Systemic lupus erythematosus: A chronic inflammatory condition caused by an autoimmune disease. An autoimmune disease occurs when the body’s tissues are attacked by its own immune system. Patients with lupus have unusual antibodies in their blood that are targeted against their own body tissues.
Lupus can cause disease of the skin, heart, lungs, kidneys, joints, and nervous system. When only the skin is involved, the condition is called discoid lupus. When internal organs are involved, the condition is called systemic lupus erythematosus (SLE). Up to 10% of persons with discoid lupus (lupus limited to the skin) eventually develop the systemic form of lupus (SLE).
SLE is eight times more common in women than men. The causes of SLE are unknown. However, heredity, viruses, ultraviolet light, and drugs may all play a role.
Eleven criteria have been established for the diagnosis of SLE:
- Malar (over the cheeks of the face) “butterfly” rash
- Discoid skin rash: patchy redness that can cause scarring
- Photosensitivity: skin rash in reaction to sunlight exposure
- Mucus membrane ulcers: ulceration of the lining of the mouth, nose or throat
- Arthritis: 2 or more swollen, tender joints of the extremities
- Pleuritis/pericarditis: inflammation of the lining tissue around the heart or lungs, usually associated with chest pain with breathing
- Kidney abnormalities: abnormal amounts of urine protein or cellular elements
- Brain irritation: manifested by seizures (convulsions) and/or psychosis
- Blood count abnormalities: low counts of white or red blood cells, or platelets
- Immunologic disorder: abnormal immune tests include anti-DNA or anti-Sm (Smith) antibodies, falsely positive blood test for syphilis, anticardiolipin antibodies, lupus anticoagulant, or positive LE prep test
- Antinuclear antibody: positive ANA antibody testing
The treatment of SLE is directed toward decreasing inflammation and/or the level of autoimmune activity. Persons with SLE can help prevent “flares” of disease by avoiding sun exposure and by not abruptly discontinuing medications.
- Skin’s Immune ‘Alarm’ May Explain Light-Induced Rashes in Lupus Patients
- Lupus-Specific Skin Disease
- Common Lupus Skin Problems
- Photophobia and Eye Symptoms of Lupus
- Secondary Conditions Can Also Lead to Light Sensitivity
- Light Sensitivity and Lupus Medications
- Research on photosensitivity among people with lupus
- Sun Exposure and Lupus
- Lupus & Sun Exposure: All You Need To Know
Skin’s Immune ‘Alarm’ May Explain Light-Induced Rashes in Lupus Patients
Their work builds on a decade’s worth of research examining the link between proteins called interferons and lupus. Interferons are released by cells in response to an invasion. Typically triggered by viruses, they also can be activated by bacteria and other external threats.
Interferons alarm other cells to bolster their defenses. The function is present in all people.
“Interferons are notoriously hard to measure, but we’ve known they are elevated in most lupus patients,” Kahlenberg says. “In this experiment, we set out to see which ones were in the skin.”
Gene editing offers insight
When the researchers compared skin cells from patients with lupus and people with healthy skin, lupus epidermal skin cells called keratinocytes — the keratin-producing cells that make up the top layer of skin — showed a significant increase in interferon kappa (IFN-κ).
Next, they generated keratinocytes without IFN-κ using CRISPR/Cas9 technology, which functions like a pair of genetic scissors, to remove the gene encoding the interferon. They then compared these skin cells with another set designed to overexpress IFN-κ.
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“We found out that all type 1 IFN signaling goes down in basal keratinocytes when you delete, or knock out, the IFN-κ gene using CRISPR/Cas9; we also observed that IFN-κ knockout keratinocytes are unaffected by UV light,” says Mrinal Sarkar, Ph.D., a research investigator with the department of dermatology at U-M.
Conversely, cells overexpressing IFN-κ died when exposed to UV light.
“We think that the probable main function of IFN-κ in normal, healthy skin is to fight off viral infections, such as HPV. But in lupus, this whole system is out of sync and overactive,” explains Johann Gudjonsson, M.D., Ph.D., associate professor of dermatology.
Research moving forward
Even without exposure to UV light, lupus skin had higher baseline levels of IFN-κ. This overabundance appears to amplify the inflammatory response and cell death.
The team is trying to uncover why IFN-κ is elevated in the skin of patients with lupus and how it regulates death in keratinocytes. They also wonder if similar mechanisms are at play in other diseases with photosensitivity as a feature, such as dermatomyositis.
