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Saturday, Jul 05, 2008 | 01:56 PM
Madisons Foundation - Moms And Dads In Search Of Needed Support
Episodic angioedema with eosinophilia
Gleich syndrome, cyclic angioedema associated with eosinophilia
Sunday, 14 August 2005
Last Updated Sunday, 14 August 2005
What
Episodic angioedema with eosinophilia is an immunological condition that involves recurrent episodes of edema (swelling) of the face, trunk and the extremities, weight gain, and itchiness. The swelling can be debilitating, especially when it involves the face and hands; however it can be treated with prednisone, a corticosteroid drug.Who
This condition is very rare with only about 20 cases reported. It affects both males and females. The age range has been from 2 to 40 years old.Signs and Symptoms
Patients with this condition have episodes of swelling in their face and extremities. The individual episodes may resolve on their own, but can recur, and the length and frequency of the episodes can vary from person to person. People affected by this condition will also gain weight during the episodes, and can have fever, itchiness and hives. Laboratory results show eosinophilia (high numbers of blood cells called eosinophils) in the blood and skin. Eosinophils are a type of white blood cell. They are important components of the immune system, which is the body’s protection against infection. They contain granules that have destructive chemicals and are involved in allergies, fighting parasitic infections, and destroying abnormal cells. Patients may also have increased blood levels of IgM and IgE. IgM and IgE are immunoglobulins, proteins made by white blood cells. They attach to foreign objects in the body, such as bacteria, and help in their destruction. The reason they are elevated in this syndrome is not known.Possible Causes
Currently, the cause of Gleich syndrome is not completely known. The current theory is that a yet unknown event activates eosinophils, and they localize to the skin. There they release their granules. Their chemicals induce other inflammatory cells (mast cells) to release their own chemicals which include histamine. histamine causes the blood vessels to dilate, and increases the permeability of the vessels. Water is allowed to move freely from the vessels into the surrounding tissue like the skin. This is what causes the swelling in angioedema.Diagnosis
Diagnosis is made by the clinical symptoms of edema, the laboratory findings of eosinophilia, and by responsiveness to corticosteroid, with no other organ involvement.Treatment
Symptoms of episodic angioedema frequently resolve without treatment. In the scientific literature, there are cases where prednisone, a corticosteroid similar to our naturally occurring hydrocortisone, resolved the symptoms of many patients. However, some patients who did not receive prednisone during ongoing episodic angioedema also recovered well. Prednisone has many side-effects, therefore their long-term, continuous use is not recommended, but rather it is used to alleviate the episodes of edema short-term. Prednisone cannot be discontinued suddenly and therefore it is taken under the care of a physician who will taper the amount over a period of time until it can be stopped. Other medications can be used, including infusions of immune globulin, if there is too much of a requirement for prednisone.Prognosis
All reported patients have had an excellent prognosis. When the recurrences of edema occur prednisone therapy is used to alleviate severe episodes of swelling. It is important that prednisone therapy be closely monitored to ensure that the amount being used is appropriate and that there are not complications from the medicine.Connect with other parents
In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.Weblinks
Episodic angioedema with eosinophilia is a very rare disease and information outside of the academic setting is not readily available.
Hereditary Angioedema Association
This is the website for the United States Hereditary Angioedema Association. Unlike episodic angioedema with eosinophilia this condition is hereditary, severe, and it is caused by a known blood protein deficiency. However, it does provide information on the symptoms of edema and has links to a clinical trial database.