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The Lupus Butterfly Rash: Information You Need to Know

 

Contents:


What is a malar or butterfly 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 reddish over the affected area. They can be flat or raised in nature.  The rash can be mild or severe, but it 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 in its more severe forms. 
 
It is called the “butterfly rash” due to its butterfly-like shape.  Actually, this particular kind of rash is so common for those diagnosed with lupus – around 40%  – it has become an international symbol for lupus and is even used in the logos of many lupus organizations – including Kaleidoscope Fighting Lupus!   

 

What can cause a butterfly rash?

  •  Systemic Lupus Erythematosus (SLE): SLE is a chronic autoimmune disease that causes an attack on the cells, tissues and organs of the body. This may actually damage the organs and/or render the organs dysfunctional.  The organs most often affected are the brain (and other parts of the nervous system), heart, lungs, liver, kidneys, blood vessels, joints, the tissues and the skin – the body’s largest organ. No one knows for sure what triggers SLE, but some suggested causes for lupus to present in the body are either genetics, or exposure to environmental pollutants. Both men and women can be affected with lupus, but it is predominately found in women between the child-bearing ages of 15-44.   It is also more common in women or African American, Asian, and Latino decent. 
  • Bloom Syndrome: This is a syndrome that causes abnormalities in the pattern of chromosomal arrangement in a person. One of its effects is the development of rashes on the epidermis including the butterfly or malar rash.
  • Lyme disease: This disease is caused by a bacteria, called Borrelia burgdorferi.  It is transmitted to people from the bite of infected blacklegged ticks. It can affect the eyes, heart, the nervous system, the musculoskeletal system and the skin. One of the effects  on the skin can be manifested in the form of the butterfly rash.
  • Erysipelas: This is a serious skin infection caused by another group of bacteria, most commonly Streptococcus pyogenes.  It can produce acute inflammation, pox and blisters on the skin and sometimes a red and painful malar rash.
  • Seborrhea or Seborrheic  Dermatitis: It is a chronic skin disease that just not causes the scaling of the skin and dandruff formation in the hair, but also leads to the development of rashes on the skin and scalp, including malar rashes on the face, chest and neck.
  • Dermatomyositis: This is a connective tissue disorder that causes skin and muscular inflammation and is one of the root causes behind malar or butterfly rash.
  • Photosensitivity: Overexposure to harmful rays of the sun also causes butterfly or malar rash, especially to those who have extreme photosensitivity.

How do I know if it’s lupus? 

Could having the butterfly rash be a sign that you have lupus, or is it something else entirely? Here is where it gets a touch confusing.  As we know, rashes, in general, can be caused by all sorts of things ranging from allergies, illnesses, body temperature shifts, to eczema, to changing your skin care products or laundry detergent.  So simply having a rash, even a butterfly rash is no reason to panic. Another factor that makes it difficult for a non-physician to determine what you may have is that Rosacea and the lupus butterfly rash can look very similar.  It is important to take into account any other unusual-to-your-body symptoms you may be experiencing; such as profound fatigue, painful swollen and/or stiff joints, fever, weight changes, and if you are presenting with Raynaud’s Syndrome.
 
The American College of Rheumatology has created a set of criteria to assist physicians in making a diagnosis of lupus. The individual must have 4 of the 11 specific criteria to be diagnosed with lupus. It is important to remember that having some of the following symptoms does not mean that lupus is the diagnosis.
 
The criteria include the following:
  • Malar rash – a rash shaped like a butterfly that is usually found of the bridge of the nose and the cheeks.
  • Discoid rash – a raised rash usually found on the head, arms, chest, or back.
  • Photosensitivity
  • Oral ulcers
  •  Arthritis – Inflammation of the joints
  •  Heart or lung involvement
  •  Kidney problems
  •  Seizures or other neurological problems
  •  Antinuclear antibodies (ANA): Positive test results
  •  Changes (lowering) in normal blood cell counts
  • Immunological disorder: positive test for certain antibodies

Differences Between Rosacea and Malar Rashes

This is not an easy distinction to make, but these following detailed descriptions may help.  It is important that if you have any concerns about a persistent and unexplained rash, you seek medical attention from a dermatologist (a physician who specializes in the treatment of the skin) immediately.

Rosacea has three stages:

1. Pre-rosacea: You flush easily, but it goes away.
2. Vascular rosacea: You flush easily and it stays longer as the capillaries in your face stay dilated longer. Your skin may be more sensitive.
3. Inflammatory rosacea: Skin stays red; inflammation and acne-type cysts occur. In men, the nose may become enlarged and bulbous (i.e., President Clinton had rosacea!).
 
