Treatment
This page covers the following:
Introduction
There are many types of treatment that are used to treat pemphigus and pemphigoid. These are usually “repurposed” from other illnesses and have been used regularly for these. Your treatment will be informed by the type and severity of your disease as well as your other medical problems and medications.
Currently, there is no known cure for pemphigus or pemphigoid, so all the treatments on offer are intended to manage the symptoms and minimise the negative impact that the diseases can have. The aim is to get your disease under such control such that it can be classed as “in remission”.
Initial Drug Treatment
We are fortunate that we can benefit from the advances in medical knowledge related to different auto-immune and other diseases and work continues to identify the most successful drugs to treat blistering diseases.
For mild cases, topical steroids are usually the first port of call. Depending on the location of the blistering, this may be in the form of creams, ointments or solutions.
For severe cases, the most effective and rapid way to bring the blistering under control is usually to take corticosteroids (note these are different to anabolic steroids) by mouth in the form of prednisolone. There can be significant side effects from taking high doses of corticosteroids, particularly long-term. These include potential emotional changes, stomach irritation, high blood pressure, high sugar levels and thinning of the bones. For more information, see the steroids page. To avoid long-term use of steroids, particularly at high doses, other drugs are often introduced as “steroid-sparing agents” which enable the dose of corticosteroids to be reduced slowly and potentially stopped altogether.
A recent trial conducted by the UK Dermatology Clinical Trials Network compared the use of prednisolone (a steroid) and doxycycline (a tetracycline antibiotic) to treat bullous pemphigoid and found that doxycycline was effective but significantly safer than prednisolone. Doxycycline or similar related antibiotics are often used as a long-term treatment for pemphigoid.
Dressings
Blistering of the skin may also need managing with aspiration of the blister fluid and dressings or dressing alone. There is further information on this in a video produced by the French APPF as well as suggestions by other patients on the dressings page.
Maintenance Treatment
To minimise the risks of long-term steroid use, your medical specialist will usually aim to start other medication(s) to use longer term with the aim of slowly reducing the dose of steroid tablet and potentially stopping this altogether. These are sometimes known as “steroid-sparing drugs” or adjuvant drugs. While these drugs have different mechanisms, they all modify your immune system responses with the aim of reducing unwanted inflammation resulting in your blistering condition. Different people respond differently to these drugs so it may be necessary to try several drugs to establish which one works most effectively for you.
The adjuvant drugs most commonly used to treat pemphigoid and pemphigus are:
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Azathioprine
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Mycophenolate mofetil
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Dapsone
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Tetracycline antibiotics (e.g. doxycycline, lymecycline)
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Nicotinamide
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Methotrexate
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Cyclophosphamide
For more details, see the page on other medications.
For very severe cases of pemphigus and less frequently pemphigoid, a biologic treatment called rituximab can be used. This targets certain immune cells which drive these conditions. Rituximab can be used as a third-line drug to treat pemphigus when other medications such as high doses of steroids, azathioprine and mycophenolate have failed to control the disease. Intravenous immunoglobulin (IVIG) is rarely used in the UK to treat pemphigoid and pemphigus.
More information
We refer you to the British Association of Dermatologist’s Patient Information Leaflets.
Further information on Cyclophosphamide and IVIG can be obtained on the Arthritis website. Treatment for Rheumatoid Arthritis often has some similarities with pemphigus and pemphigoid, as both are the result of a faulty immune system. The information on rituximab is also useful.
Information can also be obtained from the International Pemphigus and Pemphigoid Foundation.
ALL of these medications can have serious potential side effects, so nearly all require regular blood test monitoring and some require other monitoring tests e.g. urine tests and blood pressure readings.