Pemphigus Foliaceous
This page covers the following:
Introduction
Pemphigus foliaceous (PF) is a very rare autoimmune condition resulting in painful and itchy sores to form on your skin. The most common places for these to appear are on your scalp, face, neck and back. It affects people of all races, age and sex and in otherwise good health. It appears most commonly between the ages of 50 - 60 years, but it can affect people of all ages, even children. Pemphigus also occurs, more often, in people originating from North Africa, Turkey, and South America. 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?
If you have PF, you probably get blisters when the skin is rubbed (a phenomenon known as the Nikolsky sign). To confirm a diagnosis of PF, the clinician will take a small sample of your skin’s tissue and examine it under a microscope. They will also take blood samples. The skin biopsy is subjected to a direct immunofluorescence test which reveals the layers of skin that are affected by the antibodies. A blood test usually shows the circulating autoantibodies.
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?
It isn’t entirely clear why people get these rare autoimmune diseases. It is not inherited and is not catching. You may have a genetic disposition. There is some indication that you could be at an increased risk of developing pemphigus if you have a specific human leukocyte antigen (HLA) gene. HLA genes are responsible for identifying foreign invader proteins, such as bacteria, to help your immune system destroy them, but the presence of any specific gene is not enough to cause your pemphigus. There are a number of environmental factors that may trigger your PF to occur. There is some evidence that drugs such as Penicillamine, nifedipine, captopril, enalapril or nonsteroidal anti-inflammatory drugs can provoke PF. This is known as drug induced pemphigus foliaceus. If the drug is stopped, there is a 50% chance the PF will clear up. PF is sometimes provoked by sun exposure. In some parts of the world (specifically South America), there is a form of PF called fogo selvagem, believed to be the result of an insect bite, but we know of no cases of this in the UK. However, sometimes the PF appears with no obvious explanation.
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.
How is pemphigus foliaceous treated?
Although PF is often less severe than pemphigus vulgaris, the doses and types of medications required to control them are similar.
Topical steroids are often used. In the case of mild cases, these may suffice to control and ease the blistering but, otherwise, can be used in conjunction with drugs taken by mouth or by injection.
An antibiotic called doxycycline is also sometimes used to control inflammation.
For more severe PF cases, steroids (usually prednisolone) are used initially to bring the disease under control and reduce the pain and inflammation. These usually start at a higher dose, gradually reducing as the condition comes under control. Read about the side effects of steroids.
The steroids are replaced, gradually, by other drugs called steroid sparing agents. These often have side-effects, but not as serious as those created by steroids. Many work by depressing your immune system (immunosuppressants). These can also leave you susceptible to infections.
Typical drugs used to maintain control of your symptoms are azathioprine, mycophenolate mofetil or methotrexate. It sometimes takes a little while to find the right drug that works for you, with the minimum side effects.
For PF cases which fail to respond to these treatments, an intravenous drug called rituximab can be used. Read more about rituximab and its approval guidelines.
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.