Chronic angioedema and urticaria: The Strange case of the water management employee...and the Tyranny of IgE
No one likes Dictators or Tyrants. Especially in this country...the Land of the Free and the Home of the Brave, right?. But do you know that one of the smallest tyrants in the world is also the most powerful? Yep, it weighs in at barely 200,000 Daltons....
It's IgE.
Webster's dictionary defines a Tyrant as "an absolute ruler" who "uses power to oppress it's subjects". And it's my contention that most allergists fall under IgE's overly oppressive power to define who--and who not--to treat as allergy patients. Next month I'm going to launch into some allergy aphorisms of mine, and it is from these thoughts about IgE that one of my favorite aphorisms was born: "IgE is a cruel taskmaster". And the following case illustrates this perfectly...
...It was a hot August day in 2007, and I was sitting in my office, trying to mind my own business. The pleasant quiet of the day was abruptly diminished when I heard a "plop". I looked up. My nurse dropped a "new patient" chart on my desk.
Back to work. I put my journal down. The fact that my investigation into reading about Toll-like receptor heterodimer variants that protect from childhood asthma...well, it just would have to wait. So would Adenosine induction of airway hyperresponsiveness through activation of A3 receptors on mast cells. It was difficult to do, but I tore myself away from the JACI. Self-discipine, pure and simple. I had to see the patient. And the JACI would have to wait. Again.
I walked into the room. A pleasant, middle-aged man sat in a chair, next to his wife.
"You've got to help me." he said. "It's been a living nightmare for 3 years. I get swollen lips, eyes, and tongue, and sometimes I break out in hives all over my body. And nobody can help me. Nobody. I heard about you."
He had been worked up at a large midwestern university. I liked that, because the workups are usually thorough, and it leaves me to look into the mundane. And he had been worked up well...Zebra-hunting didn't turn up a thing. Hereditary or acquired C1 esterase inhibitor deficiency had been ruled out, based on normal C4, C1Q,, C1 esterase inhibitor (both functional and nonfunctional), and he had a normal tryptase, and no eosinophilia on CBC. An IgE level was entirely normal. Thyroid studies were normal and anti-thyroid antibodies were negative. Allergy evaluation included negative skin prick testing to a wide panel of seasonal and perennial allergens as well as common foods.
"I'm on a bunch of medications, but they don't really help", he said. He had been on zyrtec, Zantac, prilosec, and Prednisone. The latter was for short bursts, and only transiently helped.
"Any other symptoms?" I asked. "yes, I've got some GERD and some bad post nasal drainage" he replied. "I think dust makes my drainage worse", he said. "I sometimes have so much sinus drainage I can hardly sleep because of my coughing". "Flonase, nasonex, Astelin--I've tried them all, and they don't touch it" he said.
Another one of my axioms (aphorisms) popped into my mind--that is, the patient with upper respiratory drainage from presumed aeroallergens who also suffers from GERD has a food sensitivity until proven otherwise. It's been my experience that in diagnostic conundrums, two distinct possibilities often emerge: the patient either has a "Zebra" (i.e., a rare disease), or he has a horse painted with black-and-white stripes (i.e., a common disease with rare manifestations). The latter possibility took shape with my patient...so in view of the above, we talked some more. ..Specifically, we talked about his diet...
"You know," he said, "I had something really strange happen in Colorado a couple months ago. I went into a coffee shop, ate an organic bran muffin, and then seemed to immediately swell up and have hives. Last night, while driving to La Crosse to see you, I ate at a local restaurant here. I had the same experience".
"What did you eat?" I asked.
"Well, I had a sirloin, baked potato, salad, and bread from the bread basket", he said. (Italics mine).
"How do you do with beer?" I asked. "Well, my sinuses get worse with it so I don't drink it any more" he said.
Test results:
ID tests: dust mite: 9 mm dil 1
alternaria: 10 mm dil 1
aspergillus: 9 mm dil 1
penicillium 9 mm dil 1
all other test results negative
RAST tests:
IgE: milk, wheat, oat, corn, beef, baker's yeast, gluten: all negative. 0.00 IU/ml IgE
IgE: milk, wheat, oat, corn, beef, baker's yeast, gluten: all negative
Discussion:
My working diagnosis was that this patient suffered from a combination of non-IgE mediated food sensitivities, coupled with minor prick test-negative but mild ID-positive inhalant sensitivity to dust and mold. The combination of these contributed to his three major presenting symptoms: urticaria/angioedema, chronic PND, and GERD.
Treatment:
We placed this patient on the following program:
1. Rotary Diversified Elimination diet, avoiding major food suspects (wheat, dairy, corn, sugars, yeast, beef)
2. SLIT for dust and mold
3. Temporary continuation of his medications as previously prescribed
4. Trial of Gastrocrom for restaurant meals only
Clinical Course
Within 48 hours of beginning the diet, his daily urticarial lesions and facial swelling began to subside. His stomach began to feel better, and he went off his prilosec. He reduced his zantac by 50%. He used Gastrocrom when eating out, since this seemed to block reactions when eating at Red Lobster. I last saw him 2 weeks ago. His congestion was alot better on SLIT, and dust wasn't bothering him like before. He was afraid to stop his Zyrtec and Zantac, but...Comments:
I've said it before, but I'll say it again. The most valuable diagnostic ally in the allergists armamentarium isn't a skin test or a blood test...it's a good clinical history coupled with a healthy sense of curiosity. When this patient told me about the curious incident involving eating a whole bran muffin, and noting an immediate reaction, I began to think along the lines of a non-IgE mediated food sensitivity. If we eat a food, it is in our system (assuming a normal GI transit time) of about 3 and 1/2 to four days. So if this patient is having wheat products daily, he always has a constant "load" of wheat in his system--which fluctuates from day to day. Walter Vaughn wrote in his textbook that he had a patient who could eat wheat twice weekly, but not daily--otherwise she would have symptoms. His prior allergists had shut out the possibility of extrinsic factors triggering a reaction--since his total IgE was normal. No IgE? No reaction, right? Wrong. ...
...
George F Kroker MD FACAAI
in Case Histories
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Reader Comments (1)
Great case George. Yes indeed IgE is a cruel task master but then again so is RAST testing since it is only 75-85% sensitive. Sometimes a negative RAST is misleading. In the face of an apparent sensitivity - the symptoms got better on an elimination/ rotation diet - I would give more credence to clinical observation. There is more to allergy than IgE but there is also more to allergy testing than RAST.