Lupus and the Skin
Dr Simon J Meggitt, Consultant Dermatologist
Royal Victoria Infirmary, Newcastle upon Tyne
Skin problems occur frequently in people with lupus. However, few of these people ever see a dermatologist. Care is usually managed by a physician with an interest in lupus, often a rheumatologist. Lupus is a disease that can affect several different parts of the body, and so other specialists frequently need to be involved. Nevertheless, it is important that overall control of lupus stays with one doctor, which then also helps to reduce what can be numerous hospital visits for patients with lupus. The flip side of the coin is that skin (and other) problems frequently end up being dealt with by non-specialists.
The way round this is to have joint clinics where several doctors from different specialties see patients together. This can be helpful in managing lupus, as several heads can be better than one, especially as lupus often has many complicated facets. Some UK centres such as ours in Newcastle offer this service for people with systemic lupus, as well as dedicated clinics for patients with lupus that is confined to the skin.
Lupus in the skin can show itself in very many forms, which can be confusing enough for dermatologists to diagnose and understand, let alone other doctors. This can make explaining skin lupus to patients quite a challenge! At the moment there is general patient information for skin lupus available from LUPUS UK. However, because there are so many different types of skin problem, it's difficult to pick out from this information what is really important to any one individual.
So what would be the best care that could be offered to somebody with skin lupus? Before treatment is even considered there are several important steps that need to be taken. First, making the correct diagnosis is paramount, (i) because many other skin diseases can resemble lupus, and (ii) because the several types of skin lupus may need different treatments. It is also important for patients to realise that most people with skin lupus never go on to develop lupus in other parts of the body. Second, proper explanation of the problem and the likely outlook for a patient is crucial.
Third, those few patients who may be more likely to develop internal problems need to be identified and made aware of the risk. This may require careful monitoring over several years.
Probably the most important thing for people with skin lupus is to have their skin problem taken seriously. The effect of skin disease is often enormous and this is all too often overlooked. The wider issue here is that skin disease is often regarded as unimportant not only by the general public, but also many doctors. For example, eczema, a disease that can cause far-reaching suffering to individuals and their families, is often dismissed as 'just a bit of dry skin'. The failure to recognise the importance of skin disease extends to the political establishment: for example a recent government proposal for treatment of chronic illnesses did not even include skin disease.
In order to take skin problems seriously it is important to recognise the far-reaching impact that a skin problem can have, particularly if abnormal skin is visible to others. Light sensitivity in lupus means that this is usually the case. Self-confidence, relationships and social life are often affected, and anxiety and depression are common. People with skin problems are frequently stigmatised because of their appearance, both socially and in the workplace. For all these reasons it is vital that health care workers strive to offer the best possible care as early as possible. This is especially important for those types of skin lupus that can cause permanent damage: once scars are present no medical treatment can reverse them.
Skin lupus can be split into three major categories: discoid lupus, sub-acute lupus and rashes that occur with systemic lupus. Discoid lupus is the commonest type. Patients develop well-defined red scaly patches mainly on areas exposed to sunlight, usually the head and neck. The major problem with discoid lupus is that even after it has been successfully treated, dark or pale marks, unsightly scars and areas of permanent hair loss can be left behind. Fortunately, the vast majority of people with discoid lupus rarely develop systemic lupus. However, many people who have systemic lupus will develop discoid lupus at some point during their disease.
Sub-acute lupus is an uncommon type of skin lupus that is often triggered by sunlight. The rash usually consists of red or scaly rings, which can cover a great deal of the body. People often feel unwell and may have other symptoms, but these do not amount to having systemic lupus. People with systemic lupus can get any of the rashes described so far, and often develop the red flush across the cheeks and nose known as the 'butterfly rash'.
Treatment of skin lupus can be divided into important basic measures, topical treatment (creams or ointments) and tablet therapy. The most important basic measure is sunlight avoidance. Many people with skin lupus do not realise that they are sun-sensitive. This is often because there is often some delay between sun exposure and the start of the rash. Many people also do not realise that it is not only direct sunlight, but also sometimes light passing through window glass that can trigger or worsen the rash. The most sensible measure to avoid sunlight is to cover up wear long sleeved tops, long trousers or skirts, and ideally a broad-brimmed hat. Regular sunscreen use is also very important. Many people often miss areas such as the ears and temples. The sunscreen needs to be of high factor (more than factor 25) and also have 4 or 5 stars of protection stated on the bottle. People who have scarring from previous lupus or prominent rash on visible areas may be greatly helped by using cosmetic camouflage. The Red Cross provide a useful cosmetic camouflage service.
The main topical treatments for skin lupus (in particular discoid lupus) are steroid creams and ointments. These work well in many patients. A common mistake is to use too weak a steroid. Strong topical steroids are usually required. However, particularly on the face there is a possibility of skin thinning developing after several months of treatment. This risk needs to be weighed up carefully by the patient and doctor. However, most people would consider that the scarring that can result from untreated lupus is far more disfiguring than even the worst skin thinning.
If topical steroids are not helpful, or if skin thinning starts to develop, tablet treatment should be considered. Similar drugs are used to treat all three varieties of skin lupus. People who present with very bad skin disease often go straight onto these tablets without first using topical treatments. In general, most tablets that are used to treat skin lupus are very safe, although of course rare side effects can occur with any tablet. For unknown reasons, tablets that were first made to treat malaria are also very effective in treating skin lupus.
Hydroxychloroquine is the commonest tablet used. Side-effects are uncommon and if they do occur are usually mild and not serious. Mepacrine is another commonly used tablet that can work very well, but it has the disadvantage of turning the skin a yellowish colour. If antimalarials fail, there are other stronger tablets that can be used (eg dapsone or thalidomide). However, with these treatments, side effects can occasionally be more serious and careful monitoring is needed for the few people who need to take them. With systemic lupus, it is important for both patients and their doctor to realise that even if strong drugs are being used to treat internal illness, these may not be the best treatments for skin disease. If skin lupus remains a problem, then a dermatologist's advice should be sought.
Unfortunately for those few people with difficult to treat skin lupus, little evidence is available as to which are the best treatments, and this area urgently needs research. However, there are other priorities for the short-term. New simplified patient information will be developed for all the different types of skin lupus. Most importantly, in Newcastle we are developing a way of measuring how bad skin lupus actually is. Only with such a measure can the strength of the different treatments be properly compared in the future.
Our thanks to Dr Meggitt for reproducing this interesting talk given to the delegates at the recent National Conference.
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