Lupus is a complex autoimmune disease where the body’s immune system mistakenly attacks its tissues. Systemic lupus erythematosus (SLE) affects multiple organs. Cutaneous lupus erythematosus (CLE) 100% confines to the skin. Despite being less discussed, CLE significantly impacts patients’ quality of life, often serving as the first sign of lupus.
What Is Cutaneous Lupus?
Cutaneous lupus erythematosus (CLE) encompasses a group of autoimmune skin disorders that can occur with or without systemic involvement. It features various skin lesions that are chronic or intermittent. The primary types of CLE include:
- Acute Cutaneous Lupus Erythematosus (ACLE): Often presents as a butterfly-shaped rash across the cheeks and nose, known as the “malar rash.
- Subacute Cutaneous Lupus Erythematosus (SCLE): Features red, scaly lesions on sun-exposed areas, typically without scarring.
- Chronic Cutaneous Lupus Erythematosus (CCLE): Includes discoid lupus erythematosus (DLE), which is the most common form, along with lupus erythematosus tumidus and lupus profundus.
Who Is Affected?
CLE has an annual incidence of approximately 4 to 4.3 cases per 100,000 people, with a prevalence of 73 cases per 100,000. It predominantly affects women, particularly those aged 20 to 50. Ethnicity plays a significant role, with individuals of African, Asian, and Hispanic descent being at higher risk. Besides, up to 75% of patients with SLE develop cutaneous manifestations, and in 25% of these cases, skin symptoms are the first indication of the disease.
Types of Cutaneous Lupus
- Acute Cutaneous Lupus Erythematosus (ACLE)
ACLE resembles the classic butterfly-shaped rash across the cheeks and nose. This rash often occurs due to sun exposure and links to systemic lupus erythematosus.
- Subacute Cutaneous Lupus Erythematosus (SCLE)
SCLE presents as red, scaly lesions on sun-exposed areas, such as the chest, back, and arms. These lesions typically do not scar and may resolve without treatment. SCLE is strongly associated with sun exposure and certain medications.
- Discoid Lupus Erythematosus (DLE)
DLE is the most common form of CCLE, accounting for 80% of cases. It presents as round, red, scaly patches that can lead to scarring and hair loss. While DLE primarily affects the skin, approximately 1% to 5% of patients may develop systemic lupus erythematosus over time.
- Lupus Erythematosus Tumidus (LET)
LET is a rare form of CLE characterised by red, raised, non-scarring lesions. These lesions often appear on the upper arms and shoulders and are typically asymptomatic.
- Lupus Profundus
Also known as lupus panniculitis, this form affects the deeper layers of the skin, leading to firm, tender nodules that can result in scarring and tissue loss.
- Chilblain Lupus Erythematosus
This rare form presents as red or purple lesions on the fingers and toes, often triggered by cold exposure.
Causes and Risk Factors
The exact cause of CLE is not fully understood, but several factors contribute to its development:
- Genetics: A Family history of lupus increases the risk. For instance, individuals with a first-degree relative (parent or sibling) diagnosed with lupus have a substantially higher risk of developing the disease themselves. Studies indicate that siblings of lupus patients have an 8 to 20 times higher risk compared to the general population.
- Sun Exposure: Ultraviolet (UV) light can trigger or worsen skin lesions.
- Medications: Certain drugs, such as hydralazine and procainamide, can induce lupus-like symptoms.
- Smoking: Cigarette smoking is more common in patients with CLE and is associated with increased disease activity and damage.
- Hormones: Estrogen may play a role, explaining the higher prevalence in women.
Diagnosis
Diagnosing CLE involves a combination of:
- Clinical Examination: Assessment of skin lesions and medical history.
- Laboratory Tests: Blood tests to check for autoantibodies, such as antinuclear antibodies (ANA).
- Skin Biopsy: A small sample of skin tissue is examined under a microscope to confirm the diagnosis.
Treatment Options
While there’s no cure for CLE, various treatments can help manage symptoms:
Topical Treatments
- Corticosteroids: Applied directly to the skin to reduce inflammation.
- Calcineurin Inhibitors: Tacrolimus and pimecrolimus creams can be used for localised lesions.
Systemic Treatments
- Antimalarials: Hydroxychloroquine and chloroquine are commonly used to control skin lesions.
- Immunosuppressants: Medications like methotrexate and azathioprine may be prescribed for severe cases.
- Biologics: Belimumab and rituximab are newer options for refractory cases.
- Smoking Cessation: Quitting smoking can improve treatment outcomes and reduce disease activity.
Lifestyle Modifications
- Sun Protection: Regular use of broad-spectrum sunscreens and protective clothing.
- Diet and Supplements:
Personal Stories – Living with Cutaneous Lupus
Symptom Management After Trial And Error
A Reddit User was diagnosed with skin lupus and has faced significant lifestyle changes to manage the condition. Even brief sun exposure triggers ringworm-like spots on their skin, prompting them to adopt strict protective measures. They now rely on mineral-based SPF, wear UPF-rated clothing, and never leave home without a wide-brim hat to minimise UV exposure.
Since beginning a daily 200 mg dose of hydroxychloroquine, their symptoms have improved—flares still occur, but with much milder intensity. Initially, a twice-daily dose caused severe mental side effects, leading to a reduction in frequency. Despite ongoing fatigue and sensitivity to heat, often resulting in panic attack–like symptoms, they’ve made steady progress.
Sun Protection Is A Saviour
A 31-year-old Reddit User is currently undergoing the lupus diagnosis process and shares that it’s been mentally exhausting. She’s long practised strict sun protection—daily use of SPF products like Trader Joe’s facial sunscreen, Supergoop Unseen, and It Cosmetics CC cream for her face, along with Supergoop Play and reapplication tools like mist and powder sunscreens. While it may seem excessive, she’s determined to protect her skin, especially her face. With these precautions, she can tolerate up to two hours in the sun without major symptoms, but any longer leads to severe fatigue and feeling unwell for a full day.
Dr. Greiling Explains Cutaneous Lupus
You can also check out this highly informative video by Dr. Greiling on Cutaneous Lupus.
Living with Cutaneous Lupus
Cutaneous lupus may not affect internal organs like systemic lupus does, but its impact on daily life, self-esteem, and overall well-being can be just as profound. From visible skin lesions and chronic discomfort to the emotional toll of managing a lifelong autoimmune condition, CLE is far more than “just a skin issue.”
The good news? With growing awareness, improved diagnostic tools, and more targeted treatment options, people living with cutaneous lupus today have better chances than ever to manage their symptoms and lead full, active lives. Early diagnosis, consistent sun protection, and the right treatment plan can dramatically reduce flare-ups and improve quality of life.
If you or someone you know is struggling with persistent skin issues—especially after sun exposure—don’t ignore the signs. A dermatologist or rheumatologist can help identify whether lupus may be at the root. And remember: while there’s no cure yet, support, knowledge, and proactive care go a long way in living well with cutaneous lupus.
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