EE and/or Mast Cell Disorders in Adults

[Ciel]

Thanks StridAst. You are remarkably good at explaining things in a way that is easier to understand. Thank you so much...I'm very glad that you are here (though sorry that you have need to know all this stuff).

I'm working on reading the paper you recommended. A lot sounds right, but also makes me think that perhaps I'm just crazy and desperately trying to make things "fit" just to have an answer.

I would say I am naturally flexible, but not to any extremes. I was always naturally athletic as well and they seemed to go together. You would never guess that looking at me now though with so much weight gain. Even now, my yoga teacher sometimes comments that I am surprisingly more flexible than I look.

I mentioned headaches and facial pain - that's from TMJ and causes pain on the right side of my head and face that literally never goes away. Thankfully it varies in intensity so it's not always at migraine level. The nerve behind my eye is chronically inflamed and makes it painful to move that eye. I have had episodes of occipital and trigeminal neuralgia, recurrent ear infections and sinus inflammation, tooth and jaw pain that are all attributed to the TMJ.

My joints aren't particularly weak and I've only ever had one sprain and that was while the ankle was already healing from a more serious injury. Both of my sisters can hyperextend their elbows and one can also do it to her knees. One sister has broken both elbows and the other has had knees problems. My thumbs are the only joints of mine that extend a little further than average but I can' Touch my wrist or anything like that. My hips seem to get out of place fairly often, always with walking longer distances or over uneven terrain like snow or sand. I have issues with repetitive motion in my hand and wrists as well.

On that test I only score a 2, possibly 3.

I looked up MCAS testing in my area. It is not covered by the province and looks to cost over $1000.


Typing this stuff out is helping me organize myself mentally. One good thing out of my long-windedness!

[Ciel]

So, for example, this morning.

I was outside for about 45 min. It's cold, but not obscenely cold. Temp is -8 / feels like -13 with the windchill and it is snowing lightly. Not too windy.

I was walking, and not even very fast. I have hives under my chin, both cheeks and one earlobe. And on my rear end, my thighs and my shoulders/upper arms.

I was wearing 2 layers plus my jacket, mittens, a hat and a scarf.

[StridAst]

Yeah,  that's definitely not just an allergy.  If the only odd allergic type reaction you got was cold, than it's less suggestive of mast cell issues.   Throw in the exercise induced anaphylaxis and the other minor reactions and the pattern is there.  Tests can answer it best,  but that price tag is brutal.  

Something to consider is the treatment for mast cell disease is two types of antihistamines.  H1 and H2 types together.  Zyrtec for the H1 and Zantac for the H2 are the most common.  While they are over the counter drugs, the dosages are typically not OTC normal doses.  (I weigh 82kg and my immunologist has me take 1-2 Zyrtec twice daily as an example.  I didn't see much benefit at 1 Zyrtec per day.  At the max dose of 4 per day 95% of the normally constant unrelenting itching I used to deal with is gone.  The major flares (anxiety, nausea, severe diarrhea, sweating, fatigue, flushing, asthma, dry mouth, etc) that I have had since the high doses were started are much milder.  (They still suck though). 

There's also quercetin.  It's an OTC supplement and a known mast cell stabilizer.  So between Zyrtec, Zantac, and quercetin, there are 3 OTC things you could take to help with symptoms.  Talk to a doctor before trying anything if you are taking any prescriptions.  As an example, if you are on any proton pump inhibitors (like Prilosec) for the GI stuff, you don't want a H2 antihistamine and PPI both.

[Ciel]

I'm so glad to hear that combo is helping you! But yeah, definitely still sucks.

I am on pantoprazole magnesium 40mg twice daily and still have breakthrough symptoms. My GI doctor just prescribed dexlansoprazole 60mg once daily (extended release) but I haven't tried it because it's not covered and my current financial situation is beyond tight.

My GP once told me to take Zantac (H2) when I have breakthrough symptoms because it's a different class of medication than the PPIs. She recommended Gaviscon as well. I have done the Gaviscon but I don't think I have tried adding Zantac so I need to check the safety of that again. Thanks for the reminder!

I was on Reactine (cetirizine - I think that is the same as Zyrtec) once daily for a long time but ended up stopping because it wasn't really doing anything and I have a handful of other meds I take daily.

