Lupus Treatment in Ankara, Turkey

Lupus erythematosus is an autoimmune disease with a complex etiology and chronic course, in which the immune system mistakenly perceives the body’s own healthy tissues and organs as a foreign threat and targets them. Examined across a wide spectrum in medical practice, this pathology ranges from cutaneous forms limited only to the skin to Systemic Lupus Erythematosus (SLE), which can affect vital organs such as the kidneys, heart, and lungs. This inflammatory process, commonly known among the public as the “butterfly disease,” is based on the disruption of antibody production triggered by environmental factors in genetically predisposed individuals, causing the body to initiate a defensive battle against its own cells.

Why Does Lupus Disease Occur and What Are the Triggering Factors?

When we examine the origin of this disease, unfortunately it is not possible to find a single culprit. Lupus is an unfortunate intersection of our genetic inheritance and environmental factors. When there is a predisposition to this disease within a person’s genetic codes, the immune system is already on alert. However, for the disease to emerge, a triggering environmental or hormonal factor is usually required:

The fact that the disease is much more common in women than in men clearly reveals the role of hormonal balance in this process. In particular, the estrogen hormone has the ability to activate immune system cells. For this reason, it is not a coincidence that the highest incidence of the disease occurs in women of reproductive age. The period from puberty to menopause is considered the most risky period for lupus.

Ultraviolet (UV) rays play a central role in the pathophysiology of the disease, meaning its mechanism of formation. Sunlight disrupts the structure of the genetic material (DNA) in the nucleus of skin cells. Under normal conditions, the body clears these damaged cells. However, in lupus patients this cleaning mechanism fails. When the cells break apart, nuclear particles that should normally remain hidden inside the cell are scattered outside. The immune system cannot recognize these particles belonging to its own cells and begins producing antibodies to destroy them. This initiates inflammation in the tissue.

The main environmental factors that trigger or worsen the disease include:

• Sunlight
• Artificial UV sources
• Smoking
• Infections
• Severe stress
• Certain medications
• Hormonal changes

What Is Acute Cutaneous Lupus and the Butterfly Rash?

Among skin involvements, perhaps the most well-known and symbolic manifestation of the disease is the Acute Cutaneous Lupus (ACLE) form. This form almost always appears during periods when the systemic disease is active. The redness that appears on the patient’s face and is called the “butterfly rash” (malar rash) due to its shape is a typical symptom.

This rash spreads symmetrically from the bridge of the nose toward both cheeks. Its color may vary from bright red to a purplish tone. It usually becomes prominent immediately after sun exposure or within a few hours. It may be edematous, meaning swollen, and may feel warm when touched.

The most critical feature that distinguishes this rash from other facial redness is that the groove between the sides of the nose and the lips (the nasolabial fold) is not affected by this redness. In other words, while the cheeks are bright red, the line-shaped area beside the nose maintains its normal color. This is a very strong diagnostic clue for us during dermatological examination. These lesions seen in the acute form generally tend to heal without leaving permanent scars; however, if left untreated they may lead to persistent pigment (color) changes.

How Does Subacute Cutaneous Lupus Progress?

Subacute Cutaneous Lupus (SCLE) is the subgroup in which photosensitivity is experienced most intensely. These patients react immediately upon contact with sunlight. Lesions usually concentrate not on the face but on areas where sunlight hits more directly, such as the shoulders, the outer surfaces of the arms, the neck (“V” area), and the upper parts of the back.

Clinically, these rashes may appear in two different patterns. The first type resembles psoriasis in a surprisingly similar way. Red raised lesions with thin white scales on the surface are seen. The second type is the annular (ring-shaped) form. In these lesions the center is paler while the edges are red and active. These ring-shaped rednesses may expand over time or merge to form larger shapes.

The most important characteristic of subacute lupus lesions is that they do not leave depression or deep scars (scars) on the skin while healing. However, since they affect the pigment cells of the skin, they may leave white spots (similar to vitiligo) or brown spots that can remain for a long time in the healed areas. In patients in this group, positivity of certain antibodies (Anti-Ro/SS-A) is very frequently detected. It should also be remembered that some blood pressure medications, antifungal drugs, or stomach-protective medications may trigger this type of lupus.

Does Chronic Cutaneous Lupus and Discoid Lupus Cause Permanent Damage?

The Chronic Cutaneous Lupus (CCLE) group lies at the other end of the disease spectrum and unfortunately represents the form with the highest risk of tissue damage. The most common member of this group is called “Discoid Lupus Erythematosus” (DLE). Discoid lupus usually remains limited only to the skin, and the risk of progressing to systemic organ involvement (kidneys, heart, etc.) is lower than in other forms (around 5–10%). However, the damage it causes on the skin can be quite distressing.

Discoid lupus plaques begin as sharply defined red-purple lesions and over time become covered with thick, adherent scales. When these scales are forcibly removed, small projections resembling a “nail” or “carpet tack” can be seen underneath. This indicates that the hair follicles are clogged.

