Chronic Urticaria and mast cell experts in the Northeast

[Zizzle]

Mary Beth and other mast cell experts,

My MIL is SUFFERING right now with a flare up of her Chronic Idiopathic Urticaria and Angioedema. She's been getting these flares every 7 years since she was 12 yrs old and they last for months. The 7 year timing is totally perplexing, but it's year 7 and it's begun. She's on multiple high-dose antihistamines (Allegra, Zyrtec, Doxepin, Zantac) with little relief and has to carry an Epipen since she gets huge hives in her mouth and throat. Her docs in upstate NY are totally worthless and she wants a second opinion. She suggested Hopkins, but I said she needs to go to Dr. Castells in Boston. I called and had them fax the forms to her -- Dr. Castells reviews the records from her local octor and contacts the patient to make the appointment.

During prior flares many years ago, she did see an expert (Dr. Beltrani) who diagnosed excess mast cells on her skin. Earlier this year, I convinced her to request staining for mast cells on her colonoscopy biopsies, and they showed excess mast cells too (although perhaps not enough for a full ME diagnosis). She's taken prednisone in the past, but the hives flare back when she starts to taper. Her derm wants her to skip steroids and try Dapsone (leprosy drug) or Plaquenil (anti-malarial used in several autoimmune skin conditions - I'm on it).

Further reading has me convinced she has autoimmune, not idiopathic, urticaria, because she has multiple risk factors:
-Hashimoto's Thyroiditis
-Gluten Intolerance
-Neuropathy in her fingers and toes
-Malar rash
-Female
-Flares of hives have remissions of several years in between

So my question is, is Dr. Castells the #1 expert on the East Coast to address autoimmune chronic urticaria?? Is there someone better at Hopkins? After all these years, she needs some real answers from the #1 source.

Any other advice to help her while she waits for an appointment? She's doing acupuncture which helps calm her down at least.

Thanks!!

[tex]

Hi Zizzle,

I can't answer your question about the best doctor to deal with this issue, but I have a question for you. Have you (or her) ever considered that she might be reacting to salicylic acid in foods whenever these mast cell issues reach a high point in the cycle. I'm not suggesting that salicylic acid is the primary trigger, I'm just wondering if it could be a secondary issue that contributes to the urticaria once her mast cell levels build up past a certain threshold where a reaction is triggered.

Salicylate Free Diet

Tex

[Zizzle]

Have you (or her) ever considered that she might be reacting to salicylic acid in foods whenever these mast cell issues reach a high point in the cycle?

Ha! Getting her to go gluten free was hard enough. Her husband says she's 98% GF now. I read that many patients with urticaria flares stop using laundry detergent and do all their washing with baking soda alone. I suggested this and she nearly ripped my head off, saying she's in crisis, totally inflammed, and can't be bothered with issues of laundry and what she's eating. Her hives are worse in the morning -- which makes me think her Tide, Arm and Hammer, or other cheap detergents and fabric softeners in her sheets might be aggravating things now that she's hypersensitive. Silly me. I did convince her that she has no business having her nightly cocktails, given how much her liver is working to process the various meds she's on, and becuase of the high histamines in alcohol.

She insists that diet has nothing to do with her 7 year flares because if it did, she'd be flaring all the time. Her acupunturist said she can't help her -- she's too stressed out and inflammed. The acupuncturist thinks it's hormonal. Do our adrenals or other hormone glands reset every 7 years??

[Bifcus16]

Hi Zizzle,

Have a look on medline for CIU and either H Pylori or B12. Separate studies.

I don't have time to hunt them down right now, but there was some stupidly high percentage of CIU patients tested positive for H Pylori, and when it was treated their symptoms went away. B12 deficiency in a smaller number of cases, but still quite high.

Hopefully you could address both without seeing a specialist.

[Zizzle]

Wow, the references are endless. Thanks!

http://www.ncbi.nlm.nih.gov/pubmed/?ter ... licobacter

http://www.ncbi.nlm.nih.gov/pubmed/?ter ... caria+B-12

[Zizzle]

My MIL was given a short-course of prednisone, tapering every 3 days, and is being transitioned to Dapsone. I'm not optimistic.

[mbeezie]

Z,

I think Dr. Castells will be a good resource. My allergist was trained by her and he knows his stuff. If Dr. C turns down her case I would ask them to give you a referral to someone in your area.

If she gets desperate enough she may try low histamine/low salicylate - it's worth a try.

[Zizzle]

It turns out the GI had all the evidence she needs to see Dr. Castells. He handed over her colonic biopsy results which confirmed the ME diagnosis. Apparently normal colonic mast cell counts are under 11, and she had 20, enough for ME. Her 90 yr old dermatologist also claims to have given lectures with Dr. Castells and is completing the paperwork to get her accepted.

She'll start Dapsone this week after her blood results come back. I've been reading up on Dapsone, and was surprised to learn its very effective for my skin condition after anti-malarials fail to control the rash. It should also clear up my MIL's hives quickly. Dapsone is a sulfa-based antimicrobial, but docs attribute its effects to anti-inflammatory properties. I call BS on that. I think it treats these "autoimmune" skin conditions by temporarily killing/decreasing the pathogenic gut bacteria causing them. I had this A-Ha moment when I remembered that I've always enjoyed complete remission of my GI symptoms while taking Bactrim. Now to narrow down which bug that might be...

Incidentally, Dapsone also promptly clears up Dermatitis Herpetiformis. Can anyone explain how an anti-microbial can heal a gluten-induced rash?

[Zizzle]

It seems several antibiotics and sulfa drugs can clear up DH. How can this be? Could sulfasalazine cure my MC and my skin simultaneously??

Dermatologists have used the medications sulfapyridine and tetracycline to treat dermatitis herpetiformis, although not as successfully as with dapsone. In addition, a report from Australia noted that sulfasalazine worked in three patients who couldn't tolerate dapsone, although one patient had to discontinue the drug due to side effects.

http://celiacdisease.about.com/od/medic ... ferers.htm

[tex]

Can anyone explain how an anti-microbial can heal a gluten-induced rash?

I think so. Bear in mind that Dapsone is primarily used to treat leprosy. Please read my post at the following link. This was posted before you joined us, and there are a lot of very interesting responses in that thread, also.

Another Clue That IBDs May Be Caused By A Mycobacterium

Tex

[Zizzle]

Wow Tex, thanks for that thread. I found at least one case of Myositis seemingly caused by Mycobacterium Avium, although the authors believe her immunosuppression invited the infection. However, they retested her samples from her first hospital admission and found the mycobacteria was there all along.

Disseminated Mycobacterium avium infection masquerading as longstanding polymyositis

http://shr.sagepub.com/content/2/12/94.full

Further investigation revealed that this patient had mycobacterial infection on her first admission. The original skin biopsies were re-stained to demonstrate acid fast bacillus. This case demonstrates, for the first time, the presence of Mycobacterium avium in skin, gut and muscle in an immunosuppressed patient and highlights the need for appropriate investigation. We would recommend a high level of suspicion of mycobacterial infection if granulomatous inflammation is seen in the immunosuppressed.