By: Clare Wieduwilt
Pemphigus is an autoimmune disorder. It occurs when the immune system mistakenly attacks proteins in the upper layers of the skin. In this disease, the immune system produces antibodies that attack specific proteins that connect the epidermal cells (the cells in the top layer of skin) to each other. When these connections are disrupted, the cells separate from each other and from the lower layers of the skin, and blisters form.
The exact cause for such actions are unknown and, although rare, can be due to certain medications including penicillamine and ACE inhibitors.
The disease is found most often in middle age men and women (rarely in children) but is also evident in animals.
The major symptom of pemphigus vulgaris is the development of clear, soft, and painful (sometimes tender) blisters of various sizes, and once ruptured, the area can become infected. Blisters can be describe as:
- Peeling or easily detached
- Not itchy
- No scarring
In about 50% of people with this condition first develop blisters in the mouth (ulcers).
Other locations include scalp, trunk and other areas of the skin.
Person generally feels very ill.
The skin separates easily when the surface of unaffected skin is rubbed sideways with a cotton swab or finger. This is called a positive Nikolsky's sign.
A skin biopsy is then done to confirm the diagnosis.Doctors differentiate pemphigus vulgaris from bullous pemphigoid by noting the layers of skin involved and the particular appearance of antibody deposits.
- Antibiotics and antifungal medications to control or prevent infections
- Fluids and electrolytes given through a vein (IV)
- IV feedings if there are severe mouth ulcers
- Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain
- Pain medications if local pain relief is not enough
Body-wide (systemic) therapy is needed to control pemphigus and should be started as early as possible.
- An anti-inflammatory drug called dapsone
- Corticosteroid (First used in 1944)
- Medications that suppress the immune system (such as azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate mofetil, or rituximab)
Plasmapheresis is a process in which antibody-containing plasma is removed from the blood and replaced with intravenous fluids or donated plasma.
Ulcer and blister treatments include soothing or drying lotions, wet dressings, or similar measures.
If left untreated
Before treatment with steroids became available, up to 8 in 10 people with pemphigus vulgaris (PV) died within a year or so of the disease starting. The cause of death was often serious infection combined with lack of fluid in the body (dehydration). Dehydration can easily occur if you have widespread raw areas of skin. Having untreated PV is a bit like having widespread skin burns, with the consequent risk of infection and dehydration.
With treatment, blisters usually heal and stop forming, and the risk of death is greatly reduced. Many patients with PV are able to have long periods of inactive disease (remission). However, treatment does not always work well and side-effects from treatment can sometimes be serious. About 1 in 10 people with PV die of their condition or as a consequence of severe side-effects from treatment. Older people are more likely to have side-effects or die as a result of PV.
Living with Pemphigus Vulgaris
- once the disease is controlled and your medication is at 'maintenance' level, you should not get noticeably more infections than anyone else
- hard to eat crispy and or acidic food (tomatoes or oranges) as it can become painful
For Further Help and Information
14 Lime Court
Tel: 0208 863 3735
The Pemphigus Vulgaris Network offers general support and provides a forum for people living with pemphigus vulgaris and those personally or professionally concerned with it.
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