The disease most commonly affects the following areas:

• Scalp
• Ear auricle
• Face
• Lips

The greatest risk occurs when these lesions settle on the scalp. The inflammation attacking the hair follicles completely destroys the roots. As a result, permanent baldness (cicatricial alopecia) develops in that area, meaning hair will never grow again there. In addition, lesions located on the face or ear may leave thinning, depression (atrophy), and color changes in the skin while healing, leading to cosmetically challenging appearances for the patient. Therefore, early and aggressive treatment in discoid lupus is of vital importance to preserve aesthetic integrity.

What Is Observed in the Body When Systemic Involvement Occurs?

Lupus is not only a skin disease; when it turns into the systemic form (SLE) or is systemic from the beginning, it has the potential to affect almost every organ in the body. Patients may sometimes present only with general complaints without any signs on their skin. Weakness, fatigue, and unexplained fever are the most common complaints we hear during active periods of the disease.

Musculoskeletal involvement is quite common. Patients feel stiffness in their hands, fingers, or knees when they wake up in the morning. Pain and swelling in the joints (arthritis) may occur. Unlike rheumatoid arthritis (inflammatory rheumatism), lupus arthritis usually does not lead to permanent deformities in the joints; pain and swelling regress with treatment.

Other important systemic symptoms include:

• Mouth sores
• Kidney problems
• Chest pain
• Shortness of breath
• Anemia
• Clotting disorders
• Hair loss

Kidney involvement (Lupus Nephritis) is one of the most serious complications of the disease. It may progress silently; the patient may not notice symptoms until swelling (edema) in the legs or darkening of urine color occurs. Therefore, urine tests of lupus patients are regularly monitored. Inflammation of the heart lining (pericarditis) or lung lining (pleuritis) may also cause sharp chest pain.

Which Methods Are Used to Diagnose Lupus?

Diagnosing lupus is like completing a complex puzzle. A single blood test or a single examination finding is not enough to say “You have lupus.” The physician reaches a conclusion by bringing together the patient’s history, physical examination findings, and laboratory results. When we see a dermatologically suspicious rash, the most reliable method we use to confirm the diagnosis is a skin biopsy.

• Histopathological Examination and Biopsy:

Under local anesthesia, a small piece of skin is taken from the most active area of the lesion (punch biopsy). This sample is examined under a microscope in the pathology laboratory. Characteristic findings such as structural deterioration of cells in lupus skin, dense lymphocyte accumulation around blood vessels, and increased amounts of a jelly-like substance called “mucin” in the dermis are investigated.

• Lupus Band Test (LBT):

This is a special immunofluorescence examination method specific to lupus. It checks whether antibodies produced by the body accumulate at the junction line between the upper layer of the skin (epidermis) and the lower layer (dermis). Under the microscope, these accumulations appear as a fluorescent strip or band shining with fluorescent light. The presence of this band largely confirms the diagnosis.

• Serological Blood Tests:

Detection of circulating autoantibodies in the blood forms the backbone of diagnosis. The most commonly requested test is the ANA (Antinuclear Antibody) test. However, ANA positivity alone does not mean lupus; it may also be positive in healthy individuals or other diseases. More specific tests such as Anti-dsDNA (especially elevated in kidney involvement) and Anti-Sm are evaluated to support the diagnosis. Detection of leukopenia (low white blood cells), thrombocytopenia, or anemia in the blood count are also important signs of systemic involvement.

Why Is Sun Protection the Most Critical Part of Treatment?

Regardless of which medication is used in lupus treatment, if the patient is not effectively protected from the sun, the treatment is doomed to fail. This is because UV rays act like the molecular “on-off switch” of the disease. Exposure to sunlight kills skin cells, causing antigens to be released and prompting the immune system to attack again and again.

The protection strategy does not simply mean “applying cream at the beach in summer.” For these patients, sun protection must become a year-round lifestyle. Even on cloudy days, most UV rays still reach the earth.

Recommended methods for effective protection include:

• Using sunscreen
• Wide-brimmed hats
• UV-protective clothing
• Sunglasses
• Preferring shade
• Window films

The sunscreen used must protect against both UVA and UVB rays, and the protection factor (SPF) should be at least 30, ideally 50 or higher. The cream should be applied 20 minutes before going outside and renewed every 2–3 hours when staying outdoors. It should not be forgotten that car and house windows block UVB but allow UVA, which causes deeper tissue damage; therefore protection is necessary even inside a car during long journeys.

What Are Topical and Local Treatment Options?

In cases where the disease is limited only to the skin or when skin findings accompany systemic disease, first-line treatment consists of agents applied directly to the skin. The goal here is to extinguish the inflammation locally and allow the skin to heal.

The most commonly used agents are topical corticosteroids (cortisone creams). Creams with different strengths are selected according to the body area and the thickness of the skin. For example, strong steroids are preferred for thick-skinned areas such as the back or scalp, while weaker ones are used for the face or fold areas. The reason for this is that long-term use of strong steroids on the face may thin the skin and cause capillary vessel rupture.

When we want to avoid steroid side effects, creams containing “calcineurin inhibitors” (tacrolimus or pimecrolimus) come into play, especially in sensitive areas such as the face and around the eyes. These creams do not contain cortisone and do not thin the skin with long-term use.

