Linear IGA Bullous Dermatosis

[CalamityJane]

Happy to hear you are doing better bunnie.

I think when you are alone you appreciate that communication more. Thank You.

I agree with that, I'm the same.

[LIGA girl]

Bunnie, the cream I use is Novasone and it's 0.1% cortisone.  Glad you are feeling better now, it sounds awful what you had, I have not had any infections with my lesions to date, I am surprised that something like that hasnt happened tho ...  LG

[bunnie]

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[Pariah]

 

[graysonsmom]

Hello everyone!  I know I have been away for a couple months now - life has been too busy for me lately.  Grayson became a big brother on Dec. 14.  So far he has been great with his little sister - he is very proud of her and loves to give her kisses!  My dh has had to work non-stop the past couple months (for instance his last day off was Christmas day), essentially making me a single mom and way too tired at night to think or come on here or anything.  I hope everyone is doing well.

It has been a continuous up and down with Grayson regarding the LIGA.  We haven't yet been successful in tapering him off the prednisolone, which worries me.  I hate that he's still on it, but his derm assures me that he's on a small enough dose that he should not suffer any side-effects.  I don't know.    Right now he's on 3ml, which is the smallest amount he's been on in about 4 months.  Previously, anytime we would get him down to 3.5ml, he would start getting a really bad outbreak.  This time he seemed to do okay at 3.5 (yay!), so now he's at 3, but I am starting to see several blisters forming.  He's also been sick this week with a little virus and a really bad cough, poor kid!  Other than that, he is perfect!  Just a growing little boy.  He will be 2 on March 1, so he's at a very fun age right now!

Bunnie - I was just reading your latest posts after I started writing.  I'm so sorry you've been in such discomfort lately.  I can't even imagine.  I'm glad you have had a little bit of relief now.  I hope you are able to feel normal again soon.

[Uncle Matt]

Hi there Grasyon's Mum!!

I'm glad Grayson's LIgA hasn't got worse. I guess the tapering off of the prednisolone will just take time.

Kindest Regards,

Matt

[bunnie]

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[bunnie]

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[LIGA girl]

Hi Graysonsmom,

good to see you back again and great to hear your wonderful news: congratulations on the birth of your daughter, you have been busy!  It sounds as if Grayson is going well, 3mg of prednisone is a tiny dose, although I know how distressing it is for you to know he has to take it. My derm has talked of children she has treated with LIGA and she says there is no option for them but to take the drugs, it is the best for them in the long run. He is tolerating them well, from the sounds of it he is a healthy and happy boy! I use steroid cream as well 2x daily and that allows me to lower the dose of oral steroid a little, you may be able to try that on him with his derm's approval, it is better than the systemic drug in many ways.

The article Bunnie linked above mentions mycophenylate mofetil and I am on that one at the moment and I have to say I have had better results with it than with any other drug and without any side effect that I know of either. And don't forget that the prognosis for LIGA of childhood is very good, he will most likely grow out of it completely.

Cheers
LG

[bunnie]

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[CalamityJane]

Many congrats on the birth of your daughter. It's good to see you back here. I think we all followed Graysons story, with our minds, and especially our hearts!

Jane

[graysonsmom]

Congratulations amanda on the birth of your baby daughter! What is her name?

Her name is Bailey.  Grayson is so sweet with her and is always talking about "baby baby" (baby Bailey) or "baby bear" (Bailey bear)!  

Thank you for the article, that is very interesting and I had not seen or heard of that before.  I had read about Flucloxicillin before but if I remember correctly it is a type of penicillin and Grayson is allergic.  I could be wrong though.  I'm actually really surprised at how well he's doing so far on the 3mL dose of pred.  He's been on it for a week now and had just a couple blisters form and that's it.  I will call the derm tomorrow to update him and see what he wants us to do next and I'm hoping he has us stay at 3 for 1 more week before going down again.

Oh, and LG, we are using a steroid cream as well.  2 times a day on any blisters on his arms and legs - right now just a couple on his legs.  It does seem to help the blisters heal much quicker!

Thank you all for your kind thoughts!  It's so nice to have a place to turn to with so much support. 

[bunnie]

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[bunnie]

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[bunnie]

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[bunnie]

Hi LG, I see you have started the ritixumab. I noticed too that they had to slow down the drip to double time. This happened to me you may remember me saying, when I was on the Alpha globin IV and the Methyl Pred IV. They always had to give me the treatment over double the normal time to stop reactions.
I am nearly finished an article on autoimmunity and T cells ,and the forefront of research is in the regulatory or suppressor Tcells. Have you seen this , it's very promising! and I sincerely hope this treatment does the job!
Ritixumab
Ritixumab is a chimeric mouse/human monoclonal antibody that acts against the B cell antigen, CD20. This antigen is expressed by all B cells until they differentiate into mature plasma cells,12 and treatment with rituximab results in a marked, sustained reduction in the number of both normal and malignant B cells.13,14 Rituximab was, therefore, initially developed for the treatment of B cell lymphomas.15,16 However, very recently, this agent has been used successfully to treat conditions that have been traditionally considered as being predominantly T cell-mediated autoimmune diseases, such as RA, systemic lupus erythematosus (SLE) and Wegener’s granulomatosis.

