Pemphigus.
These first photos are when the outbreak is in the acute phase. There have been at least eight cycles over the last ten weeks. This is the blistering phase.
You can enlarge any of the photos. 24 hours before the blisters appear the area becomes red and swollen.
After the blisters appear, they burst within hours, leaving old skin and new raw skin underneath. This is the most painful stage.
Following this stage there are sometimes weeping sores and red, bubbly areas, that join together and then form a crust. It is still quite sore at this stage.
The final stage is as the skin dries out. It takes on a brown, leathery appearance. It peels away to leave new, healed skin.
There is discolouration of this skin and sometimes the blisters start before all of the old skin has peeled away.
Today, 11/01/08 we are at the brown peeling stage. It is the second day of treatment on Prednisone. If this medicine works we hope to see, no new erruptions and the traumatised area beginning to heal.
Here are the photos from day 1 - 2.
Here are the photos from day 2 -3.
This first one is taken in the morning, 12/01/08 after all the brown, dead skin has come away. It looks quite good.
These ones were taken this evening. There are some red areas that look like they may errupt again. Fingers crossed that they will keep clearing.
13/01/08 day 3- 4
I think we might be getting somewhere…
14/01/08, day 4 - 5 of treatment.
15/01/08
Ivy has had a really bad day. Her bottom has become red and inflamed. No pictures today because she was in too much pain.
16/01/08
Still a little red and Ivy is complaining that her bottom is sore. She won’t sit on it and is in pain when she walks but there are no new erruptions!!!!
17/01/08
I think I am going to officially start celebrating. We seem to have skipped the blistering stage and moved straight to the leathery peeling stage.
18/01/08
Ivy had a fantastic day but during the afternoon started complaining of having a sore bottom again. No erruptions though and no new red areas.
19/01/08
A very bad day. David changed ivy’s nappy and it looked a little red. Within an hour her bottom looked like this:
and so we start again.
23/01/08
Taken at night. Reducing Pred tomorrow.
25/01/08
Ivy’s bottom is blistered again. There are some new areas affected too, the groin creases and the labia and one blister on her lip. The blisters appear different, in that they are smaller and more fragile than before the prednisone. They burst very easily and become red and painful areas just like before commencing Pred.
The paediatrician has asked that we stay with the decreased dose though.
Things have gone from bad to worse and so on Thursday 31st January, instead of decreasing the prednisone dose we will increase it back up to full.
4/2/08
New erruption of blisters exactly five days after the last outbreak. No longer only on the buttocks. Now on the legs and insode Ivy’s mouth.
March: Official immunoflourescence direct and indirect diagnosis of Pemphigus, immune deficiency and Ectodermal Dysplasia.
April: Commenced Dapsone.
Dapsone failed: Methaemoglobinaemia, new outbreak of blisters
May: Commenced Mycophenolate.
June: Mycophenolate titrated up to full dose, prednisone reduction begins. Two small outbreaks associated with illness
July: Prednisone reduction continues.
17/07/08 - 2.2mls on alternate days 3mls with Cellcept (mycophenolate) 2mls twice daily. Started to become erythmatous tonight, buttocks are red and swollen.
Entries
The poor thing. I think it’s good to post these photos - like I always say, knowledge is power.
Hugs from miles away…xoxo
My heart goes out to your both Tiff … it must be so painful and uncomfortable for Ivy - can she still wear cloth nappies or nothing ?
Oh my goodness, poor little girl. I’m sure she has no idea what hit her. I feel for her and for you. I think its good to document this, it will be something to look back on one day as a distant memory. I’m so sorry. Hugs.
Oh poor kid- I really didn’t get it till now. And yes, knowledge is power.
Oh my goodness, the poor little girl! That looks very painful and must be very difficult for you to care for, not to mention heartbreaking. Everything crossed that the new medication works.
You could have explained to us 1000 times just how bad it was, but I don’t think any of us would have realised what it was truly like.
I am crossing everything SO HARD that the new treatment plan works.
Poor poor Ivy girl.
Oh, poor baby! That is just heart-wrenching Tiff! Praying that the prednisone works - for both your sakes.
Oh Sweetheart when you said Blisters I never imagined these Blisters..Oh Sweetheart!! and the doctors had dismissed this as thrush??? WTF…
Words just aren’t enough sometimes..
(((HUGS)))
xxx Kim xxx
When you first named the disease I googled it and saw pictures there and almost cried for the pain Ivy must be going through. Now I see this on her and blinked away tears again. I’m horrified that you’ve been trying to find out what this was for sooo, so long. I’m glad treatment has now started and I hope it works spectacularly.
Oh Tiff, my heart goes out to you all - especially you and Ivy. I pray the prednisone does what they hope it will. Hugs, tears and more hugs xox
It is looking better. Heaps better. Crossing fingers that the red bits don’t erupt again. Is she seeming happier?
That is heart breaking Tiff.
I hope & pray that this treatment works.
Is it only her bottom that these blisters break out on? Have the Dr’s (or any of your own reasearch) explained why it only (if it is) happens there?
Prayers and more prayers. My heart breaks for your little girl, I can not imagine all of your frustration and anger. Be well little Ivy, be well. Hang in there!
Her poor little bottom is looking a bit better… Fingers crossed that she heals up…(((Hugs)))
and Kisses too
Kim xxx
Wow - That is looking quite a bit better! Praying that it keeps working!
