Wednesday, June 2, 2010

Mast Cell Degranulation

Mastocytosis and Other Hive Disorders

One of the problems I have been tentatively diagnosed with in the last few years is mastocytosis, or a mast cell activation disorder (MCAD).  My gastroenterologist and my original immunologist thought it might be a possiblity, since the symptoms include headache AND digestive disorders AND skin reactions.  The immunologist thought I have a mastocytoma on my nose (I call it my permanent hive!) in addition to some of my permanent hive spots.

My Kansas City immunologist was not so sure.  She didn't rule out mastocytosis, and pushing on my red spots did induce hives elsewhere, but decided to treat the autoimmune component of my hives first to see if that was the cure all I needed.  I definitely showed autoimmune issues in my blood tests there, but the tryptase levels (used to measure the leakiness of the immature mast cells) were in normal range.

I started thinking about this again partly because I am changing immunologists if I like the new local guy (my original local doc died of an aneurysm at a very young age) and partly because I think the flexeril Dr. SassyPants prescribed is making me break out in hives and blisters.  This evening even my lips are little puffy and the inside of my nose and ears is itchy - generally not a good sign.  Unfavorable reactions to muscle relaxers can be a characteristic of mastocytosis, but I get so many unfavorable reactions to so many medications it gets hard to figure what is causing it!

One of the talented members (Lisa Sterling) of  The Mastocytosis Society web group I belong to thought my description of my spotted appearance would make a good story, and wrote a children's book about mastocytosis using my psuedonym for the web (Winny Ninny PooPoo) as the name of the lead character,  who is a child with spots.  There are many children afflicted with this disease and it can be disfiguring and painful, and this book was written to be used by doctors and families to explain the disease to a striken child and perhaps to the child's classmates/friends.  There is even a spotted Winny Ninny PooPoo doll that can be purchased with the book, and a companion coloring book!

Like some headache patients, many masto sufferers have spent years trying to get a diagnosis for their condition.  The symptoms are varied and differ in intensity from one person to another.  Some just have the cutaneous version (hives, red spots, Urticaria Pigmentosa, and /or Telangiectasia Macularis Eruptiva Perstans), some have the systemic version which can include all the cutaneous problems and also involves more than one body system (skin, liver, intestines, etc.).  Children seldom have the systemic version; it is more common among adults with this syndrome.  The systemic version can evolve into more agressive and possibly cancerous versions that involve blood cell production.  "Brain fog" and fatigue are often symptoms but are difficult to diagnose.

A mastocytoma is an abnormal collection of mast cells in one place, and rubbing that spot can cause hives to pop up and the mastocytoma to flare up also.  The histamine released by the constantly degranulating mast cells is thought to be the cause of the headaches associated with these disorders.  Still considered a rare disease, experts believe it may be underdiagnosed in the general population.  Bone marrow testing and biopsies of my lesions could definitely confirm the diagnosis or rule it out, but I'm not keen on having the top of my nose removed or getting my pelvic bone pinched.

I will discontinue the flexeril for a few days and re-challenge myself to make sure that it is the cause of my hive exacerbation.  Just call me itchy until then!!!  Am on the road tomorrow, back to a client for followup!  Sigh....


  1. I hope stopping the Flexeril gives you an answer about the hives. It's frustrating that everything has to be so complicated, isn't it?

  2. Life would be soooo simple if we just had a single pill that could cure anything. sigh...

  3. Oh dear one, I do not want to make light of this post but at a quick glance instead of seeing mastocytosis, I saw motorcycle. I do not know where my brain is at.

    Anyway dear one, I do hope you find a solution to this. I know you suffer with so much. Always thinking about you. Blessings.

  4. JBR - made me laugh imagining driving a motorcycle full speed itching all over! Have motorcycled into some bugs in my time and had to disrobe quickly to remove said bugs from my shirt and they definitely were itchy!!!

    I don't really feel like I suffer. Mostly I think I endure. There's a difference at least in my mind!!! :)

  5. I thought it was so cool that she used your story in her children's book. I hope it is able to help some of the kids. The book, pictures and dolls are neat. I hope you get some relief soon.

  6. I agree MP!

    This hive attack has been with me since 2007 (predating The Headache), it waxes and wanes. I complain, change my meds, it gets better. Then I slack off or change my meds again it gets worse. sigh.

    I thought the author was so talented! She had this vision, and made it come true, and got it published and distributed through the organization. And she is a systemic mastocytosis sufferer herself - which can be very disabling. I admire people with a creative drive like this!