What causes Angiolymphoid hyperplasia with eosinophilia?

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare vascular disorder described in 1969 by Wells and Whimster [1]. It preferably affects women between 20 and 40 years old, being of benign character. Underlying vascular malformation or local trauma are suggested as possible causes [2,3,4].

What is ALHE?

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon, vasoproliferative, idiopathic condition that manifests in adults as isolated or grouped papules, plaques, or nodules in the skin of the head and neck. Most patients present with lesions in the skin of the periauricular region, forehead, or scalp.

What is Kimura’s disease?

Kimura disease is a rare, noncancerous, chronic condition that causes the tissue under the skin of the head or neck to become swollen. Masses generally appear in a person’s mid-20s and the disease mainly affects Asian men. Kimura disease is diagnosed by a surgical biopsy and the cause of the disease is unknown.

What is hypereosinophilic syndrome?

Hypereosinophilic (hy-per-ee-o-SIN-o-phil-ik) syndrome (HES) is a group of blood disorders that occur when you have high numbers of eosinophils — white blood cells that play an important role in your immune system. Over time, the excess eosinophils enter various tissues, eventually damaging your organs.

What is epithelioid haemangioma?

Epithelioid hemangioendothelioma, or EHE, is a rare cancer that grows from the cells that make up the blood vessels. This cancer can occur anywhere in the body with the most common sites being the liver, lungs, and bone.

What is eosinophilic fasciitis?

Eosinophilic fasciitis is a rare disorder characterized by inflammation of the tough band of fibrous tissue beneath the skin (fascia). The arms and legs are most often affected. Inflammation is caused by the abnormal accumulation of certain white blood cells including eosinophils in the fascia.

What do eosinophils mean?

Eosinophils are a type of disease-fighting white blood cell. This condition most often indicates a parasitic infection, an allergic reaction or cancer. You can have high levels of eosinophils in your blood (blood eosinophilia) or in tissues at the site of an infection or inflammation (tissue eosinophilia).

What causes Castleman’s disease?

What causes Castleman disease? Infection such as human herpes virus 8 (HHV-8) and possibly others as well as problems with the body’s immune system may cause Castleman disease. Castleman disease can be associated with other cancers such as lymphoma.

What will happen if eosinophils are high?

The eosinophil count measures the amount of eosinophils in your blood. The key is for eosinophils to do their job and then go away. But if you have too many eosinophils in your body for a long time, doctors call this eosinophilia. It can cause chronic inflammation, which could damage tissues.

Does hypereosinophilic syndrome go away?

There is no cure. If HES is left untreated, the disease may be fatal. Your doctor can best answers questions about your specific prognosis in HES. Bone marrow biopsy in Hypereosinophilic Syndrome, exhibiting a markedly increased number of eosinophils.

Are hemangiomas itchy?

Epithelioid hemangioma usually forms on or in the skin, especially the skin of the head, but can occur in other areas of the body, such as in bone. On the skin, it may appear as firm pink to red bumps that may be itchy or painful.

Is epithelioid hemangioendothelioma malignant?

Epithelioid hemangioendothelioma (EH) is an uncommon low-grade malignant tumor of vascular origin that may develop in the soft tissue, lung, bone, brain, liver, and small intestine. Primary hepatic EH is rare. 1 In the literature, approximately 200 cases have been reported.

Which is the best treatment for Kimura disease?

Imatinib may be an effective treatment for Kimura disease, based on advances in research for therapy in hypereosinophilic syndrome, but further investigation is necessary. Photodynamic therapy has been used successfully in one patient who experienced recurrence of disease after initial surgical management.

Is the Kimura disease the same as ALHE?

Once thought to be the same disorder, angiolymphoid hyperplasia with eosinophilia (ALHE) and Kimura disease are now considered to be distinct disorders both clinically and histologically (table 1) [1-3]. However, features of both diseases have been observed to overlap in some patients [4,5].

How is IVIG used to treat Kimura disease?

Intravenous immunoglobulin (IVIG) was used in one patient as a steroid-sparing agent, and he remained disease free more than 6 years after follow-up. [ 48]

What kind of angiomatous nodule is ALHE?

First described in 1969, ALHE has also been called “epithelioid hemangioma,” “histiocytoid hemangioma,” “inflammatory angiomatous nodule,” “intravenous atypical vascular proliferation,” “papular angioplasia,” “inflammatory arteriovenous hemangioma,” and “pseudopyogenic granuloma” [6,7].