SEE ALSO: Investigating Kidney Biomarkers to Track Lupus
What makes this discovery particularly exciting, the team notes, is that drugs recently approved by the FDA and currently prescribed for rheumatoid arthritis can block interferons.
Using the drug baricitinib, the U-M researchers blocked interferon signaling and made lupus skin cells look like those in normal, healthy skin. Baricitinib is currently in drug trials for lupus, but not for photosensitivity.
“I’m excited to see this go from bench to bedside,” Kahlenberg says. “It may actually happen that some of our work helps to push this forward.”
She was also recently awarded the first Taubman Institute Innovative Project grant with her colleague, Johann E. Gudjonsson, M.D., Ph.D. for their study: Personalized medicine through integration of immune phenotypes in autoimmune skin diseases. The study investigates immune responses in the skin and blood of lupus and psoriasis patient and how disease flares and medications may alter these responses.
The team will analyze tissues at the cellular level, genetic level (using DNA profiling) and the molecular level (using mass cytometry imaging and single cell RNA sequencing) to develop a full picture of immune dysfunction in these patient groups. The five-year study hopes to lead to more targeted, personalized therapies in autoimmune diseases.
The work was in part supported by the University of Michigan Babcock Endowment Fund, the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health under Award Numbers R03AR066337, K08AR063668, K08-AR060802, T32AR007080, R01-AR071384 and R01-AR069071, the A. Alfred Taubman Medical Research Institute Parfet Emerging Scholar Award and Kenneth and Frances Eisenberg Emerging Scholar Award, Doris Duke Charitable Foundation Grant #2013106 and the Rheumatology Research Foundation Career Development K Supplement Award.
This article was originally published on July 25, 2018, and was updated on January 25, 2019.
Summer is here! The sun will be out! And so it is time for those perennial questions from lupus patients: Am I sun-sensitive? What will happen to me if I go into the sun? How much sun is dangerous? Should I wear sunscreens? Here are some factoids for you.
- Patients who are sun-sensitive show that sensitivity in several ways. Most commonly rash appears, or gets worse. Sometimes, however, they develop joint pains, fever and other signs of general flare-up.
- Sun-sensitivity symptoms can show up several days or even a few weeks after heavy sun exposure.
- Only about one out of three lupus patients is sun-sensitive. If you don’t know if you are, or are not, sun-sensitive, it’s OK to try a little (early morning, late afternoon) exposure for a few minutes. If you don’t have any symptoms in a few days, increase a little bit, and continue until you know that you can tolerate full sun exposure. It’s always safest to avoid very strong sun (beaches, sailing, golf courses), and to use the strongest available sunscreens.
- If you do think you are showing signs of a flare-up, check with your doctor right away. Many sun-induced flare-ups are very mild, but some are not, and they may need treatment.
- If you know that you are not sun-sensitive, it is very unlikely that it will later develop. If you know that you are sun-sensitive, assume you will always be.
Reviewed on June 10, 2018
Michael D. Lockshin, M.D., is Director of the Barbara Volcker Center for Women and Rheumatic Disease at Hospital for Special Surgery. He is the author of nearly 300 research papers, book chapters, and books, most on the topic of lupus, pregnancy, antiphospholipid syndrome and sex differences in disease.
Most people with lupus experience some sort of skin involvement during the course of their disease. In fact, skin conditions comprise 4 of the 11 criteria used by the American College of Rheumatology for classifying lupus. There are three major types of skin disease specific to lupus and various other non-specific skin manifestautions associated with the disease.
Lupus-Specific Skin Disease
Three forms of specific skin disease occur in people with lupus, and it is possible to have lesions of multiple types. In addition, a person can also have one of the three forms outlined below without actually having full-blown systemic lupus erythematosus (SLE), but the presence of one of these disease forms may increase a person’s risk of developing SLE later in life. Usually, a skin biopsy is used to diagnose forms of cutaneous lupus, and various medications are available for treatment, including steroid ointments, corticosteroids (e.g., prednisone), and antimalarials (e.g., Plaquenil).
Chronic Cutaneous Lupus Erythematosus (CCLE) / Discoid Lupus Erythematosus (DLE)
Chronic cutaneous (discoid) lupus erythematosus is usually diagnosed when someone exhibits signs of lupus in the skin. People with SLE can also have discoid lesions, and about 5% of all people with DLE will develop SLE later in life. A skin biopsy is used to diagnose this condition, and the lesions have a characteristic pattern known to clinicians: they are thick and scaly, plug the hair follicles, appear usually on surfaces of the skin exposed to sun (but can occur in non-exposed areas), tend to scar, and usually do not itch.
