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Question For Those Dxed With Mastocytosis


juliegee
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Hi,

I have a question for anyone DXed with mastocytosis (or for anyone who may know.) I recently and periodically break out with hives even though I've never tested positive for any allergies. With my last out break, I was also very nauseous and had a severe headache. Does this sound like mastocytosis? Once I began with my many antihistimines, the hives, nausea and headache subsided dramatically. I also have POTS and GI motility issues, but the nausea was much worse than normal and not responsive to my normal prokinetic and zofran.

Also, I have tried to read everything I can about mastocytosis and the images often display really dark spots/hives. Mine are flesh colored, maybe a little reddish. Sometimes, they turn into dark spots after they heal. Does any of this sound like it could be mastocytosis? Is a skin biopsy the only way to know for sure? What does this entail? Thanks in advance for any info.

Julie

Julie

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Hi Julie,

I have Systemic Mastocytosis which was diagnosed by skin, bowel, & bone biopsies. Most docs would normally do blood & urine tests first to see if there is increased histamine (urine) & tryptase (blood). In Mastocytosis histamine & tryptase are elevated all the time but in a condition called mast cell activation disorder histamine & tryptase are only elevated during an attack & for a few hours afterward. Mastocytosis is actually very rare but mast cell activation disorder is more common.

Severe headaches, nausea & diarhea are some of the most common symptoms in mastocytosis, but these symptoms also occur with mast cell activation disorder & allergies. Do you get flushing & tachycardia (tachycardia persists even when lying down) Itching? Are the spots/hives there all the time or do they go away completely? There are several types of skin lesions in mastocytosis but they do not go away entirely even with daily use of strong cortisone creams. If you scratch the spots do they swell up & get really red?

The skin biopsy they do is called a punch biopsy, its not huge but is quite deep so has to have one stitch. The bone biopsy is very painful. I recommend you demand the blood & urine tests first before you even consider a skin biopsy.

Hope this helps, I'm happy to answer any other questions you have,

Bronwyn

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Hi Bronwyn,

Thank you so much for such detailed information. I sincerely appreciate it more than you'll ever know. In the past, my allergist has ordered blood work and I don't think my tryptase levels were elevated. In mastocytosis, you say it is ALWAYS elevated; therefore I am guessing I don't have it. But, mast cell activation disorder sounds like a possibility. (Do you know how that's treated?) During this last outbreak, my nausea, headache and fatigue were awful. I could barely function. Once I added the zyrtec and singulair to my ranitidine and benadryl, I felt markedly better.

To answer your questions, during this last attack, my low BP and tachy were worse than normal. Standing for just a short time triggered this, but sitting or lying down relieved it. (As did the antihistimines.) And, my hives come in all varieties. Some of my hives turn into dark permanent dark freckles. Some look like large (boulder size-as in marbles:-) boils. Some are more traditional hives like bug bites and itch like the dickens. Yes, if I touch or scratch, they enlarge and redden.

The interplay between autonomic problems and mast cell problems seems difficult to untangle. As long as you keep on your antihistimines, does your POTS, etc saty under control? Did you develop this as a child or as an adult? One last question, my Mom and Grandmother also suffer from hives and orthostatic intolerance. Do mast cell problems tend to run in families?

Thank again, Boronwyn, you're an angel!

Julie

Hi Julie,

I have Systemic Mastocytosis which was diagnosed by skin, bowel, & bone biopsies. Most docs would normally do blood & urine tests first to see if there is increased histamine (urine) & tryptase (blood). In Mastocytosis histamine & tryptase are elevated all the time but in a condition called mast cell activation disorder histamine & tryptase are only elevated during an attack & for a few hours afterward. Mastocytosis is actually very rare but mast cell activation disorder is more common.

Severe headaches, nausea & diarhea are some of the most common symptoms in mastocytosis, but these symptoms also occur with mast cell activation disorder & allergies. Do you get flushing & tachycardia (tachycardia persists even when lying down) Itching? Are the spots/hives there all the time or do they go away completely? There are several types of skin lesions in mastocytosis but they do not go away entirely even with daily use of strong cortisone creams. If you scratch the spots do they swell up & get really red?

The skin biopsy they do is called a punch biopsy, its not huge but is quite deep so has to have one stitch. The bone biopsy is very painful. I recommend you demand the blood & urine tests first before you even consider a skin biopsy.

Hope this helps, I'm happy to answer any other questions you have,

Bronwyn

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Hi Julie,

The information that I have read suggests that Tryptase is always elevated in Systemic Mastocytosis, but as with all science there are probably exceptions. Early in disease there may be normal levels of tryptase at some times, also some specialists think that in cutaneous mastocytosis tryptase can be within normal limits when mast cells aren't active. There is also some disagreement amongst research about what the upper limit of normal tryptase should be. Last year research was published by an Australian group that tested over 2000 blood samples, that concluded that the upper limit of normal tryptase should be 7 not the current 13.5. Some Australian hospitals have now adopted 7 as the new upper limit.

