The Lupus Butterfly Rash: Information You Need to Know

At least half of individuals with lupus have the malar – or butterfly – rash. Read on to learn more about this common, characteristic condition for those living with lupus.

Introduction

Causes of the Malar Rash

Diagnosing Malar Rash and Lupus

Treating and Preventing Malar rash

Mental Health and Malar Rash

Dr. Nisha Desai:  Lupus and Your Skin

In Conclusion

 


Introduction

Often called the “butterfly rash” because of its unique shape, the malar rash is one of the most recognizable symptoms of lupus. Historians even suspect historical figures, such as Louisa May Alcott, may have suffered from lupus due to the identifiable flush painted across her face in her portraits. Malar rash is so identifiable, in fact, that the butterfly has become the international symbol for lupus as well as part of the logo and branding scheme for many lupus organizations such as Kaleidoscope Fighting Lupus.

Malar rash spreads across the bridge of the nose and onto the cheeks, avoiding the nasal folds (“smile lines”) on either side of the nose. It can be light pink, in mild cases, to almost purple when it is more severe. Malar rash can be flat or appear raised, and while it may be itchy and feel hot, it is not painful. The malar rash is a localized symptom of acute cutaneous lupus erythematosus (ACLE), a type of cutaneous lupus that is often triggered by sunlight, is transient or temporary and non-scarring.

 

Causes of the Malar Rash

Though malar rash is a common characteristic of lupus, it is not unique to lupus. Malar rash can be caused by these other disorders as well:

  • Erysipelas: Caused by bacteria, this skin infection often appears as shiny, pink, painful and swollen areas on the top layers of the skin, typically on the feet and legs (and sometimes the face). It is also called “St. Elmo’s Fire.”
  • Cellulitis: Cellulitis is similar to erysipelas, but affects subcutaneous fat and goes deeper into the dermis. It may also develop on the palms of the hands as well as the face and is much deeper red in color than erysipelas.
  • Pellagra: The result of a niacin (vitamin B3) deficiency, an individual with pellagra may not only develop a rash, but also diarrhea, dermatitis and possibly dementia as well if not treated.
  • Pemphigus erythematosus. An autoimmune disease, pemphigus erythematosus causes blistering of the skin when rubbed and may also affect the mucous membranes.
  • Dermatomyositis: Known as an inflammatory myopathy, dermatomyositis is a rare muscular disease that is often accompanied by rash.
  • Atopic, allergic, contact and seborrheic dermatitis: These are itchy inflammatory conditions that may be triggered by factors such as the environment, stress, irritants, allergic reactions and dry skin.
  • Lyme disease: This condition is also called Lyme borreliosis because it is caused by a type of Borrelia  Lyme disease may not only affect the skin, but the central nervous system, joints and heart as well.
  • Bloom syndrome: A rare, inherited condition, Bloom syndrome is often characterized by photosensitivity, telangiectases (spider veins) and increased susceptibility to infections and respiratory conditions.
  • Photosensitivity: Individuals who are photosensitive have a heightened sensitivity to ultraviolet light (UV) exposure. Certain medications and skincare products may cause photosensitivity, or it may be the result of an underlying medical condition such as an autoimmune disorder.
  • Sunburn: Skin that is exposed to UV light – specifically the burning rays of UVB – and is unprotected for prolonged periods of time may burn, turn red, swell and even blister.
  • Rosacea: A common skin condition, rosacea may appear as red skin on the face with telangiectases or even pus-filled bumps that resemble acne. Skin can be sensitive and easily irritated.

 

It is often difficult to distinguish whether a face rash is the result of sunburn, rosacea or SLE. The following are the main characteristics that may differentiate one of these conditions from another:

  • A sunburn typically occurs over a broader area of the face and/or other areas of the body that were recently vulnerable to prolonged, unprotected sun exposure – skin will also feel painful and like it is burning.
  • Rosacea burns and stings the face as the skin becomes sensitive in this area. An individual with rosacea may also have telangiectasia that is visible on the cheeks and experience no other systemic symptoms. Rosacea may also resemble acne. Certain products (alcohol, fragrance, acids) may also exacerbate the problem.
  • SLE may trigger symptoms such as a rash across the nose and cheeks as well as a fever, photosensitivity, joint pain and fatigue. While the rash may sometimes be the only symptom, it may also appear along with these other symptoms indicating a lupus flare.