If you are diagnosed with discoid lupus, you should try to avoid sun exposure when possible and wear sunscreen with Helioplex and an SPF of 70 or higher. In addition, you doctor may prescribe medications to help prevent and curb inflammation, including steroid ointments, pills, or injections , antimalarial medications such as Plaquenil, and/or immunosuppressive medications.
Subacute Cutaneous Lupus Erythematosus (SCLE)
About 10% of lupus patients have SCLE. The lesions characteristic of this condition usually do not scar, do not appear thick and scaly, and usually do not itch. About half of all people with SCLE will also fulfill the criteria for systemic lupus. Treatment can be tricky because SCLE lesions often resist treatments with steroid creams and antimalarials. People with SCLE should be sure to put on sunscreen and protective clothing when going outdoors in order to avoid sun exposure, which may trigger the development of more lesions.
Acute Cutaneous Lupus Erythematosus (ACLE)
Most people with ACLE have active SLE with skin inflammation, and ACLE lesions are found in about half of all people with SLE at some point during the course of the disease. The lesions characteristic of ACLE usually occur in areas exposed to the sun and can be triggered by sun exposure. Therefore, it is very important that people with ACLE wear sunscreen and protective clothing when going outdoors.
Common Lupus Skin Problems
About half of all lupus patients experience a characteristic rash called the malar or “butterfly” rash that may occur spontaneously or after exposure to the sun. This rash is so-named because it resembles a butterfly, spanning the width of the face and covering both cheeks and the bridge of the nose. The malar rash appears red, elevated, and sometimes scaly and can be distinguished from other rashes because it spares the nasal folds (the spaces just under each side of your nose). The butterfly rash may appear on its own, but some people observe that the appearance of the malar rash indicates an oncoming disease flare. Whatever the case, it is important to pay attention to your body’s signals and notify your physician of anything unusual.
50% of all people with lupus experience sensitivity to sunlight and other sources of UV radiation, including artificial lighting. For many people, sun exposure causes exaggerated sunburn-like reactions and skin rashes, yet sunlight can precipitate lupus flares involving other parts of the body. For this reason, sun protection is very important for people with lupus. Since both UV-A and UV-B rays are known to cause activation of lupus, patients should wear sunscreen containing Helioplex and an SPF of 70 or higher. Sunscreen should be applied everywhere, including areas of your skin covered by clothing, since most clothing items contain an SPF of only about 5. Be sure to reapply as directed on the bottle, since sweat and prolonged exposure can cause coverage to dissipate.
People with lupus may experience a lacy pattern under the skin called livedo reticularis. This pattern may range anywhere from a violet web just under the surface of the skin to something that looks like a reddish stain. Livedo can also be seen in babies and young women, is more prominent on the extremities, and is often accentuated by cold exposure. The presence of livedo is usually not a cause for alarm, but it can be associated with antiphospholipid antibodies.
About 70% of people with lupus will experience hair loss (alopecia) at some point during the course of the disease. Hair loss in lupus is usually characterized by dry, brittle hair that breaks, and hair loss is more common around the top of the forehead. Physical and mental stress can also cause hair loss, as can certain medications, including corticosteroids such as prednisone. In many cases the hair will grow back, but hair loss due to scarring from discoid skin lesions may be permanent. There is no cure-all for hair loss, but treatments such as topical steroids and Rogaine may be prescribed. Sometimes dealing with the cosmetic side effects of lupus can be difficult, but some people find using hairpieces and wigs to be an effective means of disguising hair loss.
Oral and Nasal Ulcers
About 25% of people with lupus experience lesions that affect the mouth, nose, and sometimes even the eyes. These lesions may feel like small ulcers or “canker sores.” Such sores are not dangerous but can be uncomfortable if not treated. If you experience these types of lesions, your doctor may give you special mouthwash or Kenalog in Orabase (triamcinolone dental paste) to help expedite the healing process.
Approximately one-third of all people with lupus experience a condition called Raynaud’s phenomenon in which the blood vessels supplying the fingers and toes constrict. The digits of people with Raynaud’s are especially susceptible to cold temperatures. Often people with the condition will experience a blanching (loss of color) in the digits, followed by blue, then red discoloration in temperatures that would only be mildly uncomfortable to other people (such as a highly air-conditioned room). It is very important that people with Raynaud’s wear gloves and socks when in air-conditioned spaces or outside in cool weather. Hand warmers used for winter sports (e.g., Hot Hands) can also be purchased and kept in your pockets to keep your hands warm. These measures are very important, since Raynaud’s phenomenon can cause ulceration and even tissue death of the fingers and toes if precautions are not taken. People have even lost the ends of their fingers and toes due to the poor circulation involved in Raynaud’s phenomenon. Cigarettes and caffeine can exacerbate the effects of Raynaud’s, so be sure to avoid these substances. If needed, your doctor may also recommend a calcium channel blocker medication such as nifedipine or amlodipine to help dilate your blood vessels.
