What Is Cutaneous Lupus Erythematosus

Navigating the world of autoimmune diseases can be akin to finding your way through a dense forest. It can be daunting, especially when it comes to understanding the various forms of lupus. However, you've come to the right place. Klarity is here to guide you through the intricacies of Cutaneous Lupus Erythematosus (CLE), a complex yet manageable condition. Like a seasoned forest ranger, we will help you understand the landscape, identify the landmarks, and find your path.

Cutaneous Lupus Erythematosus (CLE) is a form of lupus that primarily affects the skin, resulting in a variety of lesions and rashes.¹ Imagine lupus as a tree, with its branches representing the different forms of the disease. One of these branches is Cutaneous Lupus Erythematosus. It is one of the three types of lupus, the others being Systemic Lupus Erythematosus (SLE), which affects multiple body systems, and Drug-induced Lupus, caused by certain medications.²

There's much more to learn about Cutaneous Lupus Erythematosus, including its types, causes, symptoms, and management strategies. So, keep reading to gain a comprehensive understanding of this condition.

Overview

Lupus is an autoimmune disease, which means that the body's immune system mistakenly attacks its own tissues. When lupus primarily affects the skin, it is known as Cutaneous Lupus Erythematosus (CLE). This condition can cause a variety of skin lesions that can be severe, persistent, and potentially scarring.² It's like a storm in the forest, causing damage to the trees (your skin) and leaving visible signs of its passage.

Types of cutaneous lupus erythematosus

Much like different species of trees in a forest, Cutaneous Lupus Erythematosus manifests in various forms on the skin's landscape. CLE can be categorized into several groves, each distinguished by their own unique blend of clinical attributes, the lifespan of their lesions, microscopic characteristics, and laboratory markers. These groups are:

Acute Cutaneous Lupus Erythematosus (ACLE)

Often associated with systemic lupus, ACLE is characterized by a malar rash, commonly known as a butterfly rash due to its distinctive shape, which spreads across the cheeks and nose.² It's like a sudden storm that leaves a clear and visible mark on the landscape. Acute CLE (ACLE) involves temporary rashes that often appear in sun-exposed areas.³

Subacute Cutaneous Lupus Erythematosus (SCLE)

This type presents as non-scarring skin lesions that might appear as ring-shaped or scaly patches, typically appearing on areas of the skin exposed to the sun.^2,3 It's like a slow-moving river that changes the landscape over time, leaving its mark without causing immediate destruction.

Chronic Cutaneous Lupus Erythematosus (CCLE)

This type is characterized by disc-shaped, round lesions. These lesions often lead to scarring and discolouration and in some cases, hair loss.² It's like a wildfire that leaves a lasting scar on the landscape, changing the terrain and leaving a visible mark. Chronic CLE (CCLE) is the largest "grove" and includes several distinctive conditions, such as discoid lupus erythematosus (DLE), lupus panniculitis (LEP), chilblain lupus (CHLE), and lupus tumidus (LET).^3,5 These conditions are less likely to be associated with systemic lupus erythematosus (SLE) and more with ACLE and SCLE.³ DLE, the most common subtype³, is like a well-known tree in the forest - it's characterized by red, disc-shaped, sticky plaques and bumps that can appear anywhere on the body, but are most likely to be on the scalp, ears, and face.³ DLE heals, leaving behind changes in skin color, thinning, scarring, and permanent hair loss.^2,3 

Causes of cutaneous lupus erythematosus

The exact cause of Cutaneous Lupus Erythematosus (CLE) is unknown. The development of cutaneous lupus is thought to be multifactorial.⁵ This includes genetic susceptibility, autoimmune induction, and immune system damage.⁴ Certain triggers such as sunlight, stress, smoking, and certain medications can also induce or worsen the skin lesions.^2,5 It's like a forest that's affected by both its genetic makeup (the types of trees it contains) and external factors like weather and human activity. 

Signs and symptoms of cutaneous lupus erythematosus

The signs and symptoms of Cutaneous Lupus Erythematosus (CLE) vary depending on the type of cutaneous lupus. Common symptoms include red, scaly, and thickened skin, often accompanied by hair loss and skin color changes. Lesions may also appear, particularly after exposure to the sun.² Like a unique signature, the localized form of CLE, accounting for 90%-95% of cases, is marked by the distinctive butterfly rash.⁶ This rash blossoms with a red hue and subtle flaking, symmetrically gracing the cheeks and nose's bridge, typically avoiding the folds that run from nose to mouth. It may also be accompanied by facial swelling.⁶

Management and treatment for cutaneous lupus erythematosus

Treatment for CLE aims to reduce the severity of symptoms, prevent flares, and limit the potential for scarring. Treating CLE is like forest management, where the goal is to prevent the formation and progression of lesions (akin to preventing forest fires or disease outbreaks), and to improve skin appearance (similar to maintaining the forest's overall health and beauty). This is achieved through a combination of patient education, and topical and systemic therapies. For small, localized patches of disease, topical corticosteroids or topical calcineurin inhibitors (TCIs)⁵ can often help. But, if the damage is widespread, treatments such as antimalarials like hydroxychloroquine (HCQ) or a brief intervention with systemic corticosteroids⁵ might be needed. 

