Your Ultimate Guide to Lupus: Symptoms, Management, and Skincare

As she neared age 30, Lynn Battle began to find herself fatigued. Then, she developed an unusual rash; her joints became swollen and painful, and her body ached all over.

Battle thought she might have the flu. The “flu” persisted, though, and the symptoms grew increasingly worse. “I had just turned thirty, and everything became very hard,” she says. 

Her role as bursar at the University of San Francisco–a demanding, often stressful job–gave Battle a sense of purpose and identity. As her symptoms lingered, though, she began to suspect that job-related stress might be aggravating them. 

Battle resigned and took a less stressful position, hoping to find relief from the debilitating pain and malaise. “Then,” she says, “my energy level plummeted. I was so tired I couldn’t eat, could barely walk.” 

“Just how 30 felt” 

Battle’s symptoms soon became so limiting that they could not be ignored. “I went to the doctor thinking it was just how 30 felt.”

Her doctor told her that these symptoms were not normal and ordered tests. Battle was diagnosed with lupus as a result.

The disease began to affect Battle’s organs. Treatment could not stave off the inflammation; her organs began to shut down. Her doctors told her she had less than six months to live. 

Faced with this sobering prognosis, Battle moved from California to Birmingham, Alabama, her birth place, to be near family. “I came home to die,” she says bluntly.

“I waited around to die…”

Battle eliminated every source of stress that she could. “I waited around to die for the first year,” she says. 

After that first year, she became philosophical. “I started doing the things I wanted to do, living,” Battle explains. “I figured death would find me when it wanted to and there was no reason to sit at home and wait on it.” 

Since her diagnosis, Battle has had four hip replacements. She has had several episodes of paralysis caused by  transverse myelitis (TM), a rare and potentially deadly inflammation of the spine. She once lost her vision completely after lupus took aim at her optic nerve.

Lupus: What it is 

 

“Lupus” is a common lay term for lupus erythematosus, a group of chronic, inflammatory diseases that often feature significant skin involvement. 

Lynn Battle was diagnosed with systemic lupus erythematosus (SLE), the most common type of lupus. This type of lupus  affects multiple systems within the body, including the skin. 

It is estimated that 72.8 people among every 100,000 people worldwide suffer from SLE. Women are nine times more likely than men to be diagnosed with SLE. It disproportionately affects black, Hispanic, and Native American women, and the disease is usually more severe in these populations. Patients are usually diagnosed in their 20s or 30s. 

Cutaneous lupus erythematosus (CLE) is a category of lupus defined by skin involvement and generally includes all skin manifestations of lupus. Some doctors use it to mean skin activity without significant Systemic or internal Lupus or independent of SLE. The most common form of CLE is discoid lupus (DLE). Another version is subacute cutaneous lupus erythematosus (SCLE) which often looks like psoriasis or even ringworm (tinea corporis). 

DLE and SCLE tend to be chronic and recurring. With SLE,  the initial rash is usually gone after treating the SLE internal manifestations. Abnormal labwork  is more likely with SCLE than DLE. Most cases of SCLE and almost all DLE patients have a negative double-stranded DNA antibody test. 

Discoid lupus affects between 2 and 5 of every 10,000 people. As with SLE, it predominantly affects women. Most cases of DLE are diagnosed between the ages of 20 and 40. 

 

Immune System Dysregulation

Lupus erythematosus is a chronic, incurable autoimmune disorder. It arises from dysregulation of the adaptive immune system and an inability of some immune cells to distinguish the body’s own cells from foreign ones. With systemic lupus, there are one or more clones of harmful B-cells producing antibodies that attack the victim’s tissues.  Recently, experimental CAR-T therapies have shown promise for long term eradication of lupus manifestations by specifically destroying those cells.  

Antigens are substances that are capable of eliciting an immune response. Many come from outside of the body; some, though, are native to the body’s cells. These self-antigens, or autoantigens, are part of what distinguishes the cells of one system or organ from another. They are tolerated by the body when the immune system is functioning properly. 

 

Housekeeping Gone Awry

 

When cells in your body are damaged–by a virus, for example–they go through a process called apoptosis, or programmed cell death. This is a form of “housekeeping” necessary for the collective health of a system. During this process, the apoptotic cells release self-antigens. Under normal conditions, the immune system “cleans up” the products of apoptosis with no problem.