About 10% of all people with lupus will experience hives (urticaria). These lesions usually itch, and even though people often experience hives due to allergic reactions, hives lasting more than 24 hours are likely due to lupus. If you experience this condition, be sure to speak with your doctor, since s/he will want to be sure that the lesions are not caused by some other underlying condition, such as vasculitis or a reaction to medication. Your doctor will probably distinguish these lesions from those caused by vasculitis by touching them to see if they blanch (turn white).
Approximately 15% of people with lupus will experience purpura (small red or purple discolorations caused by leaking of blood vessels just underneath the skin) during the course of the disease. Small purpura spots are called petechiae, and larger spots are called eccymoses. Purpura may indicate insufficient blood platelet levels, effects of medications, and other conditions.
Some people with lupus may develop a condition known as cutaneous vasculitis, in which the blood vessels near the skin experience inflammation that ultimately restricts blood flow. This condition can cause hive-like lesions on the skin that may itch and do not turn white when depressed. Other skin abnormalities may also be present, including actual gangrene of the digits. If left untreated, vasculitic lesions may cause ulceration and necrosis (cell death), and dead tissue must be surgically removed. Rarely, fingers or toes with aggressive ulceration and gangrene may require amputation. Therefore, it is very important that you notify your doctor of any skin abnormalities.
Lupus is a chronic autoimmune disease which affects the body’s ability to differentiate between normal cells and foreign invaders such as bacteria and viruses. As a result, those with lupus often times experience symptoms of inflammation, swelling and damage to the joints and other organ systems—many of which can be brought on by light. And some even experience symptoms of photophobia or sensitivity to light, among other eye-related symptoms.
Photophobia and Eye Symptoms of Lupus
There is no clear understanding of exactly how many people with lupus have ocular or neurological sensitivity to light, but many—perhaps up to one-third of all patients—experience eye-specific symptoms, which may contribute to the onset of light-related sensitivities.1 For example, skin or muscle changes around the eye, lesions, visual floaters, dry eyes, inflammation and retinal changes are all side effects of lupus that can contribute to a person’s photophobic responses.2,3 And the pain from photophobia can be severe for those who must endure it:
“I have photophobia, it’s difficult if not impossible to live with. If I get prolonged exposure to light, i.e. natural light or room light, or night driving light etc, I get a type of panic attack, I feel desperate to close my eyes and when it’s severe I’m equally desperate to shut my burning eyes and can sleep soundly for several hours.”
Secondary Conditions Can Also Lead to Light Sensitivity
Many of those with lupus also have secondary or comorbid disorders which may make them more prone to ocular light sensitivity or photophobia.
Dry eyes are the most frequent ocular symptom of lupus, and it often manifests as a result of comorbid Sjogren’s Syndrome, an immune system disorder.4,5 Whether it is part of Sjogren’s Syndrome or not, dry eyes are the leading cause of light sensitivity among patients. Read more about dry eye and light sensitivity here.
*Migraine and Headache Disorders
Migraine and other recurring headaches commonly affect those with lupus, with estimates anywhere between 40% to 75% of patients experiencing these neurological effects.1,6 There is still question over whether a “lupus headache” differs from a migraine or any other primary headache as evidence shows they may have unique presentations. Regardless, we know that the presence of regular migraine attacks or headaches comes with an inherent risk for photophobia.
Scleritis is a condition of the eye in which the whites of the eyes become inflamed and inflamed. Nearly one percent of those with lupus will experience scleritis which can lead to symptoms of light sensitivity. Less common are issues like conjunctivitis (pink eye) and iritis (pupil inflammation), but they have been reported by those with lupus.
*Anxiety and mood disorders
Research suggests that a high percentage of lupus patients (particularly women) have self-reported anxiety, mood or panic disorders—all of which have also been linked to light sensitivity.7 In addition, the mere presence of a chronic disorder like lupus, especially if accompanied by comorbid migraine, is likely to bring on anxiety and other emotional problems.
Light Sensitivity and Lupus Medications
Certain medications used to treat lupus may also exacerbate light sensitivity, such as plaquenlil (also known as hydroxychloroquine).2 Oftentimes, these are rare side effects and should not deter you from appropriate treatment, but it is important to be aware of what might result from their usage.