Maybe it's time to revisit the options with my doctor.



Re: dermatographia, I'm unclear if I have it or not. If there is a spectrum of severity then I probably do have a mild version. I don't have anything near to the photos of skin writing/art that you can see online. A superficial cut or scratch usually welts up just at the site of the cut. We called them wormy cuts when I was little, lol. The pen scratch test usually produces a bright red mark that lasts for at least 30 minutes. Sometimes it gets slightly raised but doesn't spread and isn't very itchy at all, so I don't know what that means. If I have a bigger injury to my skin I often develop a blistery rash around the site or edges of the cut or scrape or blister while it is healing, and it takes forever to heal. I thought it was the bandaids because I do react to the adhesives over time, as well as latex but it happens when I don't wear a bandaid at all. When I had surgery I had tegraderm on the incisions for a week and ended up with the same blistery rash, but more welty and it spread well beyond where the adhesive was in contact with my skin.

However, skin prick tests always come back 100% negative, sometimes the control is also negative and if it does react it's very slight.

In summary: I am a conundrum.


But things might be starting to make some sense. I'm just worried my doctor will think I'm making things up or something because I keep asking about different things.

StridAst, you have been more helpful than I can express. I am so grateful.

[Ciel]

I've Spent the last few hours going over the diagnostic criteria for EDS.

On one hand, it seems to neatly connect many of my issues. On the other hand, I still have no clue.

Of the list of 100 diagnostic criteria at Ehlers Danlos for Dummies;

38 - yes
22 - possible/probable
40 - no


Still working on that 77 page article on MCAS.

Sigh.

[StridAst]

The EDS is mostly a possible connection if there is family involvement.  Mostly something to consider if there is family history of joint complications.  I strongly suspect it with my mom's side of our family, but nobody in our family is diagnosed.  Worth looking into if you feel like you fit, but it's one of those things that might or might not be present. (Though if present it would *heavily* reinforce a case for MCAS.)

Re: dermatographia.  On page 10 of that 77 page document it lists the way dermatographia tends to present in MCAS patients:

Another very common dermatologic finding in MCAS patients is dermatographism. [48]
Sometimes simple removal of clothing makes it apparent, but integrating a simple light
scratch test into the physician's standard physical exam is an easy thing to do. The
dermatographism of MCAS rarely is so vigorous as to manifest hives; instead, usually only
erythroderma is seen in the track of the scratch, arising within 1-30 seconds and often
persisting in full splendor for 5, 10, or even 15 minutes or longer.

Your description fits that easily.  It's a mild form of dermatographia, but the mild form is what's common with MCAS.  (Though far from universal). Also, this is the same way my own skin responds.  Including cuts and especially scrapes.  Just red marks, but rarely raised hives.  (Though occasionally mine do the hive thing, but usually just when something breaks my skin). Mine itch, but then I itch all the time and at the slightest provocation.

[Ciel]

Oh yes, that does sound like me, including the part where you say you sometimes get raised welt with broken skin.

I already had a doctor's appointment next week so I will talk to her then.

My mind is overloaded and I am feeling overwhelmed so I'm going to try to stop thinking too much until I see the GP.

I'm most worried that I am so desperate for an answer that I am subconsciously squeezing and twisting my thoughts to make the pieces seem to feet. I'm not really trusting my objectivity right now.

[StridAst]

Yeah, I totally know the feeling.  It's hard to be objective when you want answers so much. Good luck!  And I'm sorry you are feeling overwhelmed. 

[spacecanada]

With my thyroid issue declared independent of my dysphagia and fatigue, we're back to investigating for EoE or MCAS. My dysphagia improves with extra strength antihistamines but can become quite severe without them, even causing complete choking (no air). Biopsies for EoE in 2017 were negative. Here's hoping for answers -- again.

[GoingNuts]

Good luck SC.

[spacecanada]

Has anyone ever heard of environmental allergies triggering EoE? The internet says it can make you more susceptible, but I hardly even get a runny nose from my environmental allergies.

Other than rice cakes made in a top-10-free facility, I don't know what else triggers it but it is mostly constant. The investigation continues. Waiting for a reassessment at the allergist first.