There are several emerging therapies for cutaneous lupus erythematosus (CLE). These include anifrolumab and iberdomide, all showing potential benefits in taming some types of CLE.⁷ There are also other promising therapeutic pathways under exploration, including tyrosine kinase 2 (TYK2) and serine/threonine kinase IL-1R–associated kinase (IRAK4).⁷

Dermatologists stress strict sunscreen adherence, as UVA and UVB irradiation has been shown to induce CLE lesions.⁴

The risk of skin cancer

There is a rare but possible danger lurking within chronic lesions. Chronic lesions commonly associated with discoid lupus erythematosus (DLE) - a subtype of chronic CLE (CCLE), have the potential to transform into nonmelanoma skin cance Dark-skinned individuals may face a higher risk of skin cancer within these lesions.⁸ This is because the absence of pigmentation within the chronic lesion, combined with ongoing inflammation and sun damage, creates a vulnerable environment⁸, like a clearing in the forest where sunlight reaches the ground more easily, making it more prone to potential harm.

Diagnosis

Diagnosis of Cutaneous Lupus Erythematosus (CLE) is based on a combination of clinical findings, laboratory tests, and a skin biopsy, which involves taking a small sample of skin for examination under a microscope to help confirm the diagnosis.^2,4 The cornerstone of CLE diagnosis is a lesional biopsy for histology⁴, similar to how a tree's health is assessed by examining a sample of its bark or leaves. Histologic findings vary by subtype and include the presence of empty spaces or fluid-filled areas on the affected skin and the infiltration of certain immune cells called lymphocytes.⁴

FAQs

How can I prevent cutaneous lupus erythematosus?

While Cutaneous Lupus Erythematosus (CLE) cannot be entirely prevented, managing triggers such as sun exposure, stress, and smoking can help reduce the risk of flares. Preventing CLE is like preventing a forest fire. Patient education on heat, sun, and drug avoidance is standard. Patients should be advised to avoid manipulation of lesions⁴, as this can induce new lesions, much like how disturbing a diseased tree can spread the disease to healthy ones. Sufficient amounts of sunscreen (2mg/cm2) with a sun protection factor (SPF) of at least 50 should be applied 20-30 minutes prior to expected exposure.⁴

How common is cutaneous lupus erythematosus?

Cutaneous Lupus Erythematosus (CLE) is relatively common among individuals with lupus, with about 70% of people with lupus experiencing some form of skin involvement.²

Who is at risk of cutaneous lupus erythematosus?

While anyone can develop Cutaneous Lupus Erythematosus (CLE), it is more common in individuals between the ages of 20 and 50.² CLE is rare in children.³ Like many autoimmune conditions, CLE is more frequently encountered by people Assigned Female at Birth (AFAB)than those Assigned Male at Birth (AMAB). The incidence ratio ranges of people AFAB to those AMAB are known to be between 3:1 and 4:1 for CLE as a whole, and between 3:1 and 8:1 for DLE.³

Additionally, Black individuals tend to develop the disease at a younger age compared to White individuals.³ Moreover, African-Americans face a 5.4-fold higher risk of developing chronic cutaneous lupus erythematosus (CCLE) compared to Caucasian-Americans.⁶ 

In New Zealand, the indigenous Māori population is at a 2.47-fold greater risk of developing any CLE subtype and nearly six times more likely to develop CCLE than those of European descent.⁶

When should I see a doctor?

Just as you wouldn't ignore a persistent leak in your roof, don't ignore a persistent rash or lesions on your skin, particularly if they worsen after sun exposure. Whether lupus has been a word in your medical history or not, a consultation with your healthcare provider is crucial if you notice any of the above mentioned signs or symptoms. Early diagnosis and treatment for lupus skin disease can be your silver lining, potentially leading to an improved prognosis.

Summary

Just as a forest comprises different tree species, Cutaneous Lupus Erythematosus (CLE) encompasses diverse skin conditions. This includes Acute, Subacute, and Chronic CLE—each with unique traits and manifestations, like distinctive trees in a forest.

The causes of CLE remain enigmatic but likely involve a cocktail of genetic, hormonal, and environmental factors. Its symptoms often include persistent skin changes, such as redness, thickness, and hair loss, bearing unique patterns.

Treatment for CLE focuses on reducing symptoms, avoiding flares, and minimizing scars. Emerging treatments are on the horizon, like newly discovered paths leading to a healthier forest. Diagnosis relies on clinical findings, lab tests, and a skin biopsy.

Prevention can't guarantee total immunity but controlling triggers like sun exposure, stress, and smoking can help reduce flares. It's much like fire prevention in a forest.

Always remember, persistent skin changes call for a consultation with your healthcare provider—early diagnosis could be your silver lining for a better prognosis.