How Photosensitivity Differs for Lupus
It is important to distinguish photophobia from photosensitivity, the latter being one of the hallmark symptoms of lupus. Photophobia, as noted above, generally describes an aversion to or avoidance of light and is often used to describe how the eye absorbs and transmits light wavelengths to the brain. Photosensitivity, on the other hand primarily refers to skin sensitivity to light.
In addition to those symptoms of light sensitivity affecting the eyes, nearly two thirds of those with lupus experience these photosensitive reactions to sunlight or other ultraviolet light sources. Lupus photosensitivity may result in skin rashes commonly over the nose and cheeks, hives and/or a flare in joint pain, fever and inflammation. Exposure to indoor compact fluorescent lamps, or CFL’s may trigger photosensitivity in those with lupus due to their high ultraviolet ray emission.8,9
Photophobia vs Photosensitivity
The Ultimate Guide to Photophobia and Light Sensitivity
An Introduction to Sjogren’s Syndrome
How Migraine Attacks Can Be Triggered or Worsened by Light
1Read RW. Clinical mini-review: systemic lupus erythematosus and the eye. Ocul Immunol Inflamm. 2004 Jun;12(2):87-99.
3Palejwala NV, Walia HS, Yeh S. Ocular Manifestations of Systemic Lupus Erythematosus: A Review of the Literature. Autoimmune Diseases. 2012;2012:290898. doi:10.1155/2012/290898.
8Foering, K et al. Characterization of clinical photosensitivity in cutaneous lupus erythematosus. Journal of the American Academy of Dermatology , Volume 69 , Issue 2 , 205 – 213.
9Wysenbeek AJ, Block DA, Fries JF. Prevalence and expression of photosensitivity in systemic lupus erythematosus. Annals of the Rheumatic Diseases. 1989;48(6):461-463.
That is how the disease began in Dolores McCausland of Cape May, N.J. Two years ago, Mrs. McCausland, 75, had a biopsy taken of a sore on her arm. Doctors confirmed scleroderma as well as pulmonary hypertension, a common result of the disease’s affect on the kidneys, she said. She takes a combination of medicines to treat her blood pressure as well as an antihistamine and gabapentin to treat the pain and itching. She is also treated with oral chemotherapy and an immunosuppressant.
Mrs. McCausland said that covering her arms while driving to avoid exposure to sunlight or using her backyard pool was nearly impossible because the sores on her arms make them sensitive to touching.
“If you saw my arms, you would say, ‘Oh, my God, there’s something wrong with her,’ ” Mrs. McCausland said. “You know when you put a blood pressure cuff on your arm? My arms look like they have been squeezed, and it’s forcing the muscle out to my elbow and shoulder. It’s very painful and itchy.”
Covering the entire body, even in the heat, is critical for scleroderma patients because they have greater susceptibility to skin cancer. And since the blood vessels can also become sclerotic, or thickened, full coverage helps protect people from the shock of entering an air-conditioned environment.
“The problem is that in the summer these patients go from dramatic warm to cold, and they can become very symptomatic from that,” said Dr. Chris T. Derk, an assistant professor of medicine in the rheumatology division at Thomas Jefferson University in Philadelphia. “It’s exceedingly rare, but we have people with Raynaud’s of the heart vessels, and they can go into spasms and give you a small heart attack. You have to cover the whole body because if they can’t rewarm the hand, they can break it.”
Mrs. McCausland said she coped by keeping her house temperature warm and wearing Isotoner gloves to the grocery store, even during the summer months.
“People think I’m some kind of nut,” she said. “I have never been sick a day in my life, and I had to come up with this. ”
Research on photosensitivity among people with lupus
Photosensitivity or abnormal light sensitivity is very complex and is a major symptom of lupus. The American College of Rheumatology (ACR) loosely defines photosensitivity as “a skin rash as a result of unusual reaction to sunlight.” Beyond skin rashes that can develop, exposure to the sun can cause those living with lupus to experience increased disease activity with symptoms such as joint pains, weakness, fatigue and fever. Two-thirds of people with lupus have increased sensitivity to ultraviolet rays, either from sunlight or from artificial inside light, such as fluorescent light — or both.
Normally, skin and other cells that are sufficiently damaged die through a process known as programmed cell death, or “apoptosis.” The body then gets rid of the dead cells. But in lupus, apoptosis in the skin seems to occur more often than it should, which may in turn lead to more inflammation and other complications. In addition to worsening of skin lupus lesions, many patients also experience more generalized skin or systemic reactions to light that are not their typical skin lupus.
