angiolymphoid hyperplasia is not clear. Few theories have been proposed,

  • Trauma caused due to friction, surgery, frostbite, laceration, etc., can lead to arteriovenous shunts. This can lead to a hyperestrogenic state (an excessive amount of estrogenic activity in the body), which can be a causative factor for this condition. Also, the renin (an enzyme) induced due to arteriovenous shunts might be a pathogenic factor for this condition.

  • The second hypothesis is that angiolymphoid hyperplasia is considered a lymphoproliferative disorder and has support from the condition's frequent recurrences and progressive course. T-cell receptor gene rearrangements have been observed in these cases with angiolymphoid hyperplasia, raising the possibility of considering this condition as a low-grade T-cell lymphoma.

  • Angiolymphoid hyperplasia is a true vascular neoplasia with increased inflammatory components or a lymphoproliferative process that is yet to be confirmed.

What Are the Symptoms of Angiolymphoid Hyperplasia?

  • Angiolymphoid hyperplasia is a benign condition that proliferates locally.

  • These lesions often arise in the skin of the head and neck, particularly around the ear and the scalp.

  • It can also appear on any site of the body, like the trunk, extremities, orbit, and oral and genital mucosa.

  • Involvement of internal organs such as the colon, kidney, heart, bone, and lung have been reported but to a lesser extent.

  • The lesion appears as a single or multiple pink to reddish brown papules with or without nodules of different sizes.

  • The lesions may have pus discharge, bleeding, or pain. Regional lymph node enlargement is also rarely reported.

What Is the Treatment for Angiolymphoid Hyperplasia?

Angiolymphoid hyperplasia is a benign disease, and its treatment is challenging because of the frequent recurrences and no proper controlled clinical trials. There are reports of multiple therapeutic options that do not have consistent results. The treatment options presently available are as the following:

Watchful Waiting - In some rare cases, the tumor has shrunk or gone away without treatment. So if a person has no symptoms and is waiting is advisable to check if there is a progression in the lesion. The doctor may observe the lesion for a while to determine whether treatment is necessary.

Surgical Excision - It is the preferred treatment option for angiolymphoid hyperplasia. The failure rate is very less for surgical excision. However, a high recurrence rate is reported in some cases if complete removal is impossible due to the location, size, or presence of multiple tumor sites. Small and easily accessible tumors may be fully removed. For lesions with ill-defined margins, Mohs micrographic surgery is the suggested treatment. Mohs microscopic surgery is a procedure carried on in treating certain types of skin cancer on the face, fingers, or genitals, soft tissue sarcoma of the skin, and penile cancer. During this surgery, a small sample of tissue layer at the visible cancer is removed and checked under a microscope for cancer cells. This procedure will be repeated until all cancerous tissue is removed. This surgery will also remove a little normal tissue. In lesions with large cutaneous vessels, if embolization (a minimally invasive procedure that closes or blocks a specific blood vessel or abnormal vascular channels) is performed before surgery, a better result can be obtained.

Laser Therapy - Laser surgery is also called laser ablation; in this treatment, a high-intensity beam of light is used to destroy cancer cells. It is a good alternative for surgery in patients with multiple lesions, in sensitive cosmetic sites, and in poor surgical candidates. The lasers previously used for the treatment were argon, pulsed dye, Nd: YAG, and carbon dioxide lasers. But they caused over 50 percent of recurrence in patients. A combination of carbon dioxide and pulsed dye lasers also showed better results. However, the recurrence can be prevented by maintenance treatment.

Medications - Previously, systemic corticosteroids, Isotretinoin, Dapsone, and Pentoxifylline, were used in the systemic treatment of angiolymphoid hyperplasia, but it was not beneficial; they had a high failure rate of 80 to 100 percent. Few cases were successfully treated with Methotrexate and Thalidomide. Angiolymphoid hyperplasia associated with blood eosinophilia was treated with Mepolizumab or an anti-interleukin-5 antibody, which helped for local pruritus and hypereosinophilia, while the nodules regressed only slightly. In recent studies, oral propranolol was shown to reduce the size or number of lesions.

Topical and intralesional corticosteroids were initially used as a therapeutic agents for angiolymphoid hyperplasia, but the recurrence rate was high. Topical Timolol is helpful in the treatment of angiolymphoid hyperplasia. Other topical treatments include topical Tacrolimus 0.1 percent, topical Imiquimod 5 percent, and intralesional interferon alpha-2b.

