Pemphigus and Bullous pemphigoid are autoimmune diseases that cause blistering of the skin.
Bullous pemphigoid tends to occur mainly in older people. It is a less serious disease than pemphigus, is rarely fatal, and does not result in widespread peeling of skin. It can involve a large portion of the skin, however, and can be very uncomfortable, with itchiness often the first sign of the disease.
In bullous pemphigoid, the immune system forms antibodies directed against the skin, resulting in large, tense, very itchy blisters surrounded by areas of red, inflamed skin. Blisters in the mouth are uncommon and are not severe. The areas of skin that are not blistered appear normal.
Diagnosis and Treatment
Doctors usually recognize bullous pemphigoid by its characteristic blisters. However, it is not always easy to distinguish it from pemphigus vulgaris and other blistering conditions, such as severe poison ivy. Bullous pemphigoid is diagnosed with certainty by examining a sample of skin under a microscope (skin biopsy). Doctors differentiate bullous pemphigoid from pemphigus vulgaris by noting the layers of skin involved and the particular appearance of antibody deposits.
Mild bullous pemphigoid sometimes resolves without treatment, but resolution usually takes months or years. Therefore, most people receive drug therapy. Nearly everyone responds quickly to high-dose corticosteroids, which are tapered after several weeks. The combination of nicotinamide and
minocycline or
tetracycline is sometimes successful. Sometimes
azathioprine or
cyclophosphamide is given as well for more severe disease. Immunoglobulin given intravenously is a safe, promising new treatment, especially for people who do not respond to conventional drug therapy. Although some local skin care may be needed, most people do not require hospitalization or intensive skin care treatment.
Auto-immune diseases are caused when the immune system starts attacking the patient’s own body. The immune system is very complex, and the underlying mechanism of auto-immunity is not well understood.
Auto-immune blistering diseases are a group of auto-immune diseases which affect the skin, although auto-immune diseases occur in many other systems of the body. For example, inflammatory bowel disease (ulcerative colitis and crohn’s disease) are auto-immune diseases of the intestine, and multiple sclerosis is an auto-immune disease which affects the nervous system.
In auto-immune blistering diseases, the body makes antibodies against the adhesion proteins in the skin. Different proteins are affected in different types of diseases.
Pemphigus
Pemphigus is rare, with about 1 new case / 1 000 000 people / year. There are 3 types: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus.
Source
Ivy has been diagnosed with Pemphigus foliaceus.
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.
Further serum testing has come back equivocal, based on testing done, while on prednisone.
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.
September 2008 - IVIG (Intragam P) commenced.
May 2009 - Condition stable on Mycophenolate , Prednisone 10mg/15mg (2mls/3mls) alternating dose and IVIG. Outbreaks only occur when Ivy is very unwell or if I try to reduce the prednisone too fast. Weaning of the prednisone continues slowly.