According to the ACR, photosensitivity in lupus is determined by clinical examination or by patient history of unusual reaction to sunlight. It is important that physicians are able to recognize the varied manifestations of photosensitivity to ensure proper management of lupus.
Now, a new, small study conducted by Victoria P. Werth, MD and colleagues at the University of Pennsylvania sheds new light on photosensitivity among people with lupus. The study, published in the Journal of the American Academy of Dermatology, characterizes frequency and types of abnormal responses to sunlight in patients with different types of lupus, as well as the association of these reactions with systemic lupus. A pilot study to examine different cell types and numbers of these cells in the skin of patients with and without systemic reactions to light was also performed.
While the pathophysiology of photosensitivity is likely to vary depending on the type of reaction(s) experienced by patients, the researchers explain that defining what is meant by photosensitivity is important in evaluating the effectiveness of treatments that may impact this important problem and in further recognizing the symptoms that fulfill criteria for photosensitivity in lupus patients.
The Lupus Foundation of America is dedicated to finding new ways to better understand and treat lupus, and we thank Dr. Werth and her colleagues for their continuous efforts to help solve the cruel mystery of lupus.
More about this study
In the study conducted by Dr. Werth and her colleagues, 83% of lupus patients reported having some form of photosensitivity. Overall, worsening of skin lupus in response to sunlight was reported by 69% of the group. Many people reported early transient skin lupus lesions that occurred after a few hours to a day and then resolve within a week, while others had lesions that occurred early after sun exposure, but persisted for a few weeks to months. About 10% of people noted the worsening of skin lupus in response to sun happens only after a number of days. 60% of patients who felt their skin lupus worsened after sun exposure also had other reactions to the sun. Some patients report a transient skin eruption after sun exposure that was not their typical skin lupus, termed polymorphous light eruption (PMLE)-like reaction. Others had a generalized sense of itching, stinging, or burning of skin that affected their skin, but without a skin eruption. Lastly, 33% of patients had systemic symptoms after sun exposure, including joint pain, weakness, fatigue, or headaches. These different types of reactions can occur sometimes in the same patient.
The researchers found no relationship between race and any photosensitivity presentation. Subjects with generalized cutaneous reactions (PMLE-like and sting/itch) and those who experienced sun-induced systemic symptoms of joint pain, weakness, fatigue or headaches, tended to have more systemic lupus disease activity. The study noted a novel subset of CLE patients who experienced systemic reactions after sun exposure despite not meeting criteria for SLE. A small study of a subset of the photosensitive patients had a greater density of specific types of inflammatory cells in normal skin compared to those without systemic symptoms.
The results are based on self-report by patients using a photosensitivity questionnaire that is currently undergoing further validation. The patients predominantly had cutaneous lupus, with or without systemic lupus. The findings in patients with primarily systemic forms of lupus may be different.
Characterization of clinical photosensitivity in cutaneous lupus erythematosus
Kristen Foering, Aileen Y. Chang, Evan W. Piette, Andrew Cucchiara, Joyce Okawa, Victoria P. Werth.
Journal of the American Academy of Dermatology. 2013 August 1.
Sun Exposure and Lupus
Many people with lupus have sun sensitive skin, also known as photosensitivity. Typical skin rashes seen in lupus include the butterfly rash, which appears over the nose and cheeks, and discoid rashes that appear as disk-shaped, raised, scaly patches on any sun-exposed area of the body. These rashes are triggered by sun exposure in 50 to 75 percent of people with lupus.
“Photosensitivity is a common symptom of systemic lupus erythematosus. Ultraviolet light consists of three bands: UVA, UVB, and UVC. Patients with lupus are most sensitive to UVA and UVB light,” says Amita Bishnoi, MD, a rheumatologist at Henry Ford Hospital in Detroit.
When Sun-Sensitive Skin Is Exposed to Ultraviolet Light
Ultraviolet light is the invisible radiation in sunlight. “Sunlight may trigger a lupus rash and it can also trigger symptoms of joint pain and fatigue,” notes Dr. Bishnoi. Lupus is a disease that goes through periods of quiet and periods of increased disease activity called flares. Many people with lupus experience flares if they get too much sun exposure.
Although experts don’t know the exact cause of lupus, it is believed to be partly genetic and partly due to environmental exposures that stimulate an abnormal and exaggerated immune response. In lupus, your immune system becomes reactive to the normal cells and tissues in your body. That is why it is classified as an autoimmune disease. In addition to triggering a flare of lupus symptoms, exposure to sunlight may also be one of the main environmental causes of lupus.
