I have a tentative dx of TMEP and am looking for a knowledgable dermatologist in the Phila or NYC area.  Meanwhile I thought perhaps someone could help me understand my pathology reports.  I had an EGD done for esophagitis before TMEP was suspected.  I also had a skin biopsy done after seeing a dermatologist for a pinpoint, non-itching rash on my stomach.  The derm thought the rash looked like TMEP, so I called the GI dr. to ask him to have someone take another look at my biopsy from the EGD.  This is what that report states:  "DX:  GE junction, mucosal biopsy: GE junction type mucosa with moderate chronic, active esophagocarditis. Comment:  Giemsa stain was added on 10/7/2011 after a message was received from Dr. B. that the patient has been tentatively diagnosed by a dermatologist as having a mast cell disorder.  The Giemsa stain shows a tiny focus of prominent positivity, over 30 cells (presumed mast cells) staining in one (and only one) high power field. (On the original H&E stain, there are scattered eosinophils in this area, which is also consistent with a mast cell disorder.)
This result seems to provide support for the hypothesis of GI involvement by a mast cell disorder, with the following caveats:
-Although I suspect that this is an increase of mast cells compared to a usual case of (non-mastocytic) esophagities, I am not absolutely certain of this, since Giemsa stain is not routinely performed on biopsies from the GE junction or esophagus.
-The tissue sample is quite small.
And from the dermatologist, who tested specifically for TMEP:
TMEP is a possibility, but the density of mast cells is less than that typically encountered in TMEP and other inflammatory cells such as lymphocytes and eosinophils are more numerous.  Therefore, the differential diagnosis includes urticaria/dermal hypersensitivity reaction.
Also:  the sections of skin in which there is a perivascular inflammatory cell infiltrate that consists predominantly of lymphocytes as well as eosinophils and mast cells.  Dilated lymphatics and venules are present, suggestive of dermal edema.  Innumohistochemistry for CD117 demonstrates perivascular and interstitial mast cells, but these are outnumbered by other inflammatory cell types in most areas.

I have no idea what most of this means, but I do know one thing--my rash is NOT hives.  The dermatologist does agree that the hives dx is incorrect.  She prescribed clobetasol and the rash (which I've had for over a year on my stomach) went away almost immediately.  I used the cream a few days after all spots were gone.  Now it is about 10 days later, and the rash is back.  I am frustrated!!!  

Thanks to anyone who read through this LONG note and who might be abe to help me understand it all!

Beverly

Sorry Bev, I've just been SWAMPED!!!  sick too....PERFUME ATTACKS!  


Bev, your doctor is right, there are so very few reports done on the esophagus that it's hard to say that this would be considered anything more than eosinophilic eosophagitis.  However, this is pretty often seen in masto patients, so he's right on the nose.  

Now, what they need to do is run you through those endoscopes again and this time go back through with A GREAT DEAL MORE CARE taking more biopsies within that one section where you are showing this trouble.   Then it must be all submitted for the APPROPRIATE TESTS!!  There are specific markers for testing for the immunohistochemical testing and the best stains are giemsa, toludine blue and tryptase with tryptase being considered the best.   If then there are any focal points of MC aggregates, this is what shows how the MC are whether they are clonal or normal.   However, these tests should also be sumitted for genetic testing to see if there are any genetic defects upon those MCs.  This would give a much clearer picture of your case then.

My son has eosinophilic enterocolitis, it's in his colon.  He had very elevated levels of normally shaped loose MCs, however, they managed to find ONE FOCAL POINT OF AGGREGATED MCs!!  It proved he has mastocytosis.  However, he could not be diagnosed with mastocytic eosinophilic enterocolitis because there was only ONE focal point of aggregates and not 20!  The 20 would then fulfill the diagnosis of mastocytosis - they have the official criteria and without fulfilling that specific criteria it throws his diagnosis over to MCAS.  Understand?a

But what's the basis of your questions, really?   Do you have the more dangerous form of SM?  NO!   TMEP is indeed a form of cutaneous mastocytosis, but does it mean it's systemic yet, not necessarily.  Does it mean it's dangerous, definitely not!  How do we know?   Look at your CBCs and your tryptase levels.  

If your CBCs are showing signs of alterations, this could be due to the masto and it needs checking.  If your tryptase levels are elevated or increase, then this is what reflects the MC burden.   If either of these are altered, then a BMB should be done in order to check if the masto has gone systemic.  

However, do these tests influence your treatment?   Depends.  If your tryptase is not sky high and your CBCs are nice and pretty, then the answer is NO!!  However, if there are alterations, then your doctors have to evaluate your case and see if there are needs for other changes requiring more intervention.  

Yet, Bev, if you don't have doctors jumping all over you and doing flip flops and looking like this ,  then CALM DOWN and BE AT PEACE!!!  You're not in any real danger!!   You will likely die from anything else but masto and only when you're a old lady!!


I hope this helps you!

Hugs

Lisa

Hi Bev!!
Nice to met you, its great you found us There are so many good people--like Lisa... who can help with your questions.

You know those biopsies look pretty recent and most labs in the US store your samples for aprox 2 years--so have your DR order those samples be sent to Dr Castells lab for processing (with a DRs order of course)
Good luck!!

Hugs
Ramona

RAMONA!!!! So good so see you back! How are you?
Kim

Another question--I had my CBC done before the EGD, and it was normal.  I see you can test tryptase either by blood test or from biopsy. Is the blood test reliable?  My guess is that it is TMEP but of course the big question is if it is systemic.  Do I need more biopsies to find that out, or a BMB?  Is there a specifc order of testing that is done; i.e. skin biopsy first, then **** next, etc?