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

• DLE (Discoid Lupus Erythematosus)

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

• Discoid Lupus Erythematosus (DLE) is the most common subtype of Chronic Cutaneous Lupus Erythematosus (CCLE) 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
• The Discoid Lupus Erythematosus subclass has four manifestations:
  • Localized DLE
  • Generalized DLE
  • Hypertrophic DLE
  • Mucosal DLE
• Discoid Lupus Erythematosus most frequently affects middle-aged women and is characterized by a red skin rash in the shape of a ‘disc’ accompanied by scaling, usually on the head and neck area. Hair loss on the scalp can occur with large bald patches
• The exact cause of Discoid Lupus Erythematosus is not yet known. Only in a few cases is it associated with systemic lupus erythematosus (SLE), which is a serious disorder that can potentially involve several organs and systems of the body
• The condition may be diagnosed through physical examination, complete medical history, and specialized blood tests. The treatment of Discoid Lupus Erythematosus may involve topical medications, oral medications, and systemic corticosteroids for severe symptoms
• The prognosis depends on the severity of the disorder and its response to therapy. In case of delayed diagnosis and/or treatment, Discoid Lupus Erythematosus can result in complications such as scarring and severe emotional stress. However, apart from the skin symptoms, no other systemic presentations are usually noted

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

• Individuals of any age may be affected by Discoid 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 in a 3:1 female-male ratio
• Both fair-skinned and dark-skinned individuals can be affected, although darker-skinned Africans and Asians have a much higher risk than fair-skinned Caucasians for DLE

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

Risk factors associated with Discoid 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 10-20% 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 Discoid Lupus Erythematosus? (Etiology)

The exact cause of Discoid Lupus Erythematosus (DLE) is not fully known or understood.

• It is most likely an autoimmune disorder, where living immune T-cells and B-cells, and 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
• DLE is usually not associated with systemic lupus erythematosus (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 Discoid Lupus Erythematosus?

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

• The presence of distinctive skin lesions that appear like discs or are coin-shaped. Hence, the lesions are called ‘discoid’
• These are usually in the form of red scaly patches on the skin; there is loss of hair in the affected skin region
• The lesions develop to form large discoid macules/papules and present pain on touch
• In a majority, the symptoms are mostly confined to the head and neck region, termed localized DLE. In this region, the nose, cheek, ear, and scalp are typically involved
• In some, the lesions may also involve the arms (forearms) and back, when it is termed generalized DLE
• Involvement of mucosal membranes is occasionally noted; thus, ulcers can form on the genital mucosa, lips, mouth, and inside the nasal cavities
• The skin lesions heal with scarring
• Increased or decreased skin pigmentation, called hyperpigmentation or hypopigmentation, respectively, may be observed
• Bald patches may be noticed, especially on the scalp. In some, hair loss can be permanent, causing scarring alopecia (cicatricial alopecia). In others, there may be partial or complete hair loss
• 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.

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 Discoid Lupus Erythematosus Diagnosed?

Discoid 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 presenting 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 such as complete blood count (CBC) test, sedimentation rate, and C-reactive protein test
  • Antinuclear antibody (ANA) levels blood test to look for antibodies in blood. Only some cases show positive results for ANA antibodies
  • 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
  • Urinalysis
• 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 Discoid Lupus Erythematosus?

The complications of Discoid Lupus Erythematosus (DLE) may include:

• Secondary bacterial and fungal infection due to scratching, bleeding, and ulceration
• The hair follicles may be completely destroyed, causing permanent hair loss
• With time, scarring and atrophy of skin are usually reported
• 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
• Longstanding ulcers can result in the development of squamous cell carcinoma (SCC)
• About 10-20% of the individuals with DLE may develop systemic lupus erythematosus during the course of their disorder

How is Discoid Lupus Erythematosus Treated?

The treatment undertaken for Discoid 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
• Antimalarial treatment with hydroxychloroquine, chloroquine, or quinacrine
• Intralesional corticosteroid injections, in which medication is injected into the skin lesions
• Systemic steroid therapy
• In those who limit sun exposure, vitamin D supplements may be administered
• Immune suppressive therapy: Mycophenolate mofetil, methotrexate, azathioprine, leflunomide, cyclosporin, tacrolimus, cyclophosphamide, and minocycline
• Oral retinoids, or thalidomide, or its derivative, lenalidomide. These are all potent teratogens and must be used with restrictions in child-bearing age females
• Monoclonal antibody therapy

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

It is very important to note again that Discoid 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 Discoid Lupus Erythematosus be Prevented?

Currently, there is no effective preventive method available for Discoid Lupus Erythematosus (DLE).

• 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 DLE, 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 Discoid Lupus Erythematosus? (Outcomes/Resolutions)

The prognosis of Discoid Lupus Erythematosus 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 DLE, who respond well to therapy, have a better prognosis than those with severe symptoms and poor response to treatment
• In many cases, the prognosis is good, and with 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 Discoid Lupus Erythematosus:

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

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