What Is Cutaneous Lupus Erythematosus

Get health & wellness advice into your inbox

Your privacy is important to us. Any information you provide to us via this website may be placed by us on servers. If you do not agree to these placements, please do not provide the information.

Best Milk Alternative

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.

References

  1. Abernathy-Close L, Lazar S, Stannard J, Tsoi LC, Eddy S, Rizvi SM, et al. B cell signatures distinguish cutaneous lupus erythematosus subtypes and the presence of systemic disease activity. Front Immunol [Internet]. 2021 Nov 19 [cited 2023 Jun 28];12:775353. Available from: https://www.frontiersin.org/articles/10.3389/fimmu.2021.775353/full 
  2. Chiewchengchol D, Murphy R, Edwards SW, Beresford MW. Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature. Pediatr Rheumatol [Internet]. 2015 Dec [cited 2023 Jun 28];13(1):1. Available from: https://ped-rheum.biomedcentral.com/articles/10.1186/1546-0096-13-1 
  3. Drenkard C, Barbour KE, Greenlund KJ, Lim SS. The burden of living with cutaneous lupus erythematosus. Front Med (Lausanne) [Internet]. 2022 Aug 8 [cited 2023 Jul 5];9:897987. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9395260/
  4. Okon LG, Werth VP. Cutaneous lupus erythematosus: diagnosis and treatment. Best Pract Res Clin Rheumatol [Internet]. 2013 Jun [cited 2023 Jul 5];27(3):391–404. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3927537/ 
  5. Petty AJ, Floyd L, Henderson C, Nicholas MW. Cutaneous lupus erythematosus: progress and challenges. Curr Allergy Asthma Rep [Internet]. 2020 Apr 4 [cited 2023 Jul 5];20(5):12. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7548961/
  6. Vale ECS do, Garcia LC. Cutaneous lupus erythematosus: a review of etiopathogenic, clinical, diagnostic and therapeutic aspects. An Bras Dermatol [Internet]. 2023 [cited 2023 Jul 5];98(3):355–72. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10173173/ 
  7. Sprow G, Dan J, Merola JF, Werth VP. Emerging therapies in cutaneous lupus erythematosus. Front Med (Lausanne) [Internet]. 2022 Jul 11 [cited 2023 Jul 5];9:968323. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9313535/ 
  8. Uva L, Miguel D, Pinheiro C, Freitas JP, Marques Gomes M, Filipe P. Cutaneous manifestations of systemic lupus erythematosus. Autoimmune Dis [Internet]. 2012 [cited 2023 Jul 6];2012:834291. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3410306/

Get health & wellness advice into your inbox

Your privacy is important to us. Any information you provide to us via this website may be placed by us on servers. If you do not agree to these placements, please do not provide the information.

Best Milk Alternative
[optin-monster-inline slug="yw0fgpzdy6fjeb0bbekx"]
This content is purely informational and isn’t medical guidance. It shouldn’t replace professional medical counsel. Always consult your physician regarding treatment risks and benefits. See our editorial standards for more details.

Get our health newsletter

Get daily health and wellness advice from our medical team.
Your privacy is important to us. Any information you provide to this website may be placed by us on our servers. If you do not agree do not provide the information.

Elena Paspel

Master of Science in Engineering (Digital Health) - Tallinn University of Technology, Estonia

Bachelor of Laws - LLB (Hons), London Metropolitan University, UK

An experienced professional with a diverse background spanning law, pricing, and eHealth/Digital Health. Proficient in copywriting, medical terminology, healthcare interoperability standards, and MedTech regulations. A strong foundation in scientific research methodologies and user experience research supports the creation of compelling content for the biopharmaceutical, CROs, medical technology, and eHealth sectors.

Proven expertise in driving product vision, synthesizing complex information, and delivering user-centric solutions. Adept at streamlining workflows and processes, and drafting documentation and SOPs. Always open to collaborations and eager to connect with like-minded professionals.

Leave a Reply

Your email address will not be published. Required fields are marked *

my.klarity.health presents all health information in line with our terms and conditions. It is essential to understand that the medical information available on our platform is not intended to substitute the relationship between a patient and their physician or doctor, as well as any medical guidance they offer. Always consult with a healthcare professional before making any decisions based on the information found on our website.
Klarity is a citizen-centric health data management platform that enables citizens to securely access, control and share their own health data. Klarity Health Library aims to provide clear and evidence-based health and wellness related informative articles. 
Email:
Klarity / Managed Self Ltd
Alum House
5 Alum Chine Road
Westbourne Bournemouth BH4 8DT
VAT Number: 362 5758 74
Company Number: 10696687

Phone Number:

 +44 20 3239 9818