Linear IGA Bullous Dermatosis

[nicolespeanut]

Hi Girls,

I am just checking in with good wishes to all.

I know not too far back I corresponded with the Dad of a youngster who was diagnosed with the disease and think of Amanda and her little one.  It might be good to connect the two?  Amanda, let me know if this sounds good- you could send me a note and I could pass it along to the gentleman in hopes that he checks his mail.  I know the last time I checked with him, his son was doing well on the meds.

Fondly,
Nicole

[bunnie]

This comes from a researcher into this disease in the UK, Fenella Wojnarowska, and the paper was published in the British Assoc. of Dermatologists Journal. I was in her care on two occassions. It confirms my statements on this issue. Please note that which is high lighted in red.

Chronic bullous disease of childhood, childhood cicatricial pemphigoid, and linear IgA disease of adults. A comparative study demonstrating clinical and immunopathologic overlap.Wojnarowska F, Marsden RA, Bhogal B, Black MM.
Department of Dermatology, Slade Hospital, Oxford, England.

Linear IgA disease of adults, chronic bullous disease of childhood, and the rare childhood cicatricial pemphigoid currently are regarded as separate clinical entities despite their many shared features. All are sulfone-responsive subepidermal bullous diseases associated with linear IgA deposition at the basement membrane zone.
In this paper we present a long-term study of 25 cases of adult linear IgA disease, 25 cases of chronic bullous disease of childhood, and four cases of childhood cicatricial pemphigoid, which has revealed further similarities among all three groups. The morphology and distribution of the cutaneous and mucosal lesions were similar; mucosal involvement was present in 80% of patients with adult linear IgA disease, 64% of those with chronic bullous disease of childhood, and 100% of those with childhood cicatricial pemphigoid, and ocular scarring affected patients in all groups.
Remission occurred in 64% of those with chronic bullous disease of childhood (the disease was active in 12% after puberty), 48% of those with adult linear IgA disease, and in no cases of childhood cicatricial pemphigoid.
HLA B8 and circulating IgA anti-basement membrane zone antibody were more common in chronic bullous disease of childhood than adult linear IgA disease. There were no absolute differences among the three groups, and we suggest that adult linear IgA disease, chronic bullous disease of childhood, and childhood cicatricial pemphigoid are the same disease, with childhood cicatricial pemphigoid being a more severe form of chronic bullous disease of childhood

mucosal involvement was present in 80% of patients with adult linear IgA disease,

According to my Derm Neil Cox; (information given January 2008)

People with any IgA mediated disease are more likely to have mucosal involvement of their disease, because these tissues line or cover the areas of the body that may come into contact with organisms from outside the body, and it is the IgA antibody which is dominant to these areas, including tears and saliva.

[LIGA girl]

Hi Nicole
good to see you again, I hope you are well, no new blisters or anything. I track my progress in my journal if you are interested.

I was wondering how Grayson and Amanda are going too .... any progress, Amanda?

regards
Vicki

[nicolespeanut]

Thanks girls, I am well.  I am sorry not to check in as often but my life is busy with three children off in three different directions.  I do think of you both often and hope you are well.  I know you have eachother for support but I do make an effort to check in to stay connected.  I will check up on your progress in your journals as well.

I sent a brief note to the dad of the boy to see if he might check in and perhaps link up with Amanda.  I think it would be helpful to know someone else with a little one who has the disease.

Fondly,
Nicole

[CalamityJane]

0h your poor little fellow.  The pics almost had me in tears.  How terribily hard this must be for him, and for you. God bless you for saying you are his advocate. You are, and you will be. What a lovely little chap he seems. Chubby & healthy. I'm so sorry you are having to deal with this in one so very young.

 
Jane

[graysonsmom]

Hello everyone!!  Grayson is doing very well.  I had to take some more pictures last Tuesday because he looked so good - I was just so thrilled to see him almost looking normal again.  The last place he had a really bad outbreak was on his face, and it looked pretty bad last weekend.  But by the beginning of the week almost all of the scabs were gone and there were no new blisters!!

We met with the chief of dermatology at Texas Children's Hospital on the 12th and left there feeling very confident.  He seemed to know what he was talking about, he took some pics of Grayson, and he was teaching a bunch of the residents there about LIGA by having them come in to look at Grayson.  He told us that he thought Grayson was responding well to the meds and that he looked like he would do well with all of this.  So Grayson is still on the dapsone and we are currently tapering him off of the prednisolone.  We go back to see the derm again next month.

Nicole - I would love to get in contact with the man you were speaking of.  That would be great!  Thank you for thinking of me!

Thank you all for your kind words.  I am so glad I found my way here when Grayson was first diagnosed.  I have learned so much here and Bunnie has been extremely helpful and encouraging!

Hope you all are doing well.
Amanda

[LIGA girl]

I am so glad to hear that Grayson is doing so well Amanda, I am glad dapsone is working for him, I could not take it unfortunately. It is funny how when you have LIGA, all the student doctors are brought in and it is the first time they have seen it, in my case anyway. My derm specialises in bullous diseases and I am only the second one she has treated with LIGA.

Nicole, so good to hear from you again, I hope you are still in remission too.
Cheers
LG

[CalamityJane]

Hi Amanda, I was so pleased to read your post, and so relieved to here your sweet boy is so much better. Skin problems are difficult, or can be, but it must be so much worse to see on your young child. His little tummy just melted my heart last night!

Jane

[Uncle Matt]

I too am glad Grayson is doing well.

Hoping for continued improvement

Matt

[bunnie]

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[LIGA girl]

This is interesting bunnie as I have a lot of lesions in my mouth at present, I also get blisters there but not a lot. I also gert them on my tongue at times, I have had this for ages but have not noticed any scarring, the docs always check it too. There is no hope of my finding a dentist who knows all about these things.  I have noticed that I get a quicker build up of plaque than I used to and someone suggested to me that I may have Sjogrens as a result. I have also heard of a young woman with lupus who has lost all of her teeth after a few (3 I think) years with the disease, I am not sure what it did to her gums ....
LG

[bunnie]

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[bunnie]

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[LIGA girl]

I can imagine that the docs would rarely see the intact blisters in our mouths, Bunnie, if I get blisters in my mouth I only get one or two and they only last about half a day before they pop. My mouth is really worse than it has ever been now though it doesnt trouble me much I dont eat those things that would aggravate it.

[bunnie]

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[LIGA girl]

I can't eat crisps, salads, citrus fruits

I am the same Bunnie. though if I use soft things like lettuce in salad it is OK, I squeeze oranges and have the juice adn crisps I  can only eat when I dont have meny lesions but I dont eat them much anyway.

[bunnie]

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[bunnie]

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[LIGA girl]

That is interesting, my gum involvement has not reached a bad level yet, it keeps moving round my mouth and does not bother me much. The worst thing at the moment is my hands as most of my blistering is on my palms and fingers and hurts when I move them much.

[bunnie]

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