Edwards et al. recently published results of a randomized controlled study with 161 patients with active RA, despite treatment with methotrexate, showing that a significantly greater proportion of patients treated with rituximab had a 50% improvement in disease activity in comparison with those treated with methotrexate alone; greatest improvement was seen in those treated with two infusions of rituximab (1000 mg) in combination with methotrexate.17 What was most impressive in this trial was that the effect of treatment in disease activity was sustained over the 24 weeks of study follow up without any need for further re-treatments with rituximab. A dose-escalation, open-label trial conducted by Looney et al. in 18 patients with clinically active SLE also showed significant improvement in disease activity that persisted over 12 months of follow up, despite the lack of change in serum titres of lupus antibody (anti-dsDNA).13 However, this effect on disease activity was only seen in 12 patients whose B cells were effectively depleted from the peripheral blood by the antibody treatment, whereas very little improvement was seen in those who maintained higher levels of B cells. The largest of several small case series involving patients with Wegener’s granulomatosis included 11 patients whose disease was refractory to maximally tolerated cyclophosphamide and glucocoricoids.18 Three of these 11 patients were reported to have ocular involvement. Treatment consisted of an infusion of rituximab (375 mg/m2) every week for 4 weeks, in combination with high-dose oral prednisone that was later weaned. All 11 patients had a depletion of their circulating B cells, decreased anti-neutrophil cytoplasmic antibody and inflammatory markers and disease remission within 6 months. However, six patients required re-treatment with rituximab over the course of follow up, which ranged from 10 to 44 months, either for disease relapse or as pre-emptive treatment when elevation of the anti-neutrophil cytoplasmic antibody titre was seen in combination with a recovered B cell count. This is consistent with results seen in another small series of three patients where almost complete initial disease remissions were followed by disease flares in all patients.19 Interestingly, two of the three patients in this series were described to have granulomatous retro-orbital or sinus masses that seemed not to respond to treatment with rituximab.

The success of rituximab in these diseases, hitherto considered to be T cell-mediated, indicates that B cells must have a much broader role in the pathogenesis of autoimmune diseases than generally appreciated. Certainly, B cells have a critical role in T cell activation in addition to their role as a precursor to cells responsible for specific antibody secretion.20 In addition, the therapeutic effect of rituximab on the disease activity of SLE in the absence of a reduction in autoantibody titres implies that the production of autoantibody is not the sole activity of B cells in the pathogenesis of this disease.

As RA, SLE and Wegener’s granulomatosis are conditions that are commonly associated with scleritis and orbital inflammatory disease, rituximab may emerge as an effective treatment for cases of refractory scleritis, as well as non-infectious orbital inflammation. Indeed, a case study of refractory anterior scleritis in the setting of primary Sjögren’s syndrome has recently been published that has shown complete resolution of the disease with one 4-week course of weekly rituximab infusions (375 mg/m2) followed by maintenance mycophenolate mofetil.21 A second case report describes success managing scleritis associated with Wegener’s granulomatosis with a single rituximab infusion (1000 mg) and maintenance cyclophosphamide, inducing remission that was continued through the 7 months of follow up.22 Although these reports are promising, further studies are required to assess the efficacy of rituximab in ocular inflammatory disease.
With respect to safety, rituximab appears to be very well-tolerated; hypersensitivity reactions may occur, but major infections during the period of B cell depletion are uncommon.
Bunnie

[bunnie]

Hi again, I feel sure you will have seen these but I thought it might be of interest to anyone reading who has these bullous conditions. It sounds so promising, if it can bring on remission. Wish it had been available when I was losing my sight!
BACKGROUND: Pemphigus vulgaris (PV) is a severe autoimmune blistering disease involving the skin and mucous membranes. The response to therapy varies greatly amongst patients and treatment may be challenging. Rituximab is a chimeric monoclonal antibody that selectively targets cell surface antigen CD20, thus depleting mature B cells in vivo. METHODS: We report the results of rituximab treatment in two patients with severe PV. In both patients, high-dose oral prednisolone and adjuvant therapy with intravenous immunoglobulins and mycophenolate mofetil failed to control disease activity. Consequently, the patients were treated with two courses of four weekly intravenous infusions of rituximab (375 mg/m(2)) with a 6-month interval. RESULTS: Clinical improvement was already noticeable 3-6 weeks after the first infusion. After the second course, complete remission was achieved. Oral prednisolone was reduced and treatment with mycophenolate mofetil was continued. The patients remained in full remission 6 months after the last rituximab infusion. CONCLUSION: These cases suggest that two courses rather than a single course of rituximab may be a preferable mode of treatment. Rituximab should be considered as a promising treatment option for recalcitrant PV.
Bunnie

[LIGA girl]

Hi Bunnie

yes I have started treatment and I will continue to post about it in my journal so that anyone who wants to follow the saga can see it all there. I have had a few problems with accessing skincell and posting and also since getting the first infusion I have been very tired and feeling quite unwell but am a little better every day. I go in for the next one on Thursday.

It is great to see that there is literature supporting the use of rituximab for linear iga and similar diseases. I know if this treatment does not work I will be very disappointed. Already I have a reduced number of lesions but of course that does always change ....The immunologist I see said that there are so few of us Linear iga patients and that most will respond well to the drugs I have already taken so there aren't many who are left to try rituximab. I felt decidedly like a guinea pig as I have said before, but I am so thrilled to be able to have this treatment which may cure me even for a time as I still have lots of lesions on the drugs I am on and those drugs are no good in the long run either. My immunologist's registrar said that if it works they will write me up   .

LG

[CalamityJane]

Hi--just barging in here a mo with a huge 

Jane

[nicolespeanut]

Hi ladies!
This news is very exciting!  I have my fingers crossed that it does the job!
Fondly,
Nicole