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.

Dressings

Blistering of the skin may also need managing with aspiration of the blister fluid and dressings or dressing alone.

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.

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 faulty immune system. The information on rituximab is also useful.

Information can also be obtained from the International Pemphigus and Pemphigoid Foundation.

STEROIDS

What are steroids?

Corticosteroids are a synthetic form of cortisone – a hormone produced naturally in your adrenal glands.

Corticosteroids (usually called steroids) are commonly prescribed for autoimmune blistering conditions to reduce blistering and inflammation. Little else is as rapid acting and symptomatically beneficial as oral corticosteroids when you are in pain from your blisters, but it is important to be aware that oral corticosteroids can be associated with risks and side effects, particularly at higher doses.

Corticosteroids can be delivered in a topical form (creams, ointments, or lotions to apply to the skin or liquid drops or sprays or foam applied into the eyes, nose, or mouth etc.), or in a "systemic" form, i.e. taken as tablets or intravenously. They can also be delivered via an inhaler or nebuliser e.g. to treat asthma.

There are a range of topical steroid creams and ointments which have different strengths (potencies). Steroid mouthwashes to treat oral blistering diseases can be made up by dissolving a steroid preparation e.g. betamethasone tablet or fluticasone capsule in water. Topical steroids are quite safe to use as a maintenance treatment and a less potentially harmful alternative to systemic drugs.

Why am I being prescribed steroids for my pemphigus or pemphigoid?

Steroids are a very effective way of treating inflammatory disease. In severe cases, they can bring the disease under control very quickly and reduce pain and blistering. Levels prescribed are usually based on milligrams per kilogram body weight.

Autoimmune blistering diseases are also the result of a dysfunctional immune system, and steroids dampen your immune system.

What are the negative effects of taking steroids?

If oral steroids are prescribed, they can be a double-edged sword. Although they can deliver fairly immediate relief when symptoms are at their worst, they can carry very significant side effects, both short- and long-term. Clinicians should try to find alternative drugs called steroid-sparing agents, to reduce the need for the steroids, as soon as is feasible.

Typical side effects

You may experience few side effects, but many are quite common. Your clinician will often prescribe other drugs to counteract these. Amongst many effects, are:

• Lowered immunity
• Disrupted sleep (therefore you are normally advised to take tablets in the morning)
• Bloating or weight gain
• Increased appetite
• Cushing's syndrome (puffy 'moon face', increased fat deposits on chest and abdomen, buffalo hump on back of neck and shoulders, easy skin bruising/stretch marks)
• Mood disturbance including mania and depression
• Loss of calcium from the bones leading to bone thinning and potentially osteoporosis
• Increased blood pressure (hypertension)
• Acid reflux and digestive problems
• Increased blood sugar levels with a risk of developing diabetes
• Eye problems including glaucoma or cataracts

The inappropriate use of topical steroids e.g. too strong (or potent) for the body site treated or used for too long can result in skin thinning and easy bruising of the treated skin. This should not occur if following the advice from your doctor, but you should check with your doctor if you have any concerns.

How should I take my steroids?

It can help to minimise the side effects by taking your tablets in the morning. You should also eat well and exercise. Some other medicines and foods (such as liquorice) should be avoided.

If you use steroid mouthwashes, you are at increased risk of developing oral thrush. For this reason, you may be advised to use a regular mouthwash routinely and you need to be careful to ensure dentures are properly cleaned. If there are signs of oral thrush, you will be prescribed specific treatment for this.

Because steroids work by reducing your immune system activity, systemic steroids are associated with an increased risk of infection. For this reason, it is important to ensure you have a 'flu vaccine each year (but not any "live" vaccines – obtain advice from your nurse or doctor and always make sure they are aware you are taking systemic steroids).

Coming off steroids (tapering/weaning)

If you have been given a large dose of prednisolone and/or have been on steroids for any period of time, it is very important that you reduce the dose slowly rather than stopping them abruptly. Your doctor should give you very clear instructions on how to taper the dose and usually will advise you to do this more slowly the smaller the dose of prednisolone you are taking (this is because you need to consider the relative percentage reduction rather than the absolute reduction in milligrams). Once you get to a very low dose, you may find that alternating days of your normal low dose and no dose can help ease you towards complete withdrawal from the steroids.

As you taper the steroid dose, you may experience a recurrence of your symptoms. Your doctor will advise you not to continue tapering the dose if this happens and may advise you to increase the dose of steroid to the previous dose that controlled your symptoms.

Symptoms of adrenal insufficiency include:

• Low blood pressure (hypotension)
• Vomiting
• Abdominal pain
• Muscle weakness and fatigue
• Low energy
• Low blood potassium
• Mood changes

These symptoms may exist for some time after you have completely stopped taking steroids.

During periods of increased stress e.g. severe infection, trauma, surgery, you will likely need to take an increased dose of oral steroid to avoid developing adrenal insufficiency. It is very important that all healthcare providers know you are taking oral steroids so they can give you appropriate advice in these situations.

After prolonged use of steroids, your adrenal glands may need to be "re-activated". If your specialist believes your adrenal glands are not working properly following your long-term dependence on artificial steroids, they may ask you to take a synacthen test to see how well your adrenal glands are functioning. This test involves injecting you with a hormone called ACTH, which is naturally produced in your pituitary gland to tell your adrenals to produce cortisol. Your body cannot function in the absence of cortisol.

Sometimes, once you are on a small dose of steroids (less than 5mg per day), your healthcare professional may switch the type of steroid you are taking to another type, such as hydrocortisone.

What if I need surgery or require a general anaesthetic?

Your anaesthetist needs to know that you are taking steroids and is likely to increase your dose for the operation.

Recommendation

Where can I find more information?

You might like to read this article about reducing your steroids in the British Journal of Pharmacology.