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

• ICLE (Intermittent Cutaneous Lupus Erythematosus)
• Lupus Erythematosus Tumidus
• Tumid Lupus Erythematosus

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

• Intermittent Cutaneous Lupus Erythematosus (ICLE), or commonly Lupus Erythematosus Tumidus, is an uncommon subtype of chronic cutaneous lupus erythematosus (CCLE), Acute Cutaneous Lupus, Erythematosus (ACLE) or Subacute Cutaneous Lupus Erythematosus (SCLE) hat form a group of autoimmune disorders affecting the skin primarily. Some researchers classify ICLE as a separate entity in itself, but this is currently under debate
• 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
• Intermittent Cutaneous Lupus Erythematosus may be described as a milder form of cutaneous lupus erythematosus (CLE) presenting only skin symptoms in a vast majority of the affected individuals. Also, unlike other lupus types that mostly affect women, ICLE shows a preference for adult males
• Intermittent Cutaneous Lupus Erythematosus is characterized by the presence of the skin manifestations of the subtype of lupus under which the disorder is classified.  This diagnosis is made in cases where the findings are intermittent and milder than usually seen
• Most of the findings are on the sun-exposed areas of the body. The skin eruptions get worse on exposure to sunlight, but they improve during the winter months
• The exact cause of Intermittent Cutaneous Lupus Erythematosus is not yet known. A very small minority of cases may be associated with systemic lupus erythematosus (SLE), which is a serious disorder that can potentially involve several organs and systems of the body
• Intermittent Cutaneous Lupus Erythematosus may be diagnosed through physical examination, complete medical history, and specialized blood tests. The treatment of ICLE may involve topical applications, oral medication, phototherapy, and systemic corticosteroids for severe symptoms
• The prognosis depends on the severity of the disorder and its response to therapy. In general, the prognosis of Intermittent Cutaneous Lupus Erythematosus is much better than many other subtypes of cutaneous lupus erythematosus

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

• Individuals of any age may be affected by Intermittent Cutaneous Lupus Erythematosus
• In general, cutaneous lupus erythematosus (CLE) is frequently observed in the 20-50 year age group
• In the intermittent type, unlike other forms of lupus erythematosus, men are more prone to be affected than women
• 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 Intermittent Cutaneous Lupus Erythematosus? (Predisposing Factors)

Risk factors associated with Intermittent Cutaneous Lupus Erythematosus may include:

• The presence of systemic lupus erythematosus (SLE), an autoimmune disorder affecting several systems and organs of the body, is a risk factor. It is reported that about 25% of chronic cutaneous lupus erythematosus cases are associated with SLE
• Male 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 Intermittent Cutaneous Lupus Erythematosus? (Etiology)

The exact cause of Intermittent 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 healthy tissues instead. Such an attack by the body’s immune cells against its tissue can be severe, resulting in significant signs and symptoms
• ICLE may be associated with systemic lupus erythematosus (SLE) in some rare cases since it is a form of chronic cutaneous lupus erythematosus. 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 Intermittent Cutaneous Lupus Erythematosus?

The signs and symptoms of Intermittent Cutaneous Lupus Erythematosus (ICLE) may vary from one individual to another. It may be mild in some individuals or severe in others. In ICLE, the subcutaneous tissue (dermal tissue) is affected, and other systemic symptoms are usually not observed unless there is a rare association with systemic lupus erythematosus. Also, ICLE is not known to exhibit overlapping symptoms of other cutaneous lupus subtypes.

The signs and symptoms of ICLE may include:

• Presence of red plaques and patches, which can be circular or oval in shape (annular erythematous plaques)
• The lesions are small, raised, and fluid-filled with sharp boundaries
• Unlike other lupus types, ICLE lesions are smooth and non-scarring; meaning that on healing, no scars are left behind
• The skin lesions are observed on body regions exposed to sun, such as the face, neck, upper chest and back, and arms
• A symmetric manifestation of the skin rashes are noted in most cases
• The skin symptoms may get better during the cooler months; the symptoms are known to “come and go”

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

• Exposure to sunlight can cause severe flaring-up of the lesions
• Smoking
• Certain viral infections
• Certain medications
• Hormonal influence

How is Intermittent Cutaneous Lupus Erythematosus Diagnosed?

Intermittent 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; about 10% of the cases may present positive test results
  • 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
• 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
• A differential diagnosis is undertaken to exclude Jessner lymphocytic infiltration of the skin, a chronic and benign skin condition showing symptoms somewhat similar to ICLE

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 Intermittent Cutaneous Lupus Erythematosus?

The complications of Intermittent Cutaneous Lupus Erythematosus may include:

• Secondary bacterial and fungal infection due to scratching, bleeding, and ulceration
• Cosmetic concerns causing emotional and psychological stress
• Rarely, complications that arise from systemic lupus erythematosus may be noted

How is Intermittent Cutaneous Lupus Erythematosus Treated?

The treatment undertaken for Intermittent 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; topical tacrolimus
• Topical/oral retinoid therapy
• Phototherapy using ultraviolet A1
• In case of severe symptoms, systemic therapy using antimalarial drugs may be necessary
• Systemic steroid therapy, if needed
• In those who limit sun exposure, vitamin D supplements may be administered
• The many other treatments used for some forms of Lupus Erythematosus may be used in ICLE, based on clinical judgment.

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

How can Intermittent Cutaneous Lupus Erythematosus be Prevented?

• Currently, there is no effective preventive method available for Intermittent Cutaneous Lupus Erythematosus (ICLE)
• 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
• Educating the individual and keeping them well-informed about lupus is important and beneficial

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

The prognosis of Intermittent Cutaneous Lupus Erythematosus depends on the severity of the signs and symptoms and the response to treatment.

• In a majority of cases, the prognosis of ICLE is good with appropriate treatment. The condition follows a benign course and responds well to appropriate treatment
• Also, in a vast majority of cases, only skin symptoms are noted. The involvement of skin is generally mild, and no scarring occurs post-treatment

Additional and Relevant Useful Information for Intermittent Cutaneous Lupus Erythematosus:

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

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