Cutaneous Lupus: Symptoms, Types, Causes and Treatment

Cutaneous lupus erythematosus (CLE) is an autoimmune skin disease that can occur on its own or as part of systemic lupus erythematosus (SLE). Its presence can be distressing, both physically and emotionally, due to visible skin changes and, in some cases, scarring. Understanding CLE is essential for early recognition, management, and improving quality of life for those affected. This article provides a comprehensive overview of the symptoms, types, causes, and treatments for cutaneous lupus, synthesizing up-to-date research and clinical guidelines.

Symptoms of Cutaneous Lupus

Cutaneous lupus manifests with a variety of skin and mucosal symptoms. These can range from mild rashes to more severe, disfiguring lesions. Recognizing these signs is key to timely diagnosis and intervention.

Symptom	Description	Prevalence/Features	Source(s)
Malar rash	Butterfly-shaped erythema on cheeks/nose	Occurs in ~40-51% of SLE/CLE patients	1, 4
Photosensitivity	Skin reaction to sunlight/UV light	Seen in up to 63% of patients	1, 2, 4
Alopecia	Hair loss (scarring/non-scarring)	Scarring in 14%, non-scarring in 40%	1, 4
Oral ulcers	Painful or painless mouth sores	Reported in 19-31%	1, 4
Discoid lesions	Thick, scaly, coin-shaped plaques	25% in SLE; classic in chronic CLE	1, 2
Chilblain lupus	Painful, red-purple lesions on extremities	~20% in some cohorts	1, 3
Bullous eruptions	Blistering skin lesions	Rare (6-8%)	1, 2, 3
Raynaud’s phenomenon	Color changes in fingers/toes	Occurs in 60%	1, 2
Vasculitis	Inflamed blood vessels, purpura, ulcers	8-11%	1, 3, 5

Table 1: Key Symptoms

Overview of Cutaneous Lupus Symptoms

CLE symptoms are highly variable, influenced by subtype, sun exposure, and individual immune responses. These symptoms may be persistent or fluctuate over time.

Common Skin Findings

• Malar (Butterfly) Rash: One of the most recognizable features, appearing as symmetrical redness across the cheeks and nose, sparing the nasolabial folds. It may be flat or raised and often worsens with sun exposure 1, 4.
• Discoid Lesions: These are thick, scaly, disc-shaped plaques that may heal with scarring and pigment changes. They commonly affect the scalp, face, and ears and can cause permanent hair loss if present on the scalp 1, 2.
• Photosensitivity: Many patients report exacerbation of skin lesions after sun or UV light exposure. This photosensitivity is a hallmark of both cutaneous and systemic lupus 1, 2, 4.

Mucosal and Vascular Symptoms

• Oral Ulcers: Painless or painful sores inside the mouth, sometimes at disease onset. Ulcers may also appear in the nose 1, 4.
• Raynaud’s Phenomenon: This involves color changes—white, blue, then red—in fingers and toes in response to cold or stress, reflecting vascular involvement 1, 2.
• Vasculitis and Bullous Lesions: Some patients may develop small-vessel inflammation leading to purpura, ulcers, or blisters (bullae), particularly in severe disease 1, 2, 3, 5.

Less Common Manifestations

• Chilblain Lupus: Painful, red-purple, itchy lesions on fingers, toes, or ears, often triggered by cold 1, 3.
• Alopecia: Hair loss can be scarring (from discoid plaques) or non-scarring (diffuse thinning), affecting a significant portion of patients 1, 4.

Types of Cutaneous Lupus

CLE is a spectrum disorder with several subtypes, each with distinct presentations and implications for systemic involvement. Understanding these types supports accurate diagnosis and tailored management.

Type/Subtype	Key Features/Distribution	Systemic Risk	Source(s)
Acute (ACLE)	Malar rash, generalized erythema	High SLE association	2, 6, 4
Subacute (SCLE)	Annular or psoriasiform plaques, trunk	Moderate SLE risk, photosensitive	2, 6
Chronic (CCLE/DLE)	Discoid plaques, scalp/face/ears, scarring	Low SLE risk if isolated	2, 6, 4, 7
Chilblain lupus	Acral, cold-induced lesions	Rare SLE association	1, 3
Lupus profundus	Deep nodules, fat tissue involvement	Rare, may coexist with SLE	2, 3
Bullous lupus	Blistering, often severe	Often with SLE	1, 3
ICLE (Intermittent)	Transient, non-scarring, often facial	Low SLE risk	6

Table 2: Types of Cutaneous Lupus

Classification of Cutaneous Lupus

CLE is classified into several main types, each with unique clinical and histological features 2, 3, 6:

Acute Cutaneous Lupus Erythematosus (ACLE)

• Features: Presents as the classic malar (butterfly) rash or widespread erythematous patches.
• Systemic Association: Strongly associated with systemic lupus (SLE); skin lesions often coincide with disease flares 2, 4, 6.
• Distribution: Primarily facial, may extend to other sun-exposed areas.

