Lupus is a widespread and chronic (lifelong) autoimmune disease that, for unknown reasons, causes the immune system to attack the body’s own tissue and organs, including the joints, kidneys, heart, lungs, brain, blood, or skin. Lupus causes a wide variety of devastating symptoms. It can affect nearly every organ in the body with no predictability, causing widespread infections and inflammation. Discoid lupus, the topic of this blog, is just one of the four types of lupus and is isolated to the skin.
What are the four types of lupus?
- Cutaneous lupus erythematosus (also known as chronic cutaneous lupus): Cutaneous lupus was the first type of lupus to be diagnosed. This type affects the skin and can cause thick, red, scaly rashes on the face, neck, and scalp that can lead to scarring. There are three types of cutaneous lupus rashes (discoid being one of them) that we will discuss in detail below.
- Drug-induced lupus erythematosus: Drug-induced lupus is a rare, almost always temporary form of lupus that can occur as a side effect of certain medications, including several drugs commonly used to treat heart disease and hypertension. Unusual when compared to statistics for other forms of lupus, men are more likely to develop drug-induced lupus than women. Drug induced lupus only occurs after long-term (months to years) daily use of a medication, and once the medication is stopped, symptoms of drug-induced lupus typically disappear completely within six months. Drug induced lupus does not lead to systemic lupus.
- Neonatal lupus erythematosus: This is a rare form of lupus in newborn babies whose mothers have lupus that can cause problems at birth or in rare cases, a serious heart defect. This occurs when a mother with certain kinds of lupus [antibodies] transfers them to her child at the time of birth. The mother may have the antibodies but not have lupus herself. In fact, less than 50% of mothers of babies with neonatal lupus actually have lupus.
- Systemic lupus erythematosus or SLE: Systemic lupus causes inflammation in multiple organs and body systems. SLE is a widespread and chronic autoimmune disease that, for unknown reasons, causes the immune system to attack the body’s own tissue and organs, including the joints, kidneys, heart, lungs, brain, blood, or skin. 90% of those affected with lupus are women between the ages of 15 and 45, and of those, two-thirds are people of color.
What are the different types of lupus rashes?
There are several types of cutaneous (meaning of, relating to, or affecting the skin) lupus rashes. In this blog, we will be focusing primarily on the discoid lupus rash. For reference, the other types of lupus rashes are described below with accompanying images.
1. Acute cutaneous lupus (also known as the lupus butterfly rash or malar rash): Derived from the Latin word ‘mala’ which means cheekbone, the malar or butterfly rash is a kind of skin condition typically characterized by the appearance of rashes across the cheekbones and over the bridge of the nose. These rashes are usually red or purple in color in either a blotchy pattern or completely red over the affected area, and can be flat or raised in nature. The rash can be mild or severe but is not usually painful. It can be itchy if it is more like a rash than a blush and some patients even report a ‘hot’ feeling with more severe malar rashes. It is called the lupus butterfly rash because on the face, its shape resembles the outstretched wings of a butterfly across the nose and cheeks. Because the butterfly rash is one of the most visible and recognizable symptoms of lupus (although it only occurs in about 40% of lupus patients) many lupus organizations have the butterfly as their symbol.
2. Subacute cutaneous lupus lesions, which can be divided into two categories:
a. The first type is highly sensitive to sun exposure (called photosensitivity) and looks like red pimples as the rash begins to develop. It can also be described as a psoriasis-like lesion with red scaly patches on the arms, shoulders, neck, and trunk with fewer patches on the face. These pimples become larger and scales begin to appear as the rash persists. Patients typically complain of moderate to severe itching associated with this rash. Again, sun exposure usually worsens this rash, and it can appear on the face, chest, and arms, etc. b. The second type starts as flat lesions and get bigger as they expand outward creating a red ring-shaped lesion with a slight scale on the edges. Over time, the center of these reddened areas lightens so that eventually the rash can look like a series of circular red areas with holes in their centers. This can appear on the face, neck, chest, arms, and back. These rashes, too, are itchy and worsen with sun exposure. These rashes usually heal without scarring, but can leave a non-depressed scar or area of de-pigmentation where the rash occurred. 3. Chronic cutaneous lupus (also called DLE, discoid lupus erythematosus): These lesions are found in only about 20% of SLE patients. Chronic discoid lupus is also found in people who have no trace of systemic lupus. In discoid lupus patients, the lupus is confined to the skin only. The lesions are rarely found below the chin, occurring most often on the scalp (often causing hair loss), and outer ear, and almost never on the legs. These are usually slightly elevated red or pink areas that form flakes or a crust on the surface of the skin. The center area will become depressed and scar over time as these lesions mature. They may be itchy and get larger, spreading outward and then leaving a central scar. In individuals with darker complexions, the central area can become de-pigmented; in all individuals the outer red area may become hyper-pigmented.
Discoid lupus lesions can be very disfiguring and should be treated by a medical professional quickly and aggressively to stop their progression.
Factoid: Musical artist Seal has lupus and the scarring on his face was caused by discoid lupus.
Who gets discoid lupus and can it turn into systemic lupus?
As with SLE, discoid lupus is much more common in women than in men. Discoid lupus can occur at all ages although usually between the ages of 20 and 45, and among all ethnic groups. The condition only progresses to systemic lupus erythematosus in about 10% of people with discoid lupus. However, it is also a possibility in those people that they already had SLE and discoid lesions were just the first identifiable symptom.
No. Discoid lupus is not contagious and neither is any form of lupus. You cannot ‘catch’ lupus from someone.
How is it diagnosed and treated?
A diagnosis is usually made by physical examination, but tissue samples may be taken to a lab for diagnosis as well. If you suspect that you may have discoid lupus, please seek the advice of a dermatologist, a physician who specializes in the diagnosis and treatment of skin conditions. Because discoid lupus can lead to scarring, hair loss, and pigmentation (color) changes in the skin, early recognition, diagnosis, and treatment will increase the chances of a positive outcome. Physicians will usually prescribe topical steroids (as anti-inflammatory medication) and antimalarial medications such as Hydroxychloroquine or Plaquenil to manage discoid rashes. However, some cases of discoid lupus erythematosus may not respond as well to this standard treatment/management plan. In these cases the physician may offer other options for treatment such as retinoids or immunosuppressant medications like azathioprine, cyclosporine, mycophenolate mofetil, and methotrexate. Although there is currently no cure for discoid lupus, drug treatment is usually effective in relieving symptoms, and people with this type of lupus can lead active, productive lives.
Tips and Advice
Because these rashes are photosensitive and attack the skin, it only makes sense to protect your skin from direct exposure to sunlight, reflective sunlight, and even to florescent lights (which also emit small amounts of UV radiation). Lupus patients in general often experience photosensitivity so practicing sun safety can be of great benefit to someone suffering with any of the lupus rashes. In conclusion, if you have a persistent and unexplained rash, get it checked out by a medical professional to get proper treatment. For those of you who have been diagnosed with lupus, are under the care of a physician already, and want to reduce the chances of developing a lupus rash and prevent lupus flares, stay out of the sun! We hope this blog has been helpful and informative. If so, we welcome your comments and suggestions and encourage you to share this blog with others who may find it helpful.
Sources: www.jabfm.org, www.medterms.com, www.everydayhealth.com, www.webmd.com, emedicine.medscape.com, www.hopkinslupus.org, lupus-info, cancer.dartmouth.edu
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