Madisons Foundation - Moms And Dads In Search Of Needed Support

What

Hypersensitivity vasculitis is an abnormal immune reaction against small blood vessels. It comes about when the body makes antibodies against a drug or invading microbe, and complexes of these antibodies become deposited in the blood vessel walls. This leads to fever, rash and arthritis, but if the drug or microbe can be eliminated, the reaction will usually disappear on its own.

Who

Hypersensitivity vasculitis is a disease that can develop at any age, affecting both males and females in equal proportions. This disease appears in about 5 in every 100,000 children. In children, hypersensitivity vasculitis may be confused with Henoch-Schönlein purpura. The diseases are very similar, and may, in fact, overlap somewhat.

Signs and Symptoms

Patients with hypersensitivity vasculitis may experience a variety of symptoms, including the following: Patches of purple discolorations of the skin (purpura) are the most common symptom with the cutaneous (skin) form of this disease. These lesions are usually located on the legs, trunk, or buttocks. Itching, burning, and pain are associated with vasculitis of the skin. In some cases, these lesions may break open (“ulcerate”), forming blisters on the skin. Pain, stiffness, and other joint symptoms may also occur. Organ damage (kidneys, liver) can occur in severe cases.

Possible Causes

Many different drugs have been associated with this syndrome, including antibiotics, phenytoin, allopurinol and others. Respiratory infections, hepatitis, and HIV infection have also been found to be associated with hypersensitivity vasculitis. Although these infections are involved in this disease, it remains uncertain whether they actually cause hypersensitivity vasculitis. Hypersensitivity vasculitis is part of a group of disorders that are called leukocytoclastic vasculitides based on their appearance under the microscope. Other leukocytoclastic vascultitides have different causes, but hypersensitivity vasculitis is caused by a drug or infection by definition.

Diagnosis

Your doctor can diagnose hypersensitivity vasculitis based on the characteristic rash and arthritis in association with infections or medication use. Blood tests are often used to help classify the type of vasculitis and determine the amount of inflammation. Ultimately, a skin biopsy may be performed, where a small tissue sample is taken from the patient. This demonstrates the specific type of inflammation of the blood vessels, which allows it to be definitively identified as hypersensitivity vasculitis.

Treatment

Treatment for hypersensitivity vasculitis is aimed at reducing the inflammation, which can allow the natural healing events in the body to occur. In many cases, if the medication responsible for the disease symptoms is identified, the medication is stopped if possible. Nonsteroidal anti-inflammatory medications such as ibuprofen or naprosyn can be used to help with arthritis and fever, and if the disease is severe, steroids may be used.

Prognosis

Patients with hypersensitivity vasculitis can expect a good prognosis provided that the disease does not involve damage to internal organs. Once the infection passes or the drug is stopped, the disease will usually resolve on its own in a few weeks. Rarely, some children have chronic or recurring problems, but most do not.

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

Medline-plus
A comprehensive, easy to read article on the disorder with nice pictures of the vasculitis as well as good definitions of medical terms.

Johns Hopkins Vasculitis Center
A nice website with information about specific vasculitidies as well as ongoing research.

The Johns Hopkins Vasculitis Center.
Well written information about the disease.

Google Search for Hypersensitivity Vasculitis

References and Sources

www.emedicine.com/derm/topic185.htm www.nlm.nih.gov/medlineplus/ency/article/000874.htm vasculitis.med.jhu.edu/faqs/faqs.html