Lupus is an autoimmune disease that causes the immune system to attack its own tissues. Autoimmunity is a term for when the body cannot differentiate between unhealthy invaders (diseases, bacteria, germs, etc.) and its own healthy tissues (skin, organs, etc.). There are many subcategories of lupus, but all forms are chronic and do not have a cure. The National Resource Center on Lupus estimates that at least 1.5 million Americans suffer from some form of Lupus.
Cutaneous lupus (pronounced kyoo-tey-nee-uh-s loo-puh-s) is a form of lupus that is related to and affects a person’s skin. There are four types of lupus — systemic, cutaneous, drug-induced and neonatal — but cutaneous lupus was the first type to be identified. An inflammatory skin disease, it can occur on its own or in conjunction with other forms of lupus, most commonly system lupus erythematosus.
No forms of lupus are contagious, but like so many other skin conditions, visible effects of the disease (like rashes and scarring) can negatively influence someone’s self-esteem or confidence. Below, we take a closer look at the types of cutaneous lupus and who typically gets diagnosed with this disease.
Who gets Cutaneous Lupus?
Similar to systemic lupus, cutaneous lupus is seen more often in women, though the disease also affects men. While it can be diagnosed at any time, diagnosis is often made between 20 and 45 years of age. The disease can present on its own or in conjunction with other forms of lupus. For patients diagnosed with systemic lupus, it is estimated that 85% will experience some form of cutaneous lupus during their disease journey.
What are the Subtypes of Cutaneous Lupus?
Cutaneous lupus can be divided into three subtypes:
Chronic Cutaneous Lupus (CCLE)
Subacute Cutaneous Lupus (SCLE)
Acute Cutaneous Lupus (ACLE)
Chronic Cutaneous Lupus:
If you’ve heard the term Discoid Lupus (DLE) before, you may not have been aware that DLE falls under the CCLE subtype. It is the most common type of CCLE, and appears in the form of disk-shaped lesions that are red or pink scaly and raised. While these lesions most often develop on the scalp, outer ear, or facial area, they can appear on other parts of the body as well. The round lesions usually get larger over time and can leave a scar. Additionally, lesions that emerge on the scalp can cause hair loss, which may be permanent if scarring ultimately occurs.
Although these lesions typically don’t hurt, some report itching or flaking of the skin around the lesion area. The outer edges of DLE lesions can appear hyper-pigmented (darker in color), and patients with darker complexions may experience de-pigmentation near the center of the lesion.
It’s crucial for anyone with DLE to be vigilant about limiting sun exposure, as lesions are photosensitive (reactive to sunlight). If possible, plan outdoor activities before or after peak sunlight hours (10 AM to 4 PM), use sunscreen and wear protective clothing.
In many cases, DLE occurs alone, without any other forms of lupus, however up to 20% of patients already living with systemic lupus erythematosus experience DLE lesions at some point. Lesions caused by DLE can cause permanent scars and even disfiguring of the body. It is imperative that those with DLE see a physician who can prescribe aggressive medical treatment to help stop the progression of the disease.
Subacute Cutaneous Lupus:
Taking appearance and symptoms into consideration is key when diagnosing someone with SCLE, as this subtype can be further divided into two distinct rashes. One SCLE rash type first appears as small red pimples that begin to grow into scaly patches. These patches may resemble psoriasis, but are actually called “annular lesions.” The affected person may experience moderate to severe itching, and notice this type of SCLE rash worsening with any sun exposure. This type of SCLE most often appears on the arms, neck, shoulders, and trunk of the body, with occasional instances on the face.
The second type of SCLE rash appears in a ring shape, is deep red, and may eventually exhibit some scaling around the edges. Like DLE rashes, this ring rash is extremely photosensitive and will worsen when exposed to sun. Typically seen on the neck, face, arms, back and chest, this particular rash won’t scar the skin but may leave a de-pigmented area.
Acute Cutaneous Lupus:
When discussing lupus, the infamous “butterfly rash” almost always comes up. This rash, also known as a malar rash, is the most commonly seen form of ACLE. Per its name, the rash spreads out, similar to the shape of a butterfly, over the bridge of the nose and across the cheeks. Often reddish pink or purple in color, it can vary from mild to severe in intensity. Symptoms associated to the butterfly rash differ among patients with some reporting itchiness or a feeling of heat in the affected area. According to Molly’s Fund, approximately 40 percent of lupus patients will be affected by this type of rash. Because it has become one of the most distinguishable lupus symptoms, the butterfly has since become a universal sign for the disease among most lupus organizations and patients.
How is Cutaneous Lupus Diagnosed and Treated?
Now that you know more about cutaneous lupus, you may be wondering how it’s identified. Cutaneous lupus must be diagnosed by a physician, typically a dermatologist who may decide to take tissue samples to confirm a diagnosis. Because some forms of cutaneous lupus can cause permanent scarring and hair loss, plus loss in pigmentation, it’s crucial to see your doctor if you are experiencing a similar sounding skin rash.
In fact, proper diagnosis early on can lead to quicker treatments and in many cases, positive outcomes.
How do you Treat Cutaneous Lupus?
The standard treatment for cutaneous lupus revolves around topical steroids (anti-inflammatory creams), plus Plaquenil or its generic, Hydroxycholoroquine. This antimalarial medicine is often a first line treatment for both cutaneous and systemic lupus.
In severe cases that do not respond to these medications, a dermatologist may recommend retinoids. Immunosuppressant drugs, such as methotrexate, azathioprine, or cyclosporine, may also be prescribed.
Currently there is no cure for lupus, but these medications can help relieve symptoms and assist some patients in experiencing remission-type periods with little to no disease activity. With aggressive treatment, many patients can lead fulfilling and productive lives.