PEMPHIGUS FOLIACEOUS
This page covers the following:
- Introduction
- How is pemphigus foliaceous diagnosed
- What are the symptoms of pemphigus foliaceous?
- What caused my pemphigus foliaceous?
- How long will my pemphigus foliaceous last?
- How is pemphigus foliaceous treated?
- Where can I find out more information about pemphigus foliaceous?
Introduction
Pemphigus foliaceus is a rare autoimmune condition that causes painful and itchy blisters and sores to form on your skin. The most common places for blisters and sores to form on your body are on your scalp, face, neck and back.
The main observable difference between PF and pemphigus vulgaris (PV), which is the more frequently diagnosed form of pemphigus, is the potential involvement of mucous membranes (mouth, nose, eyes, genitals, etc) frequently seen in PV. In PF, the skin is the only site to be affected. The blisters are the result of the "glue" in the upper parts of your layers of skin, being "attacked" by circulating antibodies (autoantibodies). This "glue" is a protein called a desmosone. In the case of PF, the particular desmosone affected is called a desmoglein-1. In PV, another desmosone is also affected (desmoglein-3). When the layers of the skin separate as a result, a blister forms and fills with fluid. In PF, the layer of the epidermis that is affected is close to the surface, so these blisters tend to be flaccid and burst easily, leaving erosions or crusts.
How is pemphigus foliaceous diagnosed
Skin biopsies are usually required to confirm the diagnosis. Two biopsies will be needed – one biopsy involving the edge of a blister for routine histology in formalin and a second perilesional (normal skin) biopsy which looks for evidence of the abnormal proteins in the skin.
Blood tests may allow identification of the abnormal proteins in the blood.
What are the symptoms of pemphigus foliaceous?
PF causes painful blisters to form on your skin. The blisters, or crusts, are fragile and break open easily, creating sore erosions. After the blisters burst, they leave a scaly, crusty area. These can be very itchy and painful. PF often appears as a painful rash on the face and upper body but can affect all of the skin. Over weeks to months, the blistering can progress, and more and more new lesions appear over the body, limbs, face, and scalp. Severe cases of PF can result in lesions all over the entire body, developing into something called an 'exfoliative erythroderma'. PF does not affect the mucous membranes (mouth, nose, eyes, genitals, etc). There are currently 6 known variations of pemphigus foliaceus:
- Pemphigus erythematosus
- Pemphigus herpetiformis
- Endemic pemphigus foliaceus
- IgA pemphigus foliaceus
- Paraneoplastic pemphigus foliaceus
- Drug-induced pemphigus foliaceus
What caused my pemphigus foliaceous?
Pemphigus foliaceus occurs when cells of the body's immune system produce proteins (autoantibodies) that damage the adhesion points between skin cells. These adhesion points act like press studs holding one of the top layers of the skin cells (epidermis) together. If they are damaged, the skin cells break apart and the top layer peels off. As the top layer is very thin, the blisters may not be noticed, and the skin may appear to be peeling only.
The cause of the immune attack on the skin is not known. Genetic and environmental factors are thought to play a role: some medications containing sulfur groups are thought to be involved; in countries where the condition is endemic in young people, certain parasitic infections and insects are thought to be involved.
Triggers which may initiate pemphigus foliaceus include sunburn, drugs containing thiol, vaccines and radiotherapy.
What other problems can occur with pemphigus foliaceus?
People with pemphigus foliaceus, together with members of their family, have an increased risk of other autoimmune diseases. The most common of these is autoimmune thyroid disease.
How long will my pemphigus foliaceous last?
These diseases are chronic and long term. There is no cure but remission is possible. The disease can be managed and "normal" life resumed.
Treatment of pemphigus foliaceous
Treatment for pemphigus foliaceous usually starts with medicines to ease symptoms and prevent new blisters. These may include steroids and medicines that target the immune system. If your symptoms were caused by use of certain medicines, stopping that medicine may be enough to clear up your symptoms.
Some people may need a hospital stay to receive fluids, nutrition or other treatments.
Medicines
Your healthcare professional may suggest one or more of the following medicines. The choice of medicines depends on the type of pemphigus you have, how severe your symptoms are and whether you have other medical conditions.
Corticosteroids. For people with mild disease, corticosteroid cream or injections may be enough to control it. For others, the main treatment is a corticosteroid medicine taken by mouth, such as prednisone pills.
Using corticosteroids for a long time or in high doses may cause severe side effects. These include diabetes, bone loss, an increased risk of infection, stomach ulcers and a shift of body fat. This shift in fat can lead to a round face, also called moon face. To avoid these side effects, steroids may be used only for short periods of time to control flare-ups. And other medicines that target the immune system may be used long term to control the disease.
Medicines that target the immune system. Some medicines can stop your immune system from attacking healthy tissues. Examples are azathioprine (Imuran, Azasan), mycophenolate (Cellcept) and cyclophosphamide. These too can have severe side effects, including an increased risk of infection.
Other medicines. If first line medicines aren't helping you, your healthcare professional may suggest another drug, such as dapsone, intravenous immunoglobulin or rituximab-pvvr (Ruxience). You may need antibiotics to treat infections.
Many people with pemphigus get better, especially if treatment is started early. But it may take years and can require taking medicine for a long time.
Where can I find out more information about pemphigus foliaceous?
The British Association of Dermatologists have a Patient Information Leaflet about PF. DermNetNZ is also a good source of further information about pemphigus foliaceus.