In very thickened discoid lupus plaques that resist cream treatment, the “intralesional injection” method is applied. In this method, a steroid medication called triamcinolone acetonide is injected directly into the lesion with a very fine needle. This allows the medication to reach the exact target and accelerates healing.

What Is the Role of Systemic Drug Treatments and Antimalarials?

When topical treatments are insufficient or to reduce the risk of systemic involvement, oral medications are introduced. The cornerstone considered indispensable in lupus treatment is antimalarial drugs (especially Hydroxychloroquine). Our patients sometimes ask, “I do not have malaria, why am I using this medication?” Although these drugs were originally developed for malaria, their immune system regulating properties were discovered over time.

Hydroxychloroquine controls both skin and joint manifestations of lupus. More importantly, it has been proven that when used regularly it prevents organ damage, reduces the frequency of disease flare-ups, and decreases the risk of clotting. This medication is generally safe; however, annual eye examinations are required due to the possibility of accumulation in the retina layer of the eye with long-term use.

During severe flare-ups of the disease, systemic corticosteroids (cortisone tablets or injections) are used to rapidly suppress inflammation. The dose is adjusted according to the severity of the disease, and once the disease is controlled, it is gradually reduced and discontinued. Care should be taken because long-term high-dose cortisone use may lead to side effects such as osteoporosis, weight gain, and diabetes.

Other drug groups used in resistant cases include:

• Methotrexate
• Azathioprine
• Mycophenolate Mofetil
• Cyclophosphamide
• Belimumab
• Rituximab

These medications suppress the immune system more strongly (immunosuppressants). They are generally preferred in patients with kidney involvement who do not respond to antimalarials or who require very high doses of cortisone.

What Is Pregnancy Planning and Neonatal Lupus?

It is certainly possible for a woman with lupus to become a mother, but this process should be considered within the category of “high-risk pregnancy” and must be planned very carefully. An unplanned pregnancy may create serious risks for both the mother and the baby. The ideal scenario is becoming pregnant during a period when the disease has been in remission, meaning inactive, for at least 6 months. Active disease increases the risk of miscarriage, premature birth, or preeclampsia (pregnancy poisoning).

Medication use during pregnancy requires great sensitivity. Some drugs such as Methotrexate or Mycophenolate may cause serious birth defects in the baby and therefore must be discontinued months before pregnancy planning. On the other hand, Hydroxychloroquine is considered safe during pregnancy and its continued use is generally recommended. Stopping the medication may cause disease flare-ups during pregnancy, which can be much more dangerous for the baby than the medication itself.

A special condition called “Neonatal Lupus” is a rare condition that may be seen in babies of mothers with SLE. It occurs when Anti-Ro and Anti-La antibodies in the mother pass to the baby through the placenta. The baby may have ring-shaped rashes on the face at birth or elevated liver enzymes. This condition is usually temporary; as the antibodies passed from the mother are cleared from the baby’s body (approximately 6–8 months), the symptoms disappear. However, these antibodies may rarely cause permanent “heart block” in the baby’s heart, so the baby’s heartbeat must be closely monitored throughout pregnancy.

Which Other Dermatological Conditions Can the Disease Be Confused With?

Lupus lesions are so diverse that dermatologists must consider many diseases in differential diagnosis. Misdiagnosis may lead to incorrect treatment and loss of time.

The most common confusion occurs with “Rosacea,” also known as rose disease. Both diseases involve facial redness and sensitivity to sunlight. However, in Rosacea acne-like papules and pustules are seen, whereas in lupus there are no comedones (blackheads). In addition, sudden flushing attacks called “flushing” are prominent in rosacea patients, and lesions heal without leaving scars.

Other diseases that may be confused include:

• Psoriasis
• Dermatomyositis
• Lichen planus
• Drug eruptions
• Polymorphic light eruption
• Seborrheic dermatitis

Dermatomyositis in particular creates a facial rash very similar to lupus. However, in dermatomyositis there is a purplish discoloration on the eyelids (heliotrope rash) and rashes over the finger joints (Gottron papules). Muscle weakness is also more pronounced. A biopsy and specific antibody tests are required for a definitive distinction.

How Should Long-Term Follow-Up and Quality of Life Be?

Receiving a lupus diagnosis is not an end but the beginning of a new lifestyle. Although the disease is chronic, with modern treatments most patients can live normal and active lives. In long-term follow-up, attention is given not only to disease activity but also to treatment side effects and other health problems that may accompany it.

For example, long-standing chronic inflammation may accelerate atherosclerosis (hardening of the arteries). Therefore, lupus patients should pay extra attention to cardiovascular health and maintain balanced blood pressure and cholesterol levels. Smoking should definitely be stopped, as it multiplies this risk and undermines treatment.

To improve quality of life, patients should pay attention to the following:

• Regular exercise
• Balanced nutrition
• Vitamin D supplementation
• Stress management
• Psychological support
• Protection from infections

Living with a chronic disease can be psychologically exhausting. The visibility of skin lesions may cause patients to experience social isolation or depression. Addressing this as part of the treatment and seeking professional support when necessary is very important.