A Letter to a Patient With Unrelenting Hives
June 20, 1995
Dear Ms. G,
Both I and Dr. Kishiyama appreciate your thoughtful letter and diffucult situation. Although chronic urticaria (hives) is a difficult problem to deal with, it is a disorder that we see quite frequently. As you know, it is seen in association with a variety of autoimmune disoders like Lupus. I'll summarize below our approach to this troublesome problem.
First we attempt to determine if there is an underlying infection, autoimmune disorder, enzyme deficiency, thyroid disorder, or malignancy as the cause or contributing factor to the urticaria (in your case we already know that you likely have Systemic Lupus Erythematosis). At the same time we begin treatment with antihistamines, which is the treament of choice for urticaria. Some of our patients require very high doses of these drugs to control their symptoms. In most instances we can control the hives without the use of oral steroids like prednisone.
In one patient we had success in treating resistant urticaria with the antibiotic Dapsone, although there is only a small amount of scientific evidence to support the use of this drug. There is also some limited evidence that a calcium channel blocker (nifedipine) and a drug for gout (colchicine) may be of use in difficult to treat chronic urticaria.
Since you do have an autoimmune disorder, we do have some additional comments. In some cases urticaria may be a manifestation of a certain type of autoimmune vasculitis. This condition is typified by medically resistant hives, which tend to occur in episodes of longer duration. The hives of urticarial vasculitis are clinically indistinguishable from those of chronic idiopathic urticaria. This vasculitis can thus only be diagnosed by biopsy. If this hasn't been done already, we suggest that you ask your physician. Some studies have shown that hydroxychloroquine (Plaquenil) at doses of 200 to 400 mg/day can be helpful if this is the diagnosis. I also think that certain cytotoxic agents have been used for urticarial vasculitis.
Lastly, I would like to inform you of the work done by Dr. Malcolm Greaves in Great Britain. He believes that a significant number of chronic urticaria patients possess an autoantibody (like the anti-nuclear antibodies of Lupus) that stimulates histamine release from certain cells in the skin, leading to urticaria. Patient's can be tested for this by injecting a small amount of their own serum under the skin. If a hive-like reaction takes place at the test site, it is presumed that the patient possesses this antibody. He has reportedly treated approximately 20 such patients with intravenous gammaglobulin with some success. I should stress that chronic urticaria is currently not an approved indication for intravenous gammaglobulin (IVIG). Additionally, no one knows how this drug works in treating autoimmune diseases. Still, IVIG has been used quite successfully in the treatment of other auto-antibody mediated diseases (e.g., immune thrombocytopenic purpura).
Unfortunately, we do not presently have any such trials going on at our institution. I would encourage you or your physician to contact Dr. Greaves directly for some information. It also may be helpful to contact the various manufacturers of IVIG, as they are always looking for new indications for this treatment. A first step might be to refer to Dr. Greaves' article in the New England Journal of Medicine (Hide M, Francis DM, Grattan CEH, Greaves MW, et al. Autoantibodies against the high-affinity IgE receptor as a cause of histamine release in chronic urticaria. NEJM 1993; 328: 1599-1608.)
Finally, you are quite correct in stating that there is a lack of significant research concerning chronic urticaria. However, I hope the information I have provided will prove useful to you and your physicians. Please do not hesitate to contact us if you have any further questions.
Neil Gershman, MD