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Cutaneous Lupus

Contents:

What is lupus?

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.  There are four types of lupus, varying in severity from chronic cutaneous lupus (CCLE), the mildest form, to systemic lupus erythematosus (SLE), the most severe form affecting internal organs and systems.  It is very important to have a dermatologist properly evaluate any new or unusual rashes to determine the extent of skin involvement and diagnose whether there is any internal involvement.

What are the four types of lupus?

  1. 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.
  2. 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. Unlike when compared statistically to 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.
  3. 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.
  4. 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 cutaneous lupus?

There are several types of cutaneous (meaning of, relating to, or affecting the skin) lupus rashes. Cutaneous lupus can be categorized into four main types: chronic cutaneous lupus (CCLE), subacute cutaneous lupus (SCLE), acute cutaneous lupus (ACLE) and intermittent cutaneous lupus (ICLE). For reference, all of the types of cutaneous lupus are described below with accompanying images.

  1. Acute cutaneous lupus (ACLE): The lupus butterfly rash or malar rash is the most common form of ACLE. 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.  Other lesions associated with ACLE include oral or nasal ulcers, hives, and/or temporary hair loss, which are replaced by new hair once the disease flare is treated.
  2. Subacute cutaneous (SCLE) lupus can be divided into two categories:
    1. This type is highly sensitive to sun exposure and looks like red pimples as the rash begins to develop. It can also be described as a psoriasis-like lesion (called papulosqualmous lesions) 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.
    2. The second type starts as flat lesions and get bigger as they expand outward creating a red ring-shaped lesion (called annular lesions) 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 (CCLE): Discoid lupus erythematosus (DLE) is named for the coin-like shape of the lesions, and is the most common type of CCLE. 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 lower than 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.
  4. Intermittent cutaneous lupus (ICLE) or lupus tumidus: There is some disagreement as to whether ICLE – often called lupus tumidus (LET) – is purely a dermatological disease independent of SLE or a lupus-associated skin disease. Regardless, it bears worth mentioning. A condition that predominantly affects me, ICLE is characterized by lesions that “clinically appear as indurated, succulent, urticarial-like plaques of reddish-violaceous colour, with a smooth surface” and may be round or annular in shape. Triggers include ultraviolet radiation (UV) – after an individual is exposed to the sun without adequate protection and with extreme sun sensitivity, lesions may appear on the cheeks, neck, and anterior chest. The condition typically clears during the winter months and there is no scarring.
Factoid: Musical artist Seal has lupus and the scarring on his face was caused by discoid lupus.

Who gets cutaneous lupus and how does it relate to systemic lupus?

As with SLE, cutaneous lupus is much more common in women than in men. Cutaneous lupus occurs at all ages although usually between the ages of 20 and 45, and among all ethnic groups. Cutaneous lupus can occur in conjunction with people who have Systemic Lupus Erythematosus (SLE) or it can occur on its own. Approximately 85% of people who have SLE will have cutaneous lupus at some point during the course of their disease. Around 20% of SLE patients initially present with some form of cutaneous manifestation (skin rash), making it one of the most common initial symptoms that helps lead to earlier and faster diagnosis of SLE. Back to top

Is cutaneous lupus contagious?

No form of cutaneous lupus is contagious and neither is any other 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 chronic cutaneous lupus (also known as discoid) 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 cutaneous lupus 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 cutaneous lupus, drug treatment is usually effective in relieving symptoms, and people with this type of lupus can lead active, productive lives.

Recent advances in the treatment of Cutaneous Lupus Erythematosus

In 2016, German researchers Kuhn, Landmann, and Wenzel identified several potential options in the treatment of cutaneous lupus erythematosus. While more studies and clinical trials need to be completed to prove the true efficacy of these therapies, they do show promise.