Regardless of the condition, if an individual experiences any new and inexplicable symptoms, the advice of a healthcare practitioner should be sought immediately.

 

Diagnosing Malar Rash and Lupus

Diagnosing malar rash and determining whether or not it is a symptom of lupus can be difficult. However, a healthcare practitioner, in particular a dermatologist, will be able to properly diagnose whether the rash is caused by lupus or another condition.

To begin with, a practitioner will look at an individual’s health history and run all necessary lab tests that include checking for antibody activity, kidney damage, joint involvement, abnormal blood cell counts and any other skin issues. They will also take into account if an individual is feeling fatigued, running a fever, is achy or has any other questionable symptoms.

Once the practitioner has all of the lab results and the individual’s complete health and symptom history, they will evaluate the information against a diagnostic tool such as the EULAR/ACR Classification Criteria for SLE. In 2019, the European League Against Rheumatism (EULAR), in partnership with the American College of Rheumatology (ACR), developed this new protocol for diagnosing lupus. Symptoms are weighted on a scale of one to ten and a cumulative score of ten or more points across all symptoms may indicate that an individual more than likely has lupus. Cutaneous symptoms are weighted heavily on the scale. ACLE alone weighs six points, indicating the significance of malar rash in diagnosing lupus.

 

Treating and Preventing Malar Rash

In individuals with lupus, treating the individual holistically will also treat the rash they may have.  Effective systemic treatments include:

  • Antimalarials: Hydroxychloroquine (Plaquenil), chloroquine and quinacrine.
  • Corticosteroids: Prednisone and methylprednisolone.
  • Oral retinoids: Isotretinoin and acitretin.
  • Immunosuppressants: Methotrexate, mycophenolate mofetil, mycophenolate sodium, azathioprine and clofazimine.
  • Immunomodulators: Dapsone, thalidomide and lenalidomide.
  • Biologics: Intravenous immunoglobulin (IVIG) and rituximab.

 

Effective topical treatments that specifically target skin rashes, lesions and ulcers include:

  • Topical corticosteroids: Methylprednisolone, triamcinolone, acetonide, betamethasone valerate and clobetasol.
  • Calcineurin inhibitors: Tacrolimus and pimecrolimus.
  • Physical therapies: Pulsed-dye laser therapy and cryotherapy.

 

Ultraviolet light (UVA and UVB) can trigger malar rash. So, staying protected from the sun and any other sources of UV light along with practicing good skincare habits can help to prevent malar rash and treat skin. The following are preventative and self-care steps to take to protect skin and maintain good skin health:

  • Wear sunscreen: Wearing a broad-spectrum sunscreen that covers both the UVA and UVB spectrum daily is imperative. Using SPF 50 or higher is ideal. Individuals with acne-prone or sensitive skin may opt for physical barrier sunscreens, such as titanium dioxide and zinc oxide, especially for the face. These sit on top of the skin to block UV rays and are often recommended over chemical sunscreens (e.g. avobenzone, oxybenzone), which are absorbed deeper into the skin and may cause irritation to sensitive-prone areas. Apply the sunscreen at least 20 minutes before exposure, and if spending time outdoors, reapply every two hours or more frequently if you are exposed to water or excessively sweating. An added bonus is that sunscreen also prevents premature aging of the skin!
  • Wear protective clothing: Hats, sunglasses, long sleeves and long pants can help protect skin from the sun. Lightweight, ultraviolet protection factor (UPF) clothing is now more readily available and works similar to sunscreen by shielding the skin from damaging rays. If UPF clothing is unavailable, however, stick to darker colored fabrics that are tightly woven from synthetic fibers, such as polyester, to block out as much sun as possible.
  • Seek shade: While the heat of the sun may feel great especially after a long winter, minimize the amount of direct sun exposure. Seek the shade of an umbrella, an awning or a tree when outside. Plan breaks to go inside when possible.
  • Use minimal and gentle skincare products: While the urge may be to splurge on the latest skincare hype, a minimalist approach to skincare may be the most helpful. Use products that are free of acid, alcohol, dyes, fragrance and other irritants. Tried-and-true ingredients will help keep skin looking and feeling its best and avoid the risk of causing a reaction.
  • When in doubt, ask a dermatologist: A good dermatologist can help to make wise skincare choices specifically suited to an individual’s unique skincare needs.

