Hi Graysons mom! I'm so happy to hear from you.
We were finally able to taper Grayson off of the prednisolone about 3.5 weeks ago. Now he is just on his dapsone and a couple of steroid ointments for the blisters. I have a call in to the derm though, because he has constantly had many blisters since being off the pred. Not all over like when this first started, but maybe a large outbreak on his leg and several on both arms and around his mouth(or something like that). So I don't know if that is just how it is, or not. I feel like he should be blister-free at least some, but I don't know.
Yes it is like that Amanda. This relapsing or flares once prednisilone has stopped is common in almost everyone, however with blisters now showing in the mucous membranes, I think your derm will rethink the situation, he may increase the dapsone?
At the very first it tends to be scattered, then after a few months it tends to dominate one area. Even on Dapsone outbreaks maybe two a week are normal. I can quote you this from a letter I had from Proffessor Zone a research derm into linear IgA in Canada.
Clinical management should stress maitenance on the smallest dose of Dapsone necessary to control disease. Occassional new lesions (two or three per week) are to be expected on an optimal dapsone dose , and are not an indication for altering drug dosage
The thing is with preds is that once started they become a vicious circle, the disease flaring on cessitation. The derm may suggest adding or changing to another immunomodulator, sulphapyridine, for eg.but I don't know of course, when concerning such a young little one. The thing is Amanda, is that the derm has the difficult job of balancing the long term effects regarding preds against the benefits of taking it, as you know Aliciab can attest to. These are hard decisions Amanda for anybody , 100 times worse when a child is involved, and I would suffer all Graysons blisters myself, if it would free him and you from this,
truly I would.
With regard to the lumps rather than blisters, this appears typical on the scalp and neck, I often have them, in fact I don't think i have ever been really free of them!
About Graysons eyes. I presume the area you are talking about is the lid margin? That is the flat area where the eyelashes grow out of. However to make clear the situation on eyes.
First Amanda it is important to understand that every specialist has their own method of treatment, and few take kindly in a "lay person" saying different to what they believe to be true. This becomes more apparent with very rare conditions, and in such situations often the patient understands more about the situation than they do themselves, not only because they are experiencing it, but more vitally that in order to understand the process , (in this country anyway) the patient is handed round to so many specialists, you wouldn't believe. This is not only to gain knowledge and input from other specialists, but as a learning curve for them too, where they can read first hand exactly who the patient has seen, what was said and done etc., but
primarily to listen and learn from the patient, not only the symptoms but the presentation, and course of events, simply because they have it!
So I tell you here what I personally know to be the case, a)because it happened to me, and b) what all of these specialists
came to know because of my own situation. please note in bold.
Any irritation or blistering, and I stress,
in the eyes or
in the
mouth must be taken very seriously.
Irritation and inflammation of the conjunctiva is the first sign of involvement in the eyes, and what many OPHS aren't aware of (and I'm talking of some corneal specialists too) is that there can be involvement in the eyes without direct evidence or symptoms, it
typically presents as simple inflammation in the first instance, such as conjunctivitis, , and
this is where Ophs are often fooled. Liga is a good mimicer!
The "pink eye" I had lasted on and off for over 7 months , not just one occassion, but then seemed to jump from one irritating situation to another very rapidly. Always, the Oph. should treat it
as if it were liga, because it is highly unlikely that irritation/inflammation in the eyes, (conjunctivitis) when it has not happened before the onset of disease, yet is occurring when the disease is active ,
more than once, re-occurring off and on , could be anything else.
It very first appears, clinically and pathologically as inflammation, in other words simple conjunctivitis. It is
this inflammation that progresses the disease if not treated correctly or early enough. Despite that, if the
inflammation moves a notch up, (
I am not referring to blistering.) often once started, it is difficult to arrest it, however I must add that since my first treatments better drops have been formulated with very good effects.
Blisters were
not present
until much later. Mine first appeared about 9 months from the very beginning of the "pink Eye" situation.
Later on as the inflammation gets worse deposits can be seen with the use of flouresence, a yellow dye, they show up as white dots. It is important to remember that the condition flares and subsides when these tissues are involved too. For eg. I could have irritation which would subside and get better, then on another occassion the inflammation could be much worse and be more difficult to get it down, but the conjunctiva was getting thinner and thinner over the cornea on each occassion, due to the inflammation, (it is thin over the cornea anyway) it was then that the corneas were "caught up" in the inflammation, (as described to me) resulting in the blistering of the corneas.
if a food allergy or sensitivity could make this disease worse?
With regard to allergies. Certainly exactly as you say amanda, an allergy could exascerbate the situation. I had patch tests with various things, a whole line of tiny plasters right up my arm of various things and two or three came back positive. I was tested for food allergies , but have none.
It would show up in his blood specs amanda.Other tests were carried out by actually putting drops into my eyes, every 15 mins over 24 hours, when it was thought I was becoming allergic to them through use. For eg. Chloremphenocol drops for infection. I had used these on loads of occassions but then the drops unexpectedly started to burn and blister my skin in and around the eyes. This controlled test was carried out at the Radcliffe Oxford under Peggy Frith and proff.Bronn, and proved I had
become allergic to it. Cephuroxane drops was another similar test done in Moorfields London, under John Dart. Check out the allergies Amanda, because if Grayson is allergic to something this could be exascerbating the situation , and causing exascerbation within these tissues. If you have an
immune response to an allergy, the fact that one already is experiecing blistering from an
autoimmune response, will naturally produce further blistering. Another example is, Having a predisposition to autoimmunity (even though not active) if such a person has an immune response to an allergy, the manifestation of that response is more likely to manifest as the autoimmune one would, in our case blisters. Regardless Amanda any
indication of an allergy present, any lack of or over production of essential nutrients
will show up. After any noted indication, further explority tests may be required. I hope I explained that well!
One thing I would ask about Amanda is another biopsy. It can be done with just blood if the derm knows how to do it, but as yet as far as I am aware personally, the test is still not readily available, in any country other than Japan. This is due to the complicated and intricate set up in the lab apparantly. It took three weeks for my derm to just have set it up! It took many man hours he told me by everyone just to get this result from my blood samples. I remember they took the samples in a special way too, but I cannot remember the details, however I could get them.
This new test determines if the igA antibody is also affecting the collagen area just below the BMZ, it is actually touching it. If it is, then this shows the reason for the liga not responding to treatment as well as those who have it just in the lamina lucida area. The finding doesn't alter the treatment at all, it simply gives a reason for the disease not responding as well despite various treatments. The other thing it does is to explain why there are often
deep raw areas once a blister has burst. This is because it affects the very upper layers of the dermis. I have actually had blisters on that blood raw skin. When you get an area of a cluster of blisters doing this, you end up with an area similar to third degree burns.
Take a look at the link I left in a previous post to my blog on Linear IgA.
I do trust this offers you some further explanations to your questions Amanda. I fully understand your worries and tiredness from worrying. It is easy to disguise the information, it is far harder to uncover it, but that you see Amanda, this knowledge, is your starter for ten. Nobody is suggesting it will happen, but at least YOU are aware, and this gives you clout if you are ever confronted with the situation.
Please bear in mind also that more than 3/4 of people with liga have mucosal involvement to no serious ill effect.My very best wishes and a big (but gentle hug ) to darling Grayson.
Bunnie
