Cutaneous Lupus Erythematosus: More Than Just a Skin Condition

Introduction:

Cutaneous lupus is an autoimmune condition that damages the skin when the immune system unintentionally targets healthy skin cells. This causes redness, itchiness, and pain, and eventually, it results in skin scarring.

Skin lupus can develop on its own or as a symptom of systemic lupus erythematosus (SLE), the most severe form of the disease affecting your joints, kidneys, heart, lungs, blood vessels, and brain.

Your body becomes severely inflamed as a result of lupus. When you have cutaneous lupus, your skin is impacted by inflammation. A butterfly rash spreads across the face in most people with cutaneous lupus. This condition has no known treatment. Many Clinical Research Organizations are researching potential cutaneous lupus treatment options that may lessen symptom flare-ups’ occurrence and severity. 

What are the Different Types of Cutaneous Lupus Erythematosus?

Cutaneous Lupus is classified as:

• Acute cutaneous lupus erythematosus (ACLE): This frequently occurs as a sign of systemic lupus erythematosus. The malar rash, also known as the butterfly rash, spreads across the nasal bridge from the cheeks. Approximately half of SLE patients develop this rash after being exposed to ultraviolet light (sun). This condition may be difficult to distinguish from others, such as rosacea. 

The nasolabial fold (the area on the face that runs from the corners of the upper lip to the nose) is one distinguishing feature of malar rash, whereas rosacea does. The malar rash can appear months or years before lupus symptoms appear.

• Subacute cutaneous lupus erythematosus (SCLE): This manifests as sores on sun-exposed areas of the body like the upper trunk and arms. SCLE lesions are characterized by scaly red annular (ring-like) redness and central clearing. They may also be polycyclic, with the appearance of multiple rings interacting. Another distinguishing feature of SCLE is that it is frequently found in sun-exposed areas.

• Discoid lupus erythematosus (DLE): This form of the disease results in a persistent red rash with scar-like centers that are frequently surrounded by darker hyperpigmentation, primarily affecting the ears and scalp. These lesions can cause permanent hair loss if the DLE is long-standing and hair follicles are lost. 

DLE can affect the face as well, but it rarely affects areas below the neck. DLE can affect up to 25% of SLE patients and accounts for approximately 75% of all cases of cutaneous lupus. To avoid scarring, prompt treatment is required.

What causes Cutaneous Lupus erythematosus?

Multiple factors contribute to the pathogenesis of cutaneous lupus erythematosus.

Genetic predisposition

• High prevalence within the family

Environmental factors

• Cigarette smoking
• Sun exposure
• Medications

Immune systems 

• Autoantibodies

How does a Lupus Rash Appear?

A lupus rash might look like this:

• A butterfly or scaly rash: An itchy, scaly rash in the shape of a butterfly that covers your cheeks and the bridge of your nose, Although this rash won’t leave any scars behind, you might notice some skin discoloration like dark or light spots.
• Red, ring-shaped lesion: Lesions that are red, ring-shaped, and neither itchy nor scarring. Your arms, shoulders, neck, or any other area of your body that has been exposed to the sun may develop them. Your skin may darken as a result of these lesions.
• Scaly, coin-shaped lesions: Your scalp and face develop red, scaly coin-shaped lesions. These don’t hurt or itch, but they can leave scars and change the color of your skin. These lesions may clog the hair follicle, causing your hair to fall out. If the lesions leave behind scars after they heal, hair loss might become permanent.
• Between the knuckles of the fingers: A rash between the fingers’ knuckles.

How to Diagnose Cutaneous Lupus Erythematosus?

To confirm a diagnosis of cutaneous lupus, a skin biopsy is frequently required. Local anesthesia is administered before the physician performs either a shave biopsy, which involves scraping the skin or a punch biopsy, which is more commonly used in lupus. 

To obtain the latter, the physician inserts a pencil-like device with a hollow hub at the end into the affected area’s skin; a cylinder biopsy of the tissue is removed, and the “punch” is closed with stitches, leaving a small scar.

What is the Treatment for Cutaneous Lupus Erythematosus?

The physician may recommend medications based on the severity of your symptoms. These drugs do not cure lupus, but they can help with symptoms and flare-ups.

Lupus rash treatment may include the following:

• Swelling and inflammation are reduced by topical steroid ointments such as fluocinolone acetonide (Synalar®) or hydrocortisone butyrate (Locoid®).
• Pain and swelling are reduced by anti-inflammatory medications such as dapsone (Aczone®) or low-dose methotrexate (OtrexupTM, Rasuvo®).
• Antimalarial medications, such as hydroxychloroquine (Plaquenil®), alleviate fatigue, rashes, and joint pain.
• Calcineurin inhibitors, such as tacrolimus (Protopic®, Prograf®) or pimecrolimus (Elidel®), suppress your immune system and thus reduce inflammation.

The goal of treatment is to improve the skin’s appearance, reduce scarring, and prevent the development of new skin lesions.

How to avoid Flare-ups of Cutaneous Lupus Erythematosus Rash?

After being exposed to ultraviolet (UV) light, 40% to 70% of people with lupus experience symptom flare-ups. People with lupus should exercise extra caution when exposed to sunlight or artificial light to reduce flare-ups.

To avoid lupus butterfly rash flare-ups and protect yourself from UV rays, follow these steps:

• Every day, apply a broad-spectrum sunscreen with at least SPF 30.
• Avoid direct sunlight between the hours of 10 a.m. and 4 p.m.
• Avoid using tanning beds.
• Limit your exposure to indoor fluorescent lights.
• Wear sun-protective clothing, such as hats with wide brims and long sleeves.

Summary:

Acute cutaneous lupus is skin-related lupus. ACLE patients frequently have rashes that resemble sunburn. These rashes are most commonly found on the nose and cheeks. Sun protection can help many people avoid severe flare-ups. Medication may be prescribed by your healthcare provider to reduce the frequency and severity of symptoms. 

Lupus is a chronic disease with the right treatments, lifestyle changes, and regular monitoring it can be managed. Early detection and treatment can improve prognosis by preventing scarring, hair loss, and other long-term skin issues. Many Clinical Research Organizations based in the US are conducting clinical trials on potential treatment options that could help lessen the impact of various skin conditions.