The treatments for mast cell activation disorder and mastocytosis are similar, in that medications are aimed at alleviating the symptoms. Usually this involves taking both H1 & H2 Antihistamines (Benadryl, Zyrtec, Fexofenadine H1) (Periactin, Ranitidine H2); Mast cell stabilisors (sodium cromoglycate, ketotifen); Leuketrine antagonists (Singulair); Proton pump inhibitors, which are used to reduce gastric acid (Omeprazole, Losec) & if required anti-inflammatory &/or pain medication.

The trick to most of the meds is to take them every day & not just when you start getting symptoms, that way you block the histamine etc before it can cause the symptoms. Your already on H1, H2 & singulair so your on the right track. I suggest you talk to your doctor about the others & maybe see if you can get your tryptase checked again. I'm not sure about the availability of some of the above meds in the US or their names. My doctor has a couple of my meds ordered in from the UK & NZ.

Ketotifen works fantastically - no itch, no headache BUT I gained 5kg in 5 weeks on it. Almost everyone who takes it gains weight, it slows the metabolism down. Even when I cut the dose in half I continued to gain weight. I eventually stopped taking it because I'd rather be itchy with a headache than obese.

You may also want to consider some topical cortisone cream for the hives, I also use lots of sorbolene cream which I sometimes keep in the fridge - very soothing on itchy red spots.

Mastocytosis does not usually run in families but I don't know about activation disorders, allergies etc. As for autonomic stuff tangled up with mast cell stuff I'm definitely a bit twisted :)

I also have Autoimmune Autonomic Neuropathy. I developed both conditions as an adult & both have continued to get worse. Some symptoms are well controlled by meds & some are not & some days are ok & some days are horrible.

Ok your probably asleep now, I've dribbled on here for ever, feel free to ask more questions but please remember I'm not a doctor & I do suffer from brain fog so my advice has no guarantees. :D

Bronwyn

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Hi Bronwyn,

You are a font of information and I really appreciate it! I've taken something like your Ketotifen....Doxepin here. It's not the same thing, but I had the same reaction you did. It stopped the hives (taken with all the rest of the anti-H's,) but it caused me to put on serious weight. and it made me really sleepy. But, I took it at night so that was OK. I still have some around here....that may be my next step.

I've been putting cortisone cream on my face. Wow! that really helps, but others have mentioned that it's not good to use on face skin...oops. I need relief somehow.

I also have an autonomic neuropathy afecting my tum and bowels- they don't work without meds now. How do you know yours is an autoimmune autonomic neuropathy? Dumb question- sorry. I'm also sorry that you're not able to control all of your symptoms and things are getting worse for you. These hives are intermittent for me, but chronic and they drive me CRAZY. I can't imagine having to deal with this all of the time.

You are a dear, Zoogle, and remain in my prayers. I may have more questions for you as I progress with testing etc. to get to the bottom of things.

Hugs-

Julie

Hi Julie,

The information that I have read suggests that Tryptase is always elevated in Systemic Mastocytosis, but as with all science there are probably exceptions. Early in disease there may be normal levels of tryptase at some times, also some specialists think that in cutaneous mastocytosis tryptase can be within normal limits when mast cells aren't active. There is also some disagreement amongst research about what the upper limit of normal tryptase should be. Last year research was published by an Australian group that tested over 2000 blood samples, that concluded that the upper limit of normal tryptase should be 7 not the current 13.5. Some Australian hospitals have now adopted 7 as the new upper limit.

The treatments for mast cell activation disorder and mastocytosis are similar, in that medications are aimed at alleviating the symptoms. Usually this involves taking both H1 & H2 Antihistamines (Benadryl, Zyrtec, Fexofenadine H1) (Periactin, Ranitidine H2); Mast cell stabilisors (sodium cromoglycate, ketotifen); Leuketrine antagonists (Singulair); Proton pump inhibitors, which are used to reduce gastric acid (Omeprazole, Losec) & if required anti-inflammatory &/or pain medication.

The trick to most of the meds is to take them every day & not just when you start getting symptoms, that way you block the histamine etc before it can cause the symptoms. Your already on H1, H2 & singulair so your on the right track. I suggest you talk to your doctor about the others & maybe see if you can get your tryptase checked again. I'm not sure about the availability of some of the above meds in the US or their names. My doctor has a couple of my meds ordered in from the UK & NZ.

Ketotifen works fantastically - no itch, no headache BUT I gained 5kg in 5 weeks on it. Almost everyone who takes it gains weight, it slows the metabolism down. Even when I cut the dose in half I continued to gain weight. I eventually stopped taking it because I'd rather be itchy with a headache than obese.

You may also want to consider some topical cortisone cream for the hives, I also use lots of sorbolene cream which I sometimes keep in the fridge - very soothing on itchy red spots.

Mastocytosis does not usually run in families but I don't know about activation disorders, allergies etc. As for autonomic stuff tangled up with mast cell stuff I'm definitely a bit twisted :(

I also have Autoimmune Autonomic Neuropathy. I developed both conditions as an adult & both have continued to get worse. Some symptoms are well controlled by meds & some are not & some days are ok & some days are horrible.

Ok your probably asleep now, I've dribbled on here for ever, feel free to ask more questions but please remember I'm not a doctor & I do suffer from brain fog so my advice has no guarantees. ;)

Bronwyn

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