When the immune system is dysregulated, as occurs with lupus, apoptosis triggers the generation of antibodies against self-antigens. These antibodies bind with self-antigens in healthy cells as they would with foreign antigens and damage them. The damaged cells release proinflammatory molecules, which cause even more cellular damage. Entire organ systems are compromised. . 

Causes

 

The causes of this immune system dysregulation are not clearly understood. Evidence suggests that it is a multifactorial disorder arising from a combination of genetic predisposition, environment, and immune system function. At this time, genetic testing cannot diagnose lupus, nor rule it out. 

 

Heredity

 

Genome-wide association studies (GWAS) on both CLE and SLE patients have uncovered an association between lupus and mutations on different genes related to apoptosis. This is true of both SLE and CLE, although the specific genes seem to differ between the two types of lupus.

Environment

Inheriting these mutations does not guarantee the development of lupus. Coming into contact with certain exogenous antigens, though, can trigger the development of the disease. Lynn Battle feels that exposure to air pollution played a significant role in her disease.

“My doctor was not surprised when he realized where I was from,” she says. She points out that in the 1960s and 1970s, Birmingham was a thriving steel town with the air pollution typical of the industry in that period; it also had one of the highest rates of lupus in the United States. 

Research supports Battle’s hypothesis. According to studies, people exposed to high levels of particulate matter are at increased risk of developing lupus. Higher ozone levels are associated with an increased risk of hospitalization among lupus sufferers. 

Ultraviolet radiation, both UVA and UVB, is another significant environmental trigger. Exposure to sunlight, tanning beds, and other sources of UV radiation frequently set off lupus flares, and they can also worsen existing flares. 

Immune System Factors

 

Immune system dysregulation plays a prominent role in the development and severity of lupus symptoms. Severe or prolonged stress is associated with immune system dysregulation and chronic inflammation.   

“My initial lupus doctor told me that lupus was a monster that fed on stress!” Battle tells us. 

Many researchers now believe that viruses can also trigger the development of lupus. Links have been found between lupus and Cytomegalovirus, Epstein-Barr (which causes mononucleosis), and varicella-zoster virus (chickenpox and shingles virus).  

Treatment for SLE also affects the immune system. Severe cases of lupus involving the organs require immunosuppression therapies. This leaves lupus patients vulnerable to infections or cancers which are sometimes fatal. 

Symptoms of SLE

“Lupus” takes its name from a distinctive rash that often spreads across the nose and the cheeks of lupus patients called a malar rash; a 13th Century physician compared it to the mask-like coloration of a wolf’s face. 

The malar rash is often referred to as a “butterfly rash.” It is flat, red, and itchy. It often worsens with exposure to sunlight or other sources of ultraviolet (UV) radiation. This rash can occur elsewhere on the body. In addition to these flat, itchy rashes, lupus patients can develop bullous lesions that mimic a blistering skin problem.  

Although the butterfly rash is not a universal symptom of SLE, the skin is the second-most common organ affected by SLE (the kidney is the first). Somewhere between 70% and 85% of lupus sufferers experience skin involvement. Skin complaints are often the first symptoms patients notice. 

Other lupus-related skin problems include: 

• Swelling of the eyelids
• Sensitivity to sunlight and other sources of UV light
• Raynaud syndrome–pain, tingling, and white or blue skin of the fingertips, toes, and sometimes nose that occur in response to cold air or stress 
• Splitting of the nails/swelling around nail beds
• Hair loss
• Purpura–red or purple spots of discoloration caused by leaking blood vessels. Small spots are called petechiae. Larger, bruise-like spots are called ecchymoses.
• Blisters on the gums, tongue, and inside of the mouth. 

A rare subtype of systemic lupus erythematosus called bullous systemic lupus erythematosus (BSLE) affects about 1% of lupus patients. It manifests primarily as mainly as large, fluid-filled blisters affecting the genitals and the inside of the mouth.

While it is rare, many lupus patients have no skin involvement at all. As exemplified by Lynn Battle’s experience, lupus often affects multiple systems. Symptoms can include: 

• Unexplained fevers
• Fatigue/malaise
• Joint pain and swelling
• Chest pain and/or shortness of breath
• “Brain fog” and memory loss
• Protein in urine 
• Swelling (edema) of hands and ankles  

Symptoms of Discoid Lupus

Discoid lupus erythematosus (DLE) is named for the round or oval red areas of skin that characterize the disease. It causes patches of red, scaly, slightly raised skin, and there are often geometric and rhomboid shaped areas too. There’s often a thickness and spiny roughness to the “plaques” of DLE.  Discoid lupus most commonly affects the face, ears, scalp, neck, and hands.  

