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

• SCLE (Subacute Cutaneous Lupus Erythematosus)

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

• Subacute Cutaneous Lupus Erythematosus (SCLE) is a subtype of cutaneous lupus erythematosus (CLE) that form a group of autoimmune disorders affecting primarily the skin
• 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
• Subacute Cutaneous Lupus Erythematosus more frequently affects young women and is characterized by the presence of scaly papules and plaques on the arms and trunk. The skin eruptions may get worse on sun exposure
• The exact cause of Subacute Cutaneous Lupus Erythematosus is not yet known. In some cases, it is associated with systemic lupus erythematosus (SLE), which is a serious disorder that can potentially involve several organs and systems of the body
• Subacute Cutaneous Lupus Erythematosus (SCLE) has four common subtypes with differing manifestations. The subtypes are:
  • Annular SCLE
  • Papulosquamous SCLE
  • Drug-Induced SCLE
  • Neonatal Lupus Erythematosus
• Four less common subtypes include:
  • Erythrodermic SCLE
  • Poikilodermatous SCLE
  • Erythema Multiforme-like SCLE (also known as Rowell Syndrome)
  • Vesicobullous Annular SCLE
• The condition may be diagnosed through physical examination, complete medical history, and specialized blood tests. The treatment of Subacute Cutaneous Lupus Erythematosus may involve topical medications, oral medication, and systemic corticosteroids for severe symptoms.  Protection from sunlight and particularly ultraviolet light is necessary.
• The prognosis of Subacute Cutaneous Lupus Erythematosus depends on the severity of the disorder and its response to therapy. In general, the prognosis of the subacute type is better than the acute type of cutaneous lupus erythematosus

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

• Individuals of any age may be affected by Subacute Cutaneous Lupus Erythematosus; but, it is more commonly seen in young and middle-age
• In general, cutaneous lupus erythematosus (CLE) is frequently observed in the 20-50 year age group. 1 in 6 (15%) individuals with CLE are known to have SCLE
• 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 Subacute Cutaneous Lupus Erythematosus? (Predisposing Factors)

Risk factors associated with Subacute Cutaneous Lupus Erythematosus may include:

• Presence of systemic lupus erythematosus (SLE), an autoimmune disorder affecting several systems and organs of the body
• Female gender
• In 1 in 3 individuals, exposure to certain drugs is noted
• Genetic predisposition: A family history of cutaneous lupus erythematosus, which may be related to HLA genetic makeup, may increase one’s risk
• Presence of an underlying illness
• Neonatal Lupus Erythematosus occurs in children born to woman with Lupus antibodies which are passed through the placenta

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 Subacute Cutaneous Lupus Erythematosus? (Etiology)

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

• It is an autoimmune disorder, where antibodies of the body that fight diseases or infections, attack one’s own 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
• SCLE 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 Subacute Cutaneous Lupus Erythematosus?

The signs and symptoms of Subacute Cutaneous Lupus Erythematosus (SCLE) may vary from one individual to another. It may be mild in some individuals or severe in others and may include:

• Presence of a papulosquamous red rash that presents with patches of skin with pink to red papules with fine scales
• Over time, the lesions become plaques with a central clearing called annular plaques. They do not heal with a scar but sometimes have a faint decrease in pigment at the previously involved site
• The skin lesions are usually non-itchy and may involve the arms, chest, and back
• Half of the individuals with SCLE are known to have mild symptoms of systemic lupus erythematosus, such as joint pain, fatigue, and anemia

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

• Exposure to sunlight
• Smoking
• Certain viral infections
• Certain medications
• Hormonal influence

How is Subacute Cutaneous Lupus Erythematosus Diagnosed?

Subacute 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)
  • Autoantibody panel blood test to detect anti-Ro antibodies and anti-La antibodies; 70% of individuals with SCLE have anti-Ro and anti-La antibodies
  • Blood chemistry panels
• Body fluid analysis
• 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 Subacute Cutaneous Lupus Erythematosus?

The complications of Subacute Cutaneous Lupus Erythematosus may include:

• Secondary bacterial and fungal infection due to scratching, bleeding, and ulceration
• Cosmetic concerns causing emotional and psychological stress
• Severe systemic lupus erythematosus (SLE) causing involvement of vital organs, such as the brain, lungs, heart, liver, and kidneys, resulting in organ dysfunction
• Presence of antiphospholipid syndrome associated with underlying active SLE
• Pregnant women with SCLE are at an increased risk for delivering babies with neonatal lupus syndrome. Their infants are at risk for congenital conduction heart defects, other heart disease, and rash

How is Subacute Cutaneous Lupus Erythematosus Treated?

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

• Topical corticosteroids in cream or ointment form
• Intralesional corticosteroid injections, when drugs are injected into the skin lesions
• The first line oral medication of SCLE is the antimalarial drug hydroxychloroquine sulfate. If that alone is not successful, another antimalarial medication may be added or substituted

Please note that SCLE and other forms of cutaneous lupus erythematosus are not linked to or caused by malaria. It was discovered empirically that antimalarials are effective in these and other autoimmune diseases.

• Systemic steroid therapy
• Other immunosuppressive medications such as mycophenolate mofetil, azathioprine, or methotrexate
• Oral retinoids or thalidomide. Both of these medications can cause birth defects and must be used with caution in women who might become pregnant
• Other medications are under investigation for their possible use in SCLE
• Sun protection is a necessary and important part of the treatment plan for SCLE and other cutaneous forms of lupus
  • This must include the important components of avoiding midday sun, wearing protective clothing, preferably with a fabric with SPF rating, hats, gloves, and so on
  • SPF sunscreen is applied every day, twice. Opaque make-up can also help with protection
  • In those who limit sun exposure, vitamin D supplements may be administered

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

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

• Currently, there is no effective preventive method available for Subacute Cutaneous Lupus Erythematosus (SCLE)
• 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 SCLE, careful and periodic monitoring of the pregnancy is advised and recommended. The infant can develop first-degree heart block, which leads to a slow heart rate
• Educating the individual and keeping them well-informed about lupus is important and beneficial

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

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

• Individuals with mild cases of SCLE, 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 Subacute Cutaneous Lupus Erythematosus:

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

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