What are the other Names for this Condition? (Also known as/Synonyms)

• CLE (Cutaneous Lupus Erythematosus)

What is Cutaneous Lupus Erythematosus? (Definition/Background Information)

• Cutaneous Lupus Erythematosus (CLE) constitutes a wide group of autoimmune disorders that primarily affect the skin. It may or may not be associated with systemic lupus erythematosus (SLE), which may cause systemic symptoms involving the entire body and several important organs. CLE is usually skin findings without systemic involvement
• Autoimmune disorders cause the body to mistake normal healthy tissues and organs for dangerous foreign material and attack them leading to long-term chronic inflammation. Such an attack by the body’s immune cells against its own tissue can be severe, resulting in significant signs and symptoms
• There are three main subtypes of Cutaneous Lupus Erythematosus, and these include:
  • Acute Cutaneous Lupus Erythematosus (ACLE)
  • Subacute Cutaneous Lupus Erythematosus (SCLE)
  • Chronic Cutaneous Lupus Erythematosus (CCLE)
  • In addition,  Intermittent Cutaneous Lupus Erythematosus (ICLE) is seen uncommonly

Each of the above subtypes has further subclasses with varied manifestations. The skin presentations are based on the involved subtype, although there may be overlapping symptoms between the different subtypes. Also, the signs and symptoms may vary widely from one individual to another; they may be mild or severe.

• Cutaneous Lupus Erythematosus typically results in the formation of red skin lesions, usually on the sun-exposed parts of the body, chiefly the head and neck (face) region. Exposure to sunlight can aggravate the symptoms. Lesions may also affect the oral and genital mucosal surfaces
• Depending on the severity, the skin lesions may be focal (confined to a small area) or diffuse (involving wider areas of the body). In many, the lesions heal to leave scars behind. Longstanding lesions are known to increase the risk for skin cancers
• Cutaneous Lupus Erythematosus may be diagnosed through physical examination, complete medical history, and specialized blood tests. The signs and symptoms are similar to many conditions; hence, the physician should be aware of the condition to consider it in the diagnosis
• The treatment of Cutaneous Lupus Erythematosus may involve topical applications, oral medication, phototherapy, and systemic corticosteroids for severe symptoms
• The prognosis depends on the subtype of Cutaneous Lupus Erythematosus involved, the severity of the disorder, and its response to therapy. With early and adequate therapy, the prognosis may be good or improved

The subtypes and subclasses of Cutaneous Lupus Erythematosus (CLE) based on dermatologic manifestations are:

• Acute Cutaneous Lupus Erythematosus (ACLE)
  • Localized ACLE
  • Generalized ACLE
  • Toxic Epidermal Necrosis-like ACLE
  • Intermittent Cutaneous Lupus Erythematosus
• Subacute Cutaneous Lupus Erythematosus (SCLE)
  • Annular SCLE
  • Papulosquamous SCLE
  • Neonatal Lupus Erythematosus
  • Erythrodermic SCLE
  • Poikilodermatous SCLE
  • Erythema Multiforme-like SCLE
  • Vesicobullous Annular SCLE
• Chronic Cutaneous Lupus Erythematosus (CCLE)
• Discoid Lupus Erythematosus (DLE)
  • Localized DLE
  • Generalized DLE
  • Hypertrophic DLE
  • Mucosal DLE
• Lupus Erythematosus Tumidus (LE Tumidus)
• Lupus Profundus (Lupus Panniculitis)
• Chilblain Lupus
• Lupus Erythematosus and Lichen Planus Overlap Syndrome

(No Subclasses)

Who gets Cutaneous Lupus Erythematosus? (Age and Sex Distribution)

• In general, Cutaneous Lupus Erythematosus is frequently observed in the 20-50 year age group
• Typically, women are more prone to be affected than men
• Both fair-skinned and dark-skinned individuals can be affected, although darker-skinned individuals (Africans and Asians) have a higher risk for CLE

What are the Risk Factors for Cutaneous Lupus Erythematosus? (Predisposing Factors)

The risk factors associated with Cutaneous Lupus Erythematosus may include:

• Presence of systemic lupus erythematosus (SLE), an autoimmune disorder affecting several systems and organs of the body
• Female gender
• Genetic predisposition: A family history of Cutaneous Lupus Erythematosus, which may be related to HLA genetic makeup, may increase one’s risk

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Cutaneous Lupus Erythematosus? (Etiology)

The exact cause of Cutaneous Lupus Erythematosus (CLE) is not fully known or understood.