Subacute Cutaneous Lupus Erythematosus (SCLE)

• Features: Annular (ring-shaped) or psoriasiform (scaly) plaques, typically non-scarring.
• Photosensitivity: Marked sensitivity to UV, with lesions often on the upper back, shoulders, chest, and neck.
• Antibodies: Frequently associated with anti-Ro (SSA) antibodies.
• Systemic Risk: Moderate; some patients develop mild systemic features 2, 6.

Chronic Cutaneous Lupus Erythematosus (CCLE)

• Discoid Lupus Erythematosus (DLE): The most common chronic form, characterized by thick, indurated, scaly plaques that may scar and cause pigment changes. Scalp involvement leads to scarring alopecia 2, 6, 7.
• Other CCLE Variants: Includes lupus tumidus (edematous plaques), lupus profundus (involving fat tissue), hyperkeratotic lupus, and chilblain lupus 2, 3.

Other and Rare Subtypes

• Chilblain Lupus: Often affects fingers, toes, or ears, and may be confused with frostbite or perniosis 1, 3.
• Lupus Profundus (Lupus Panniculitis): Presents as deep, firm nodules due to inflammation of subcutaneous fat 2, 3.
• Bullous Lupus: Characterized by blistering lesions, usually in those with active SLE 1, 3.

Intermittent Cutaneous Lupus Erythematosus (ICLE)

• Features: Transient, non-scarring lesions, often facial; usually less severe and less likely to associate with systemic disease 6.

Causes of Cutaneous Lupus

CLE arises from a complex interplay of genetic, environmental, and immunological factors. Understanding these mechanisms illuminates why certain individuals are affected and guides research into innovative therapies.

Factor	Role in Disease	Notable Details	Source(s)
Genetics	Predisposes individuals to CLE	Variations in immune-regulation genes	9, 10, 15
UV Light	Triggers/worsens skin lesions	Induces apoptosis, increases antigenicity	9, 10, 11
Immune dysregulation	Central to pathogenesis	Type I IFN, autoantibodies, cytokine cascades	9, 10, 11, 15
Infections	May trigger flares, worsen lesions	S. aureus colonization, viral triggers	12
Medications	Can induce SCLE	E.g., certain antihypertensives, antifungals	2
Smoking	Aggravates disease, reduces treatment response	Linked to higher CLE risk/severity	16

Table 3: Causes and Triggers

Genetic Susceptibility

• Genetic Factors: Polymorphisms in genes regulating immune responses (e.g., interferon pathway, cell death, antigen presentation, and immune cell regulation) increase susceptibility to CLE 9, 10, 15.
• Family History: Having relatives with autoimmune diseases may increase risk, though most cases are sporadic.

Environmental Triggers

• Ultraviolet (UV) Light: A major external trigger. UV exposure induces apoptosis in skin cells, releasing nuclear antigens and triggering immune responses. This explains the hallmark photosensitivity in CLE 9, 10, 11.
• Medications: Some drugs (e.g., certain antihypertensives, antifungals, proton pump inhibitors) can induce or worsen cutaneous lupus, especially SCLE 2.

Immunological Mechanisms

• Immune Dysregulation: CLE is marked by overactive innate and adaptive immunity, especially type I interferon-mediated pathways. Keratinocytes and immune cells release proinflammatory cytokines, driving chronic skin inflammation and the formation of interface dermatitis 9, 10, 11, 15.
• Autoantibodies: The presence of specific autoantibodies, such as anti-Ro/SSA, is common—especially in SCLE.

Infection and Microbiome

• Bacterial Colonization: Increased colonization with Staphylococcus aureus is found on lupus skin lesions, likely due to interferon-driven disruption of the skin barrier. Infections may trigger or perpetuate flares 12.

Lifestyle and Other Factors

• Smoking: Smoking is associated with increased risk and severity of CLE. It also negatively impacts the response to antimalarial drugs, a first-line CLE therapy 16.
• Hormonal Factors: While not as well defined, CLE is more frequent in women, suggesting a possible hormonal influence 11.