  • Monoclonal antibodies (sirukumab, tocilizumab, sifalimumab, anifrolumab) – these therapies may decrease disease activity including the reduction of skin manifestations and rashes by suppressing the immune system in different ways. Tocilizumab has been used for severe rheumatoid arthritis. Disappointingly, in August of 2018, anifrolumab did not pass its TULIP 1 Phase 3 clinical trials.
  • Kinase inhibitors (ruxolitinib, baricitinib) – used topically, these “anti-inflammatory kinase-inhibitors provide a very promising class of new drugs for the treatment of CLE.” The FDA recently conditionally approved Olumiant, a trade name of baricitinib, for the treatment of rheumatoid arthritis.
  • B lymphocyte stimulator inhibitors (belimumab) – as an adjunct therapy, belimumab (Benlysta) has recently been licensed for use in individuals with lupus who still have high disease activity due to an intolerance to treatment or an “unacceptably high need for corticosteroids.”
  • PDE4 inhibitors – found in T cells, B cells, and dendritic cells, phosphodiesterases (PDE) – specifically phosphodiesterases type 4 (PDE4) – are enzymes that “play a major role in the pathogenesis of inflammatory diseases.” PDE4 inhibitors may therefore prove to be a viable therapy in the treatment of cutaneous lupus erythematosus. Otezla is an example of a PDE4 inhibitor that is commonly used to treat plaque psoriasis.
  • Regulatory T cells (Treg) – though conflicting data of the role of Treg in SLE has been published, more studies may establish the feasibility of using Treg therapy as a treatment alternative.

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 fluorescent 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. We welcome your comments and suggestions and encourage you to share this blog with others who may find it helpful!

 

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References

Cutaneous lupus erythematosus. (2012). Retrieved from: http://www.americanskin.org/resource/lupus.php

Frequently asked questions about cutaneous lupus. (2018). Retrieved from: https://www.utsouthwestern.edu/education/medical-school/departments/dermatology/research/cutaneous-lupus-registry/disease-faq.html

Grönhagen, C. M., & Nyberg, F. (2014). Cutaneous lupus erythematosus: An update. Indian dermatology online journal, 5(1), 7-13.

Kuhn, A., Landmann, A., & Wenzel, J. (2016). Advances in the treatment of cutaneous lupus erythematosus. Retrieved from: http://journals.sagepub.com/doi/pdf/10.1177/0961203316641771

Lin, J. (2018). Subacute cutaneous lupus erythematosus (SCLE). Retrieved from: https://emedicine.medscape.com/article/1065657-overview#aw2aab6b2b2

Lindsey, H. (n.d.). Lupus: Everything you need to know about the disease, what causes it, and how to treat it. Retrieved from: https://www.everydayhealth.com/lupus/

Lupus Overview. (n.d.). Retrieved from: https://www.webmd.com/lupus/arthritis-lupus

Medical definition of discoid lupus. (n.d.). Retrieved from: https://www.medicinenet.com/script/main/art.asp?articlekey=8067

Oakley, A. (2016). Cutaneous lupus erythematosus. Retrieved from: https://www.dermnetnz.org/topics/cutaneous-lupus-erythematosus/

Oke, V., & Wahren-Herlenuius, M. (2013). Cutaneous lupus erythematosus: Clinical aspects and molecular pathogenesis. Retrieved from: https://onlinelibrary.wiley.com/doi/pdf/10.1111/joim.12057

Okon, L., & Werth, V. (2013). Cutanous lupus erythematosus: Diagnosis an treatment. Retrieved from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3927537/pdf/nihms-517024.pdf

Pajwani, S. (2018). Early diagnosis and treatment of discoid lupus erythematosus. Retrieved from: http://www.jabfm.org/content/22/2/206.full

Types of Lupus. (n.d.). Retrieved from: https://www.hopkinslupus.org/lupus-info/types-lupus/

 

 

Author: Liz Heintz

Liz Heintz is a technical and creative writer who received her BA in Communications, Advocacy, and Relational Communications from Marylhurst University in Lake Oswego, Oregon. She most recently worked for several years in the healthcare industry. A native of San Francisco, California, Liz now calls the beautiful Pacific Northwest home.

All images unless otherwise noted are property of and were created by Kaleidoscope Fighting Lupus. To use one of these images, please contact us at [email protected] for written permission; image credit and link-back must be given to Kaleidoscope Fighting Lupus.

All resources provided by us are for informational purposes only and should be used as a guide or for supplemental information, not to replace the advice of a medical professional. The personal views expressed here do not necessarily encompass the views of the organization, but the information has been vetted as a relevant resource. We encourage you to be your strongest advocate and always contact your healthcare practitioner with any specific questions or concerns.

 

 

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