Le Lupus érythémateux et la peau (Pour les professionnels)

Lupus Erythematosus and the Skin (For Professionals)

Have you heard about Lupus Erythematosus (LE)?

• This is an autoimmune disorder which is caused by antibodies against the nucleus of cells. They are usually found in women aged 20 to 50, but males can also be affected. It is a systemic disorder (affects the whole body) but it can also cause skin changes.
• The changes in the skin associated with lupus are many. LE types which manifest themselves as skin problems are systemic LE, discoid LE, subacute LE, Lupus Tumidus, neonatal LE, Chilblain LE, cutaneous lupus mucinosis, and drug-induced LE.

• We shall deal with each of the LE type associated with skin changes one by one:

1. The classical form is called systemic lupus erythematosus (also called acute lupus erythematosus), which manifests as a malar eruption or ‘butterfly rash’ (red patches across the cheeks). Other skin changes in SLE are photosensitivity (a rash on all sun exposed skin), mouth ulcers, urticaria (hives) and diffuse hair thinning. Rarely, it may cause blisters (bullous LE).
2. Discoid lupus erythematosus (Also called chronic lupus erythematosus). In this type of LE, there are red, scaly patches (rough when touched) which may later on become scars or unsightly marks, which may be localized or widespread. You can see them on the cheeks, the nose, the ears, the upper back, the V of the neck and the back of hands. Sometimes, they may affect the palms and soles and even the hair. One can even become bald (scarring alopecia). It can also manifest itself in the lips and oral area as ulcers and mouth sores, which can even lead to squamous cell cancer.
3. Subacute lupus erythematosus. In this type, the rash is non itchy and reddish located at the upper back and chest and may either be ring shaped, scaly, purplish spots or lumps. It may follow sun exposure (Photosensitivity).
4. Lupus Tumidus. In this type, the rash is photosensitive (sensitive to sunlight) and appears red bumps and patches like a rash. Some may even be ring-like. What is remarkable about these skin changes is that they disappear during the winter without any marks or scars. It is often considered a form of chronic lupus erythematosus.
5. Neonatal lupus erythematosus, is found among babies born to mothers with LE and may appear as a ring like rash which often disappears after a few months. One should be careful because one complication in these babies is heart block.
6. Chilblain lupus erythematosus, which are present in those living in cold areas or are smokers. They suffer from abnormal blanching of fingers and toes in response to cold weather, followed by numbness and slow rewarming by the fingers which go blue then red (Raynaud phenomenon). Mild arthritis of finger joints may also occur. Usually you can see closely that there are dilated blood vessels at the base of the fingernails.
7. Cutaneous lupus mucinosis, which is rare and appears like small bumps, plaques and nodules or larger bumps of the cheeks, upper chest, upper arms or back. By the way, mucin deposition is a classical feature on histology in lesions of lupus erythematosus.
8. Drug-induced lupus erythematosus, which are often caused by drugs like Hydralazine, Carbamazepine, Lithium, Phenytoin, Sulphonamides and Minocycline.

• A person who is suspected to have LE should consult a doctor and have some tests taken like CBC (or FBC: complete blood count), ANA (anti-nuclear antibodies) and other autoantibodies and other tests. Skin biopsy may also be done (histology and immunofluorescence).

• If one has LE, one should make effort to improve his or her appearance and prevent scarring. This can be done by stopping the drugs taken if the LE is drug induced. If you are smoking, you should stop smoking (or consuming nicotine).
• Make sure that you are well protected from the sun. You should stay indoors whenever possible when your shadow is shorter than you (typically between 10am and 2pm). If you cannot avoid going out, you should wear a broad brimmed hat, long sleeves, high collar, long trousers or skirt, socks and shoes. You should also apply broad spectrum sunscreen of at least SPF 30+ (sun protection factor) to all exposed skin.
• You may also apply makeup and other cosmetics to hide your scars and depigmentation.

• Your doctor may give you steroid creams or ointment to be applied to the skin lesions. Use them as directed by the physician and use them in a specified amount of time only. Some doctors may prescribe pimecrolimus cream or tacrolimus ointment.
• Your doctor may also consider giving you antimalarial drugs such as chloroquine, hydroxychloroquine and quinacrine which have anti-inflammatory properties. Use them only as specified by the physician and attend all follow-up appointments.
• Your doctor may also prescribe you with some treatments such as oral retinoids (acitretin, isotretinoin), Dapsone, Methotrexate, Cyclosporin, Gold, Clofazamine, Cyclophosphamide, Thalidomide, intravenous immunoglobulin and biological response modifiers including Rituximab.  Always use them according to your doctor’s prescription.