How Does Ultraviolet Light Stimulate an Autoimmune Response in Lupus?
One of the functions of a normal immune system is to get rid of old, dying, or defective cells. This normal cell death process is called “apoptosis.” Research shows that when your skin is exposed to ultraviolet light it may cause many of your skin cells to die. These dead cells are powerful stimulators of the immune system. In people with lupus, the process of apoptosis is slowed and the pronged presence of these dead skin cells produces the inflammatory response in your skin that causes a lupus rash. A sunburn can cause massive skin cell death, and in someone with lupus it can trigger the immune system enough to cause inflammation not only in the skin but also in the joints, muscles, and internal organs.
How Can You Protect Yourself From Ultraviolet Light Exposure?
“It is best to avoid prolonged sun exposure. Make sure you use sunscreen with SPF (sun protection factor) greater than or equal to 15, and one that blocks both UVA and UVB rays. Wear protective clothing. Use a broad-brimmed hat, long-sleeved shirt, and long pants if you are going to be outside for long periods of time. Also, watch out for photosensitive medications, which can make you even more sensitive to sunlight,” advises Bishnoi.
Here are more tips for protecting sun-sensitive skin:
- Avoid any prolonged sun exposure but be especially careful at mid-day, when ultraviolet light is strongest. Remember that clouds do not filter out all of the ultraviolet rays of the sun.
- Most people apply less sunscreen than they need. To achieve the maximum SPF, you need to apply at least one ounce of sunscreen per application. The most frequently missed areas are the back, the sides of the neck, and around the ears.
- Sunlight is not the only source of ultraviolet light. Fluorescent lights and photocopiers emit some ultraviolet light. Tanning beds are not safe for people with lupus.
- Some antibiotics, like tetracycline, can make you more sensitive to sunlight, so ask your doctor or pharmacist about photosensitivity any time you start a new drug.
- Car and house windows screen out UVB rays but not UVA. You can buy films to coat these windows for UVA protection.
Because most people with lupus are photosensitive and sunlight can trigger symptoms from skin rashes to internal organ damage, protecting yourself from sun exposure is a vital part of lupus management. It’s important to know how ultraviolet light from the sun and other sources may stimulate an autoimmune response. Make sure you use a sunscreen of at least 15 SPF and that you are using enough sunscreen to get complete protection.
Lupus & Sun Exposure: All You Need To Know
Many of us look forward to summer for its sunnier and warmer days. However, for people with lupus, this can be a particularly difficult period, where the sun’s rays can actually trigger and intensify symptoms. Here’s how the sun affects individuals with lupus and some useful tips for patients to stay extra protected under the sun.
First of all, what exactly is lupus? It’s an autoimmune disease where the body’s immune system attacks its own healthy tissues. This can affect different parts of the body, like the skin, joints, and organs. It’s estimated that over 5 million people in the world have some form of the disease.¹ Depending on the person, the seriousness of the disease ranges from mild to life-threatening with different symptoms, like fatigue, joint pain, physical impairment, and organ problems. There’s currently no cure for the disease, though there are medical treatments and preventive measures to help control it. Even now, the exact cause isn’t fully understood. Most researchers believe it’s a combination of genetic and environmental factors, where someone who’s genetically susceptible encounters an environmental trigger that sets off the reaction, also known as a flare. Some of the more commonly known environmental triggers include ultraviolet rays, certain drugs, and infections.
Many people with lupus experience increased sensitivity to sunlight, known as photosensitivity. As many as 75% of patients are photosensitive.² This can cause rashes to appear on the skin. One of the most common amongst patients is the butterfly (malar rash), which appears on the face across the nose and cheeks. Other skin problems include disk-shaped lesions and scaly red circles (Discoid and subacute lupus erythematosus respectively). For some individuals, it can also activate flare-ups of the disease, triggering some of the other symptoms like fatigue and joint pains. In more severe cases, this could also lead to fever and organ inflammation. Given the effect that the sun’s rays can have, lupus patients need to pay special attention to sun protection. First off, it’s important to apply a broad-spectrum sunscreen with an SPF of at least 30. UV protective clothing, a wide-brimmed hat, and sunglasses are also recommended forms of sun protection. To stay protected in the sun, you can also use the free sun safety app, QSun. Available for iOS and Android, the app gives tailored sun safety recommendations, such as how much sunscreen you should apply and the hours that the sun is strongest during the day. If you think you may have lupus, you should seek the attention of a medical professional immediately.