Skin thickening can occur, especially if treatment is delayed; patches of thinning skin (atrophy) may be observed, as well. Discoid rashes on the scalp can result in hair loss with smooth white scars, if not treated promptly. Strong topical steroids should be used on these rashes every day without interruption until plaques are thin and fading.

Most lesions of DLE are undertreated. Dermatologists are very aggressive with topical steroids for well established DLE plaques.  

A burning sensation or mild itching in the skin often precedes the development of lesions; the skin might begin to feel hot as it reddens and swells slightly. Severe itching is far more likely to be atopic dermatitis or contact dermatitis. 

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Discoid lupus can occur on its own or as a symptom of SLE; about 1%-5% of those diagnosed with discoid lupus are later diagnosed with SLE.  In general, however, with a dermatologist’s care and sun avoidance, the DLE patient does not turn into an SLE patient . It’s also observed that most patients who present with SLE never develop significant DLE skin lesions. Scalp hair loss can be a problem with SLE.  

Diagnosis of SLE

Systemic lupus erythematosus  is notoriously difficult to diagnose, as it often mimics other illnesses; 46.5% of lupus patients report being diagnosed with other illnesses years before their doctors recognized they have lupus.

Lupus is frequently mistaken for rheumatoid arthritis, fibromyalgia, chronic fatigue, and dermatitis–symptoms are sometimes even misattributed to anxiety or depression. A drug rash and drug induced lupus must be sorted out by an experienced dermatologist. Skin infections and rashes from underlying infection like lung Mycobacteria, Mycoplasma, and Histoplasmosis must be ruled out. 

There is no single test to diagnose SLE. Antinuclear antibodies (ANA) testing is a good screening test for lupus, but many people with mildly elevated ANA never develop lupus. At the same time, many people with a positive ANA have no clinical signs of lupus; doctors caution these people to be vigilant with sun exposure.

 

Diagnosis depends upon a thorough patient history, physical exam, lab work. Because SLE remits and relapses (and often strikes a different body system with each relapse), diagnosis can take years. Lupus can share many lab abnormalities with scleroderma, dermatomyositis, rheumatoid arthritis, and what’s called mixed connective tissue disease. 

 

 

Diagnosis of DLE

 

Discoid lupus and other types of CLE may initially be mistaken for inflammatory skin disorders such as eczema, psoriasis, lichen planus, vitiligo, or cutaneous infections. Typical-looking DLE rashes should have a diagnostic skin biopsy on their first consultation with a dermatology provider.  

Rarely, lupus presents as a localized depressed scar or divot in the skin, which may even look like a tumor. Patients are sometimes misdiagnosed with allergic contact dermatitis or skin infections. The first lesion of DLE can look like a thin basal or squamous cell skin cancer, requiring a biopsy for differentiation.

A skin biopsy is usually essential and ordered by a rheumatologist or dermatologist who suspects DLE. They will look for areas of hyperkeratosis (thickening) of the outermost layer of skin, in addition to plugs of keratin around the hair follicles. They will also look for signs of inflammation and lymphocytes, both superficial and deep in the dermis, associated with hair follicles and sweat glands. Lichen planus, another common skin problem, can mimic lupus both clinically and with its biopsy.

Blood tests may be ordered to look for antinuclear antibodies (ANAs), which occurs in about 20% of new DLE cases. Many patients with DLE never develop abnormal lab work. For them, it’s purely a sun induced, autoimmune phenomenon,localized to the skin, between their skin and specific cells from the bone marrow. 

Complications

 

Complications of SLE can be life threatening. Kidney failure, inflammation around the heart (pericarditis), brain inflammation, and stroke are just a few of the risks SLE patients face. 

The most common complications of DLE include scarring and patchy hair loss, skin atrophic scars, skin hyper-or hypopigmentation.  African American patients often have disfiguring color loss early on in the disease that mimics vitiligo.  Untreated discoid lesions may also increase the risk of skin cancer. Discoid sores within the mouth can increase the risk of squamous cell carcinoma.

 

Management

 

Treatment of SLE often involves several different types of therapy. This can include immunosuppressants such as methotrexate, dapsone, cyclophosphamide and azathioprine, usually while following with both dermatology and rheumatology.  