• It is an autoimmune disorder, where antibodies of the body that fight diseases or infections attack one’s healthy tissues instead. Such an attack by the body’s immune cells against its tissue can be severe, resulting in significant signs and symptoms
• CLE is associated with systemic lupus erythematosus (SLE) in some cases. It may also occur when there is a severe flare-up of SLE. This can often result in inflammation in other parts of the body
• Some individuals may have a genetic predisposition for the condition; which may then be triggered by infections, certain drugs, or on exposure to sunlight

What are the Signs and Symptoms of Cutaneous Lupus Erythematosus?

The signs and symptoms of Cutaneous Lupus Erythematosus (CLE) may vary from one individual to another. It may be mild in some individuals or severe in others. It also depends on the subtype and associated SLE, if any are present.

The signs and symptoms may include:

• The presence of red skin lesions or thickened skin patches
• There may be loss of hair in the affected skin region
• The lesions may be painful or painless
• The usual sites of presentation are the head and neck region (including the face), and the arms, and trunk
• Involvement of mucosal membranes may be noted; thus, ulcers can form on the genital mucosa, lips, mouth, and inside the nasal cavity
• In some, the lesions can be localized to small areas of the skin (localized CLE); while, in others, the skin lesions can be extensive, affecting various parts of the body (generalized CLE)
• The skin lesions heal with scarring in many subtypes
• Increased or decreased skin pigmentation may be noted
• In all types of CLE, exposure to sunlight either induces the appearance of skin symptoms or makes the existing symptoms worse

The signs and symptoms of systemic lupus erythematosus (SLE), if present, may be noted. Apart from the skin symptoms, this may include low-grade fever, appetite loss, joint pain, enlarged lymph nodes, and involvement of various body organs.

Certain factors can worsen the signs and symptoms (causing flare-ups), and these include:

• Trauma
• Smoking
• Certain viral infections
• Certain medications
• Hormonal influence

How is Cutaneous Lupus Erythematosus Diagnosed?

Cutaneous Lupus Erythematosus may be diagnosed using the following tests and exams:

• Complete physical examination with a comprehensive evaluation of one’s medical history
• Assessment of the signs and symptoms
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Blood tests that include:
  • Routine blood testing (sedimentation rate/C-reactive protein)
  • Antinuclear antibody (ANA) levels blood test to look for antibodies in blood
  • Serological blood test for anti-double-stranded DNA (ds-DNA)
  • Extractable nuclear antigen antibodies panel blood test to detect anti-Ro antibodies and anti-La antibodies
  • Blood chemistry panels
• Body fluid analysis, if needed
• Skin/tissue biopsy: A tissue biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed), and microscope findings, the pathologist arrives at a definitive diagnosis
• Direct immunofluorescence studies on tissue biopsies
• Differential diagnosis to exclude conditions presenting symptoms similar to CLE

In order to diagnose systemic lupus erythematosus, a set of criteria established by the US-based organization American College of Rheumatology may be used.

The American College of Rheumatology (ACR) has established a set of 11 criteria to make (or rule-out) the diagnosis of Systemic Lupus Erythematosus (SLE). These criteria are closely related to the symptoms of Lupus, which include:

• Skin signs and symptoms of Lupus: Malar rash (butterfly-shaped rash stretching across the cheeks and nose); photosensitivity rash due to sunlight; discoid (red and raised) rashes on the skin
• Mouth and nose signs and symptoms of Lupus that include oral and nasal ulcers
• Joints signs and symptoms of Lupus, such as joint pain or arthralgia
• Blood changes in lupus: Decreased red blood cells, white blood cells, and platelets
• Renal signs and symptoms of Lupus that may range from proteinuria (the presence of proteins in the urine) to end-stage kidney disease
• Brain signs and symptoms of Lupus, such as seizures, stroke, meningitis (infection of the protective coverings of the brain), and psychosis
• Heart and lung signs and symptoms of Lupus that include chest pain, pulmonary hypertension, pleuritis, myocarditis (inflammation of heart muscle), or pericarditis
• Serology test: Antinuclear antibody (ANA) positive
• Serological blood test: Anti-double-stranded DNA (ds-DNA) positive

Out of these 11, four criteria are needed to make a diagnosis of SLE.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Cutaneous Lupus Erythematosus?