A combination of these measures can help defend the skin against triggering and damaging UV light, as well as help repair skin so that it is as healthy and supple as possible.  This may help to minimize both the occurrence and appearance of malar rash.

 

Mental Health and Malar Rash

How an individual looks can affect how they feel. Coping with malar rash may make an individual feel self-conscious and needlessly ashamed of their appearance. While many may feel bad about the way they look from time-to-time, coping with the effects and physical symptoms of chronic illness such as lupus can take its toll. Mental health therapy is one way to learn ways to cope as well as raise self-esteem. There is no stigma against needing and seeking help.

Using makeup can be another way to feel better. There are many types of makeup and brands that cater to various skincare needs. Picking up a tip or two about how to properly apply these products can help to camouflage a rash if an individual has difficulty embracing it. A simple internet search can unearth the necessary tools and techniques of the trade.

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Dr. Nisha Desai:  Lupus and Your Skin

Watch as Dr. Nisha Desai, dermatologist and hair loss specialist at the Northwest Dermatology and Research Center in Portland, Oregon, as she discusses the symptoms, management and treatment of cutaneous lupus, discoid lupus, panniculitis and vasculitis.

 

 In Conclusion

The malar or butterfly rash is one of the most common and visible symptoms of lupus. While there is no cure for these rashes, taking the right precautions to prevent them, and receiving proper treatment when they appear can lessen their effects and improve your overall health.

 

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References

All about the skin. (n.d.). DermNet NZ. Retrieved June 2, 2020 from https://dermnetnz.org/

Aringer, M., Costenbader, K., Daikh, D., Brinks, R., Mosca, M., Ramsey-Goldman, R., … Johnson, S., (2019). 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Arthritis & Rheumatology. Retrieved from: https://ard.bmj.com/content/annrheumdis/78/9/1151.full.pdf

Lupus-specific skin disease and skin problems. (2020). Johns Hopkins Lupus Center. Retrieved June 2, 2020 from https://www.hopkinslupus.org/lupus-info/lupus-affects-body/skin-lupus/

Okon, L. & Werth, V. (2013). Cutaneous lupus erythematosus: Diagnosis and treatment. Best Practice & Research: Clinical Rheumatology, 27(3), 391-404. doi: 10.1016/j.berh.2013.07.008. Retrieved June 2, 2020 fromhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3927537/pdf/nihms-517024.pdf

Photosensitivity & your skin. (2020). Skin Cancer Foundation. Retrieved June 2, 2020 from https://www.skincancer.org/risk-factors/photosensitivity/

Rosacea. (2020). Mayo Clinic. Retrieved June 2, 2020 from https://www.mayoclinic.org/diseases-conditions/rosacea/symptoms-causes/syc-20353815

Uva, L., Miguel, D., Pinheiro, C., Freitas, J., Gomes, M., & Filipe, P. (2012). Cutaneous manifestations of systemic lupus erythematosus. Autoimmune Diseases, article 834291. doi: 10.1155/2012/834291. Retrieved June 2, 2020 from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3410306/pdf/AD2012-834291.pdf

Winkelmann R., Kim, G., & Del Rosso, J. (2013). Treatment of cutaneous lupus erythematosus review and assessment of treatment benefits based on Oxford Center for evidence-based medicine criteria. Journal of Clinical and Aesthetic Dermatology, 6(1), 27-38. Retrieved June 2, 2020 from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3543290/pdf/jcad_6_1_27.pdf

 

 

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.