2/26/2013
I am having trouble with the exact type and therefore prognosis of a 3 year old female child who has nonpruritic urticaria with cold air exposure since she was 3 months old, initially daily, now 2/week. It lasts as long as cold air exposure lasts, then resolves. No anaphylaxis. Ice cube test is neg x2. Family hx:neg Routine lab ie CBC, metabolic panel is ok, sent for tryptase. No fever, joint pain. She is going to CO, she has been there before and done ok. I have warned Mom to avoid cold water immersion. Is this acquired cold urticaria that typically resolves over time or FACU. The neg ice cube test and nonpruritic hives have me confused. Your help will be appreciated. Thank you.

Thank you for your inquiry.

I am going to ask Dr. Allen Kaplan, who, as you know, is an internationally recognized expert in urticaria in general, and especially in cold urticaria, to respond to your inquiry. We will forward his response to you when we receive it.

In the meantime, here is a website that will give you the names and locations of laboratories that do genetic testing for cold auto-inflammatory syndrome. Although the lack of systemic symptoms, as you noted, mitigates against this disorder, in my limited experience with it, in its earlier phases, I have seen skin manifestations appear initially without the fever or joint pain. So, I feel you may still wish to consider this diagnosis.

As noted above, as soon as we receive a response from Dr. Kaplan, we will forward it to you. Thank you again for your inquiry and we hope this response is helpful to you.

Sincerely,
Phil Lieberman, M.D.

Thank you for the additional information you provided Dr. Kaplan.  We received a response from him. Thank you for your inquiry and we hope this response is helpful to you.

Sincerely,
Phil Lieberman, M.D.

Response from Dr. Allen Kaplan:
Although this is a cold-induced rash, I do not really know what to call it or how to treat it. The rash does not itch, it is only on exposed areas but it disappears so fast, it is hard to know what to call it. Nevertheless, sometimes swelling of the hands is seen when holding something cold which is typical of cold urticaria, and I assume the swelling takes many hours to normalize. On top of that, the ice-cube test is negative. This combination does not correspond to anything I know. There is a systemic cold urticaria that is ice-cube negative, but it spreads and is very pruritic.

Sincerely,
Allen Kaplan, M.D.