The complications of Cutaneous Lupus Erythematosus (CLE) may include:

• Secondary bacterial and fungal infection due to scratching, bleeding, and ulceration
• Permanent hair loss (alopecia), if the scalp is involved
• Eating and chewing difficulties, if mouth sores are present
• With time, scarring and atrophy of skin is usually reported; many cases of CLE report scarring and disfiguration
• Cosmetic concerns causing severe emotional and psychological stress
• Depression and social isolation
• Severe systemic lupus erythematosus (SLE) causing involvement of vital organs, such as the brain, lungs, heart, liver, and kidneys, resulting in organ dysfunction
• Longstanding ulcers can result in the development of squamous cell carcinoma (SCC)
• Some individuals with CLE may develop SLE during the course of their disorder

How is Cutaneous Lupus Erythematosus Treated?

The treatment undertaken for Cutaneous Lupus Erythematosus consists of decreasing inflammation and autoimmune activity in the body and minimizing the occurrence of flare-ups. The treatment measures may include:

• Topical medication and ointments
• Oral retinoid therapy and thalidomide. These medications cause birth defects and must be used with caution, if at all, in women who are/might become pregnant
• Intralesional corticosteroid injections when drugs are injected into the skin lesions
• Photodynamic therapy that includes photopheresis therapy
• Phototherapy using ultraviolet A1
• Laser therapy for cosmetic skin lesions
• Cosmetic surgery to remove disfiguring scars
• Antimalarial drugs, hydroxychloroquine or chloroquine, are often used
• Systemic steroid therapy
• In those who limit sun exposure, vitamin D supplements may be administered
• Immunosuppressive medication used include methotrexate, cyclophosphamide, azathioprine, mycophenolate mofetil, tacrolimus, minocycline, cyclosporin, leflunomide, and others
• Intravenous immunoglobulin (IVIG) therapy
• Inflammation-decreasing monoclonal antibody therapy, including belimumab and rituximab
• Other medications are being tested in clinical trials

Treatment measures for underlying systemic lupus erythematosus, if present, may be instituted.

It is important to note that Cutaneous Lupus Erythematosus in pregnant women should be carefully evaluated by a qualified healthcare provider before starting and/or performing any treatment. This is because some of the treatment measures may have the potential to affect the developing fetus, and hence, the guidance of a healthcare expert is crucial.

How can Cutaneous Lupus Erythematosus be Prevented?

Currently, there is no effective preventive method available for Cutaneous Lupus Erythematosus.

• Flare-ups can be prevented by managing medications, avoiding sun exposure, utilizing suitable sunscreens and clothing (cover-up skin exposed to sunlight), and close monitoring of the symptoms
• Avoiding factors that aggravate the condition through the following measures:
  • Avoidance of smoking
  • Undertaking early treatment of viral infections
  • Addressing issues causing hormonal imbalance in the body
• In pregnant women with CLE, careful and periodic monitoring of the pregnancy is advised and recommended
• Educating the individual and keeping them well-informed about lupus is important and beneficial

What is the Prognosis of Cutaneous Lupus Erythematosus? (Outcomes/Resolutions)

The prognosis of Cutaneous Lupus Erythematosus depends on the subtype of CLE, the severity of the signs and symptoms, its association with systemic lupus erythematosus, and the response to treatment.

• Individuals with mild cases of CLE, who respond well to therapy, have a better prognosis than those with severe symptoms and poor response to treatment
• Through proper treatment and monitoring of the condition, many individuals are able to lead normal, healthy lives while managing flares
• It is important that appropriate treatment be provided during pregnancy and close monitoring of both the mother and developing fetus be undertaken

Additional and Relevant Useful Information for Cutaneous Lupus Erythematosus:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/diseases-conditions/systemic-lupus-erythematosus/