Other Treatments

  • Photodynamic Therapy - It is a treatment that uses specific drugs that are activated by light to kill cancer and other abnormal cells.

  • Cryotherapy - Also called cold therapy, uses extremely low temperatures to remove abnormal tissues.

  • Radiotherapy - Also called radiation therapy, that uses high-energy x-ray or other particles to kill cancer cells.

  • Sclerotherapy - This therapy involves an injection of a solution directly into the vein. The injected solution irritates the lining of the blood vessels, leading to the blood vessels collapsing, scarring, and fading. Injecting Polidocanol or Bleomycin was successful and resulted in complete healing.

Conclusion:

Angiolymphoid hyperplasia is an uncommon condition that mostly affects the skin and mucosa. The exact etiology of the condition is not clear. There are different treatment options for treating angiolymphoid hyperplasia, including surgical excision, lasers, cryotherapy, and radiation therapy. However, no treatment options have been proven to provide consistent results. Also, recurrence after most cases is common.

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Frequently Asked Questions

The treatment for angiolymphoid hyperplasia typically involves surgical removal of the affected lesions. In some cases, laser therapy or cryotherapy may be used as alternative treatment options. The treatment selection is influenced by various factors, including the extent and position of the lesions, alongside individual patient-related aspects. Regular follow-up visits are important to monitor for any recurrence or progression of the condition.
Another term for angiolymphoid hyperplasia is Angiolymphoid Hyperplasia with Eosinophilia (ALHE). Both terms refer to the same condition characterized by the proliferation of blood vessels and eosinophils in various tissues, commonly affecting the head and neck region.
The underlying disease process of angiolymphoid hyperplasia involves the abnormal growth and proliferation of blood vessels and inflammatory cells, particularly eosinophils. This leads to the formation of nodules or lesions in affected tissues, often accompanied by symptoms such as itching, pain, or swelling.
Kimura disease and angiolymphoid hyperplasia are two distinct but closely related conditions. While both conditions involve the abnormal proliferation of blood vessels and eosinophils, they differ in their clinical presentation and anatomical distribution. Kimura disease typically presents as painless swelling in the head and neck region, often accompanied by enlarged lymph nodes. On the other hand, angiolymphoid hyperplasia commonly manifests as solitary or multiple nodules on the skin, usually on the scalp or face. Histopathological examination is necessary to differentiate between these two conditions.
When diagnosing angiolymphoid hyperplasia, it is important to consider other conditions with similar clinical presentations or histological features. Some of the differential diagnoses to consider include angiosarcoma, hemangioma, pyogenic granuloma, lymphoma, and other eosinophilic disorders. Proper evaluation, examination, and histopathological analysis are essential for accurate diagnosis and differentiation from these potential conditions.
The potential complications associated with angiolymphoid hyperplasia can differ based on the location and extent of the lesions. In some cases, the lesions may cause discomfort, pain, or cosmetic concerns. If the lesions are located near vital structures, such as the eyes or major blood vessels, there is a risk of functional impairment or bleeding. Recurrence of the lesions is also possible, requiring additional treatment or monitoring. Prompt diagnosis and appropriate management can help minimize the risk of complications.
The curability of angiolymphoid hyperplasia depends on various factors, including the extent of the lesions and the response to treatment. Surgical excision is often the primary treatment approach, and complete removal of the lesions can lead to a cure. However, there is a possibility of recurrence in some cases. Additional treatment options, like laser therapy or cryotherapy, could prove effective; however, they necessitate several sessions to achieve the desired results. Regular follow-up and monitoring are important to detect any recurrence or progression of the condition.
Angiolymphoid hyperplasia is generally considered a benign condition; however, it can be a serious concern depending on its location, size, and associated symptoms. In some cases, the lesions can cause discomfort, pain, or functional impairment. Additionally, if the lesions are located near critical structures, there is a potential risk of complications such as bleeding or obstruction. Prompt diagnosis, appropriate management, and regular monitoring are necessary to address any potential seriousness associated with angiolymphoid hyperplasia.
Angiolymphoid hyperplasia with eosinophilia (ALHE) is a variant of angiolymphoid hyperplasia that is distinguished by the occurrence of eosinophils, a particular type of white blood cells, within the affected tissues. The presence of eosinophils distinguishes this variant from typical angiolymphoid hyperplasia. The exact cause of angiolymphoid hyperplasia with eosinophilia is still unknown and needs more research.
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Dr. Kaushal Bhavsar from iCliniq
Dr. Kaushal Bhavsar

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