WOOT! No blisters! *dances* I truly hope that the prednisone eventually completely stops the cycle.
wow what an improvement - haven’t heard from you about tomorrow ?
Fingers Crossed!!!
poor little luv. I hope something starts to work soon so she can have some relief
Oh poor little pet. I was being so hopeful too!
Good heavens. I never imagined this when you spoke of it. How can they possibly think that is a simple yeast rash?!? My heart breaks for little Ivy. And you.
I find it hard to believe the docs were fobbing you off and not very seriously looking for a cause for this. Before you got your diagnosis what on earth were they attributing it to? My son had allergy problem rashes, thrush etc but nothing like this.
WHY? WHY? WHY?! I cannot imagine your frustration. Hang in there. Poor little thing…
Poor Ivy! I wish there was something better they could do.
Reduce the pred and the blisters come back increase it and you have roid rage. A rock and a hard place honey.
You have my permission to break something if you like. It must be so frustrating!
I never imagined how bad it was. My thoughts and hopes are with you that this treatment works.
xx
Ugh- poor baby! I’m so sorry!!
Can I ask what this condition is called?
oh Tiff- poor Ivy - I am lost for words. Hope the increase in Pred. starts to work.
Without having seen it for real you can’t imagine what poor Ivy is going through.It is a lot for a young toddler to put up with let alone her mum.
Hope the new meds kick in too .Thinking of you.
Ouch ouch ouch!
Poor Ivy girl. This sucks for her and for you. (((hugs)))
I’m so sorry tiff
oh no, poor little thing. I can only imagine how awful this must be for you all
OMGOSH Tiff your poor little girl. Im so so sorry she has gone through that. Of cause for you to have to watch, a mother couldnt have to see her children suffer like that.
You have been through so much since ive met you and you are the strongest lady I know. I really hope things look up for you and little Ivy.
Take care hun
Oh and happy birthday to little william. xx
I’ve never seen Pemphigus before. I would have called it Bullous Impetigo but my guess is that this kept coming back!
Very interesting from a pediatrician perspective- I won’t forget this diagnosis- thank you and very sad for your poor daughter. The pain she must go through. It must be hard as her mommy to watch her go through this.
Oh my Gosh!!
My mom got hit with this in Dec-2007. At first she thought it was just a rash. The itching was so bad that she used hydrocortisone cream on the affected areas. That didn’t work. Then she tried poison oak treatment but that didn’t work either.
Her doctor misdiagnosed her with scabies. The scabies medication didn’t work either.
The little bumps got worse and became blisters… and huge ones too! My mom couldn’t sit on the toilet seat because it was so painful. The blisters were from the neck down.
We went to the ER and finally it was properly diagnosed as Bullous Pemphigoid. My mom was given a prescription of 100 mg prednisone. Unfortunately, that was on a Friday and my mom’s condition got worse over the weekend. Blisters were popping up and some were breaking.
We went to the dermatologist and they said that they could no longer handle her condition in the office environment. They sent her to U.C. Davis Medical Center in Sacramento, CA. The dermatologist said that she had to get my mom there because they have a burn unit.
The doctor was concerned that when the blisters popped or broke, my mom would rapidly dehydrate like a burn victim dehydrates.
They put her on IV’s and also, they removed 2 out of 3 high blood pressure medications that she was taking!!! The doctors suspected that Norvasc and Benazapryl probably were triggering my mom’s problems. They said that long term use of high blood pressure medications can trigger Bullous Pemphigoid in elderly people.
They continued to give my mom a minimum of 60 mg prednisone along with an immunosuppressant drug Azathioprine.
The nurses would cut my mom’s blisters and put special dressings on her. The blisters finally stopped after a few days. I wish I would’ve kept good notes like you did for your daughter’s case.
My mom was released to a professional nursing facility for 21 days. There was no way that my brother and I could take care of her because of the locations of some of the wounds in the crotch area and underarm pits. My mom was still in a lot of pain.
We got her home and had a follow up visit with my mom’s primary care physician. The nurse noticed that my mom was jaundiced!!! It turns out that her body was rejecting the Azathioprine! It was poisoning her liver!!!
So she was admitted through ER and hospitalized again. She had lots of blood work done and a liver biopsy. It was confirmed. Her liver was being poisoned by the Azathioprine.
So we stopped the Azathioprine. She no longer had blisters… Until a few days later and they started coming back!
The dermatologist increased her prednisone again and the blisters started going away. Later the dermatologist said that we cannot keep my mom on prednisone since long term use is NOT good.
After she was released from the hospital, we started to taper her prednisone dose from 60 mg down to about 25. The blisters started to come back.
The dermatologist said that my mom would have to be put on another oral immunosuppressant drug or go into immunoglobin (IVIG) therapy. She chose the new oral drug, Cellcept. It is EXPENSIVE!! The doctor said that it would take about 6 to 8 weeks before the Cellcept would kick in.
In the meanwhile, my mom had to take 40 mg of prednisone daily along with Tetracycline and Nicomide.
My mom is still on prednisone and we have tapered her from 40 mg daily to 20 mg and are continuing to taper. So far, no new blisters. But her skin develops a brown discoloration every now and then, but no itching and no blisters.
The pharmacist suspects that my mom might have to take Cellcept for the rest of her life. As for IVIG, it sounded great in theory, but the idea of having to get frequent IV’s for long periods of time didn’t appeal to her would be too inconvenient.
I pray that Ivy will get through this very difficult time in her life. Take care.