I remember the first time I learned about photosensitivity and how the blazing South Florida sun was affecting my disease state. For more than 20 years of my life, I lived in Fort Lauderdale, where almost every single day is hot and sunny, and often humid. Though my original diagnosing rheumatologist never mentioned sun sensitivity in our appointments, when I heard about it at a local support group, I instantly had answers to so many lingering questions.
Though I was diagnosed at 23, my mother and I can remember me having strange reactions after being out in the sun even as young as 9. It was hard to be the only kid in a group of friends who would break out in a mouthful (and sometimes noseful) of canker sores after spending a few hours at the beach or riding my bike near my home. The sun was almost impossible to get away from in South Florida, and because both my mother and I had never heard about photosensitivity, we thought surely something else was causing my strange symptoms.
In addition to the sores, I would get severe rashes on my body, eczema, and fevers after particularly long bouts outdoors. As I reached 18, I greatly reduced my time at the beach or outside with friends, because even though I wasn’t diagnosed yet, I knew something didn’t feel right after being in the sun. After my diagnosis, and over the past 17 years as my disease state has worsened, photosensitivity became an even larger issue. Even just five to 10 minutes in the direct Florida sun could spiral me into a major flare.
I felt trapped in my home, unable to go outdoors to do errands or see friends until after 7 or 8 p.m. Getting into my car in the summer in Florida, when interior car temperatures would hover around 100 degrees, was an impossible task. These limitations became so overwhelming over the years that my husband and I made the decision to pack up and move north near the Canadian border, leaving friends and family behind.
If photosensitivity is new to you, it basically means a sensitivity to UV light, either from the sun or indoor fluorescent light bulbs. Not all lupus patients will experience photosensitivity, though a recent study on Lupus Foundation of America‘s website conducted by Victoria P. Werth, MD, revealed that 83 percent of lupus patients reported having some form of light sensitivity. It is also considered a major symptom of lupus, and patients can experience fevers, rashes, fatigue, increased joint pain, mouth sores, and increased disease activity after exposure.
Many people have asked if my move was worth it. I tell them, “Absolutely.” Though most of our friends and family members prefer the heat, my husband and I love the cold weather. Near my home in western New York, the sun is not very strong, and I am actually able to venture outdoors for up to an hour in most cases to go for a hike or visit a local farm to pick fruits and vegetables.
I would never have been able to do those things in Florida, especially for an hour. I also feel better in the cold weather and have fewer fevers. In fact, while I had a fever almost every single day in South Florida, I typically have only experienced them one to two days a week now. I still limit my time outdoors in the summer, and only typically hike in the fall, winter, and early spring. In addition to limiting overall time outdoors, I have added a few photosensitivity-related wearable products to my wardrobe.
Here are two of my favorites:
Shade: You may have heard me talk about the Shade sensor in the past, but I bring it up again because it is truly an incredible product that offers great insight into our body’s reaction to the sun and other UV light. The sensor can clip onto any top or jacket you are wearing and connects wirelessly to an app on your phone. During the day, the sensor will measure the amount of UV light you have received. You can also track daily symptoms through the app, and over time, this intuitive device creates a threshold of how much sunlight you can get before experiencing an increase in symptoms. You can learn more about the device here.
Coolibar: Coolibar has quickly become one of my other favorite wearable products in the fight against UV light. From adorable tops and bathing suits to wide-brimmed hats and dresses, their fabrics offer UPF 50+ protection. This is the highest protection rating clothing can have, and it means that only one-fiftieth of the sun’s UV rays are able to reach your skin rather than 50/50. You can find their awesome clothes here.
How do you stay safe in the sun? Tell us about it.
Note: Lupus News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Lupus News Today or its parent company, BioNews Services, and are intended to spark discussion about issues pertaining to lupus.
Bio: Marisa Zeppieri is a journalist, author and former Mrs. New York 2015. Her verticals include health, nutrition, food, recipe creation and food photography. She is also the founder of LupusChick.com, a NY-based nonprofit and FB community that helps patients and families dealing with incurable autoimmune disease. Currently working on her second book, she enjoys traveling with her husband and rescued terrier, Bogey. ×Bio: Marisa Zeppieri is a journalist, author and former Mrs. New York 2015. Her verticals include health, nutrition, food, recipe creation and food photography. She is also the founder of LupusChick.com, a NY-based nonprofit and FB community that helps patients and families dealing with incurable autoimmune disease. Currently working on her second book, she enjoys traveling with her husband and rescued terrier, Bogey.