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

• CCLE (Chronic Cutaneous Lupus Erythematosus)

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

• Chronic Cutaneous Lupus Erythematosus (CCLE) is a subtype of Systemic Lupus Erythematosus (SLE) that form a group of longstanding autoimmune disorders
• Systemic Lupus Erythematosus often affects the skin, as well as the internal organs. The four subtypes affecting the skin primarily are:
  • Chronic Cutaneous Lupus Erythematosus: A large proportion of cutaneous lupus erythematosus cases belong to the CCLE subtype
  • Acute Cutaneous Lupus Erythematosus
  • Chronic Cutaneous Lupus Erythematosus
  • Intermittent Cutaneous Lupus Erythematosus
• 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

There are several variants of Chronic Cutaneous Lupus Erythematosus, which include:

• Discoid Lupus Erythematosus (DLE); a majority of CCLEs are of the discoid subtype. DLE present with differing manifestations include:
  • Localized DLE
  • Generalized DLE
  • Hypertrophic DLE
• Lupus Erythematosus Tumidus (LE Tumidus)
• Lupus Profundus (Lupus Panniculitis)
• Chilblain Lupus Erythematosus (Chilblain LE)
• Lichenoid Cutaneous Lupus Erythematosus-Lichen Planus Overlap Syndrome (LE-LP Overlap Syndrome)

Chronic Cutaneous Lupus Erythematosus usually affects young and middle-aged women and is characterized by red skin rashes, usually on the head and neck area, arms, chest, and back (sun-exposed areas). Mucosal lesions may be seen in the mouth, nasal cavity, and genitalia.

• Chronic Cutaneous Lupus Erythematosus presentations are based upon the involved subtype, although there may be overlapping symptoms between the different subtypes. Since the involvement with systemic lupus erythematosus is noted in some cases, it may cause symptoms involving the entire body and several important organs
• Chronic Cutaneous Lupus Erythematosus may be diagnosed through physical examination, complete medical history, and specialized blood tests. The treatment of CCLE may involve topical applications, oral medication, phototherapy, and systemic corticosteroids for severe symptoms
• The prognosis depends on the severity of the disorder, the variant of the chronic type noted, and its response to therapy. In case of delayed diagnosis and/or treatment, Chronic Cutaneous Lupus Erythematosus can result in complications such as scarring and severe emotional stress. With adequate therapy, the prognosis may be good

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

• Individuals of any age may be affected by Chronic Cutaneous Lupus Erythematosus; but it is more commonly seen in the 20-50 year age group, affecting young and middle-aged adults
• Women are more prone to be affected than men
• Both fair-skinned and dark-skinned individuals can be affected, although darker-skinned Africans and Asians have a higher risk than fair-skinned Caucasians for CCLE
• Around 4 in 5 cases of cutaneous lupus are of the Chronic Cutaneous Lupus Erythematosus type

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

Risk factors associated with Chronic Cutaneous Lupus Erythematosus may include:

• Presence of systemic lupus erythematosus (SLE), an autoimmune disorder affecting several systems and organs of the body. It is reported that about 25% of Chronic Cutaneous Lupus Erythematosus cases are associated with SLE
• 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 Chronic Cutaneous Lupus Erythematosus? (Etiology)

The exact cause of Chronic Cutaneous Lupus Erythematosus is not fully known or understood.

• It is most likely 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 own tissue can be severe, resulting in significant signs and symptoms
• CCLE is associated with systemic lupus erythematosus (SLE) in some cases. But, it may also occur when there is a severe flare-up of SLE. When associated with SLE, it 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 Chronic Cutaneous Lupus Erythematosus?

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

The signs and symptoms may include:

• The presence of red skin lesions or thickened 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), the arms, and the trunk. These represent the major areas of sun exposure
• Involvement of mucosal membranes may be noted; thus, ulcers can form on the genital mucosa, lips, mouth, and inside the nasal cavity
• In many, the skin symptoms are mostly confined to a region (localized CCLE). In other cases, widespread involvement of the skin may be noted (generalized CCLE)
• The skin lesions heal with scarring
• Increased or decreased skin pigmentation
• Exposure to sunlight makes the condition worse

The signs and symptoms of systemic lupus erythematosus, 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. However, in over 75% of the cases, no systemic symptoms are noted due to an absence of SLE.

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

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

How is Chronic Cutaneous Lupus Erythematosus Diagnosed?

Chronic 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. In many cases, negative results for ANA antibodies are seen
  • 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. Generally, these antibodies are not elevated
  • 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

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

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 Chronic Cutaneous Lupus Erythematosus?

The complications of Chronic Cutaneous Lupus Erythematosus may include:

• Secondary bacterial and fungal infection due to scratching, bleeding, and ulceration
• Permanent hair loss, if the scalp is involved
• Eating and chewing difficulties, if mouth sores are present
• With time, scarring and atrophy of skin are usually reported; most cases of CCLE 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 CCLE may develop systemic lupus erythematosus during the course of their disorder

How is Chronic Cutaneous Lupus Erythematosus Treated?

The treatment undertaken for Chronic 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 for skin lesions and hair loss
• Topical/oral retinoid therapy and oral thalidomide. Retinoids and thalidomide cause birth defects and must be used cautiously or not at all in women who 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
• In case of severe symptoms, systemic therapy using antimalarial drugs may be necessary
• Systemic steroid therapy; injection of corticosteroids into the affected region
• In those who limit sun exposure, vitamin D supplements may be administered
• Immunosuppressive medication: Methotrexate, azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil, or the oral antibiotic minocycline for its immune-altering properties
• Intravenous immunoglobulin (IVIG) therapy
• Monoclonal anti-inflammatory antibody therapy

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

It is very important to note that Chronic 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 Chronic Cutaneous Lupus Erythematosus be Prevented?

• Currently, there is no effective preventive method available for Chronic 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
  • Avoid scratching or picking the skin lesions
  • Undertaking early treatment of viral infections
  • Addressing issues causing hormonal imbalance in the body
• In pregnant women with CCLE, 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 Chronic Cutaneous Lupus Erythematosus? (Outcomes/Resolutions)

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

• Individuals with mild cases of CCLE, who respond well to therapy, have a better prognosis than those with severe symptoms and poor response to treatment
• 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 Chronic Cutaneous Lupus Erythematosus:

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

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