Treatment of Cutaneous Lupus

Managing CLE requires a comprehensive, individualized approach. Treatment goals include reducing inflammation, preventing flares, minimizing scarring, and improving quality of life.

Treatment	Indication/Use	Notes/Considerations	Source(s)
Sun protection	All patients	Essential preventive step	13, 14, 16
Topical steroids	Localized, mild lesions	First-line; risk of skin atrophy	13, 14
Calcineurin inhibitors	Sensitive areas, steroid-sparing	Effective, especially on face	13, 14, 16
Antimalarials	Moderate/severe or widespread disease	Hydroxychloroquine, chloroquine; first-line systemic	13, 14, 16
Systemic steroids	Severe, highly active CLE	Short-term use; avoid long-term	13, 14
Immunosuppressants	Refractory/severe cases	Methotrexate, mycophenolate, azathioprine, etc.	14, 16
Biologics	Refractory or severe disease	Belimumab, rituximab, JAK inhibitors (investigational)	14, 15, 16
Thalidomide/lenalidomide	Last-resort, refractory CLE	Risk of neurotoxicity, teratogenicity	14, 16
Lifestyle mods	Smoking cessation, vitamin D, infection management	Supportive role	16

Table 4: Treatment Options

General Measures

• Patient Education: Teaching patients about CLE, its triggers, and management improves adherence and outcomes 11.
• Sun Protection: Strict photoprotection is crucial. Recommendations include broad-spectrum sunscreens (SPF 50+), protective clothing, and avoidance of peak sunlight 13, 14, 16.

Topical Therapies

• Corticosteroids: First-line for localized, mild CLE lesions. Potency should match lesion severity and anatomical site (lower on the face, higher elsewhere). Risks include skin thinning and atrophy, especially with prolonged use 13, 14.
• Calcineurin Inhibitors (Tacrolimus, Pimecrolimus): Useful in facial or intertriginous areas where steroids may cause side effects. These agents are safe for long-term use and effective in steroid-resistant cases 13, 14, 16.

Systemic Therapies

• Antimalarials: Hydroxychloroquine is the systemic mainstay, effective for most CLE subtypes, especially widespread or scarring disease. Chloroquine and quinacrine may be added in refractory cases. Regular ophthalmologic monitoring is needed due to retinal toxicity risk 13, 14, 16.
• Systemic Corticosteroids: Reserved for severe or rapidly progressive lesions; should be used short-term due to long-term side effects 13, 14.
• Immunosuppressants: Methotrexate, mycophenolate mofetil, azathioprine, and dapsone are options for antimalarial-refractory or severe CLE. These require careful monitoring for toxicity 14, 16.

Biologic and Novel Therapies

• Biologics: Agents targeting B-cells (e.g., belimumab, rituximab) or interferon pathways (e.g., JAK inhibitors) are emerging options, especially for refractory cases. Clinical trial data is still limited, but these targeted therapies are promising 14, 15, 16.
• Thalidomide/Lenalidomide: Effective in severe, recalcitrant CLE but associated with significant risks (neuropathy, teratogenicity). Use is limited to selected, closely monitored patients 14, 16.

Supportive Strategies

• Smoking Cessation: Essential, as smoking worsens disease and reduces response to standard therapy 16.
• Vitamin D Optimization: Recommended due to photosensitivity and sun avoidance, which can lead to deficiency 16.
• Management of Infections: Prompt treatment of skin infections (e.g., S. aureus) is important, as these can trigger or worsen CLE lesions 12.

Conclusion

Cutaneous lupus erythematosus is a complex, multifaceted disease that significantly impacts those affected. Early recognition, accurate classification, understanding of underlying causes, and implementation of evidence-based therapies are crucial for optimal management.

Key points:

• CLE manifests in diverse ways, with symptoms ranging from subtle rashes to severe, scarring plaques.
• There are several main types—acute, subacute, chronic (discoid), and rare forms—each with unique features and systemic associations.
• Pathogenesis involves genetic susceptibility, immune dysregulation (notably type I interferon pathways), environmental triggers (especially UV light), and lifestyle factors like smoking.
• Treatment is individualized: sun protection and topical agents for mild disease, antimalarials for widespread or severe cases, and systemic immunosuppressants or biologics for refractory disease.
• Patient education, lifestyle modifications, and infection management are vital components of comprehensive care.

By staying informed and proactive, patients and healthcare providers can work together to effectively manage cutaneous lupus, minimize complications, and enhance quality of life.