As rosacea is caused by inflammation and is easily irritated, it may indeed feel hot. Never apply moisturizer to rosacea to try to calm it unless it’s a dermatologist-recommended moisturizer. Rosacea can be treated with prescription topical medications that also contain moisturizer.  Rosacea rarely clears up on its own and has a tendency to worsen with time so, again, please see a medical professional for any unexplained persistent rash or irritation. 
 

Types of Lupus Rashes:

Now that we have discussed and described the symptoms of rosacea rashes, here are the three types of lupus rashes in order to make contrasts and comparisons.

1.  Acute cutaneous lupus (also known as the lupus butterfly rash or malar rash)As described at the beginning of this blog, the name is 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.

 

Treatment

It is of crucial importance to consult a dermatologist for the treatment of malar rash or any other facial rashes since facial skin is usually very sensitive in nature. Some of the prescribed medicines and/or measures are:

  •  Sunscreen lotions: For those whose skin is too very sensitive to the rays of the sun and are on the treatment for malar rash must apply UVA and UVB sunscreen lotions with SPF of not less than 30 in order to be protected from the harmful UV rays.
  •  Avoid sunlight as much as possible. Many lupus patients have photosensitivity.
  •  Steroidal creams: Application of steroidal creams prescribed by the doctor helps in reducing the inflammation involved in case of malar rash and may even provide relief from itching, should that occur.  These creams may cause dilated blood vessels and should be used cautiously on the face. 
  •  Drugs: Non-steroidal drugs which are both anti-malarial and anti-inflammatory in nature are prescribed to treat lupus malar or butterfly rash. Moreover, disease modifying anti-rheumatic drugs (DMARDs) and Immunosuppressive drugs are recommended for severe malar rash. They help in treating the disease well and also prevent the recurrence of the disease.
  • Alternative home remedies for soothing skin: Besides these medications, one can also treat malar rash at home by applying vitamin E oil, olive oil, cod oil, and even a pinch of baking soda on the affected area since it reduces the irritation and pain involved. One may take a bath using oatmeal ingredients in warm water and may also apply fresh Aloe Vera gel on the affected area so to allow the fast and effective healing of the butterfly rash.

Prevention Tips

Because these rashes attack the skin, it only makes sense to protect your skin from direct exposure to sunlight and even strong florescent lights.  Follow these five rules to minimize sun exposure and help prevent or at least reduce much of the discomfort from lupus rashes for those with photosensitivity.
  1. Stay in the shade, especially during the midday hours.
  2. Wear clothing that protects exposed skin.
  3. Wear a wide-brimmed hat to shade the face, head, ears, and neck.
  4. Wear sunglasses that block as close to 100% of both UVA and UVB rays as possible.
  5. Use sunscreen with SPF of 30 or higher and protects agains both UVA and UVB.

Dr. Nisha Desai Presentation on Lupus and Your Skin

This presentation on the possible skin manifestations of lupus by Dr. Nisha Desai took place during the Kaleidoscope Fighting Lupus Symposium:  “Lupus Through the Looking Glass.”  This video includes descriptions of the cutaneous forms of lupus, vasculitis, discoid lupus, panniculitis and others – as well as diagnostic techniques and courses of treatment.

 

 In Conclusion

As you can see, there are several causes for the reddening and rashes that can present themselves in the form of a facial, malar rash.  If you have a persistent and/or unexplained rash, get it checked out by a medical professional  (a dermatologist) for proper diagnosis and treatment.  If you are living with lupus and have experienced a malar rash, you probably already know many of the triggers that might produce the rash, but it doesn’t hurt for everyone to take precautions.  We hope this blog has been helpful and informative. 

 

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References

Bisno, A.L., Stevens, D.L. (1996). Streptococcal infections of skin and soft tissues. The New England Journal of Medicine. 334(4): 240–245.
Hoverson, K., Jarell, A., & Wohltmann, W. (2017). Lupus erythematosis tumidus of the scalp masquerading as alopecia areata. Cutis 99(2), E22-E24.
Kasper, D.L., Fauci, A.S., & Hauser, S.L., (2015). Harrison’s Principles of Internal Medicine. New York, NY: McGraw-Hill.
Lyme disease. (n.d.)  Retrieved from https://www.cdc.gov/lyme/index.html

 

Author:  Kerry Sundbom (2015)

Updated by the Kaleidoscope Fighting Lupus Team (2019)

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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.