Antimalarials such hydroxychloroquine are also frequently used. Hydroxychloroquine is well tolerated and safe for most people, unless they are taking multiple medications that prolong the QT interval of cardiac rhythm.  An oral corticosteroid (Prednisone) may be prescribed temporarily as adjunctive therapy to relieve inflammation. A course of oral Prednisone is often prescribed for painful sores in the mouth associated with lupus.

In 2021, the FDA approved a biologic treatment called Anifrolumab for adult lupus patients with moderate to severe symptoms.  More and more often, rheumatologists and hematologists are turning to the monoclonal antibody Rituximab to treat early, severe SLE; for six months or longer, it knocks out the B-cells that produce harmful antibodies . 

Dr. Harlan advises patients who don’t respond to these therapies to seek out a medical center offering CAR-T therapy. This is for the most intractable cases of Lupus.   

 

 

Treatment of Skin Involvement With SLE 

 

Malar rashes or rashes on the body may be treated with calcineurin inhibitors such as pimecrolimus. Topical steroids are another common treatment for SLE-associated rashes; when a rash lingers, doctors may have patients alternate between the two to prevent topical steroid withdrawal (TSW) or skin atrophy, which are common side effects of long term topical steroid use. If topical treatments are not effective, hydroxychloroquine or another antimalarial may be prescribed in conjunction with topical treatments. 

When his adult lupus patients present with red smooth rash without thickening, Dr. Harlan usually has them apply a low potency corticosteroid such as SmartLotion® three to four times daily for up to two or three weeks and then taper to once daily as needed. This allows them to get inflammation and discomfort under control without the risks of high potency steroids. 

For bullous SLE, a low dose (25-50 milligrams daily) of dapsone is often an early treatment of choice, in addition to prescription topical steroids. Patients may see marked improvement within days; new blisters stop forming and existing blisters begin to heal rapidly. If improvement is not seen, a higher dose of dapsone may be prescribed. 

Topical steroids or topical calcineurin inhibitors are usually the first-line treatments for DLE. No other medications have been approved specifically for the treatment of DLE. Treatments such as hydroxychloroquine are often prescribed by dermatologists off-label for stubborn lesions. 

Typical initial treatment for a DLE flare involves topical application of a high potency topical steroid. Lesions normally begin to improve within two weeks of starting therapy if strict sun protection is adhered to. Treatment is tapered when lesions are no longer red and scaling. Switching to a low potency topical steroid with excellent safety like SmartLotion® is often recommended by board-certified dermatologist Dr. Steve Harlan and his network of dermatologists who recommend it. 

Once a flare has been resolved, low potency topical corticosteroids (like hydrocortisone butyrate), topical calcineurin inhibitors, and sometimes retinoids like Tretinoin are used to maintain remission. SmartLotion® eczema cream works well in these instances, because it can be used long-term for involved facial areas with little risk when used under a physician’s care for preventing lupus flares.

Self-Care for Lupus

Lupus patients must be mindful of their triggers and practice a high level of self-care. This means avoiding exposure to sunlight and other sources of UV radiation–even fluorescent lights can trigger a flare or aggravate existing symptoms. 

Sunscreen should be applied daily, and religiously. Doctors often recommend an SPF of 70 used in combination with a tinted product; this is helpful for both women and men. 

Sunscreen is not a substitute for other protective measures. It should be used in conjunction with light-blocking clothing. Tanning beds should be avoided at all costs.  Dr. Harlan warns lupus patients to guard against sunlight directly reaching any unprotected skin, and to take 2,000 units of vitamin D daily. 

Stress management is also a critical piece of the lupus management puzzle. “[My doctor] said if it couldn’t stop the sun from coming up tomorrow it wasn’t worth stressing over and feeding the monster,” Lynn Battle tells us. “So that is what I live by.” 

Connection–A Crucial Part of Healing

Lupus has exacted heavy tolls from Lynn Battle–but she has now lived with the disease for 32 years. Within that period, she became a mother and raised a daughter on her own. She is an accomplished artisan who has mastered handicrafts such as glass making, weaving, and hand-dyeing. She still struggles during SLE flares, and while symptoms are sometimes reversible (she has now regained most of her vision), they often are not. 

Community has been a vital part of Battle’s fight against lupus. She appreciates her friends and finds purpose in reaching out to others at every opportunity. 

“It’s funny,” Battle says. “I moved back [home] because of family, but it has been friends that have kept me afloat at every turn. That’s why I always try to help when I can.” 

 

 

  Cee Van

  Medical Writer

  HarlanMD.com