Lupus Erythematosus

Dr. Mohamed nasr

Types of Lupus:

Cutaneous (skin) lupus primarily affects the skin but may involve the hair and mucous

membranes also commonly called discoid lupus

Systemic lupus erythematosus (SLE) affects any system in the body

Drug-induced lupus erythematosus (DILE) Side effect of long term use of certain medications Symptoms overlap with those of SLE

Neonatal lupus very rare acquired from the passage of maternal autoantibodies

Cutaneous Lupus

ACUTE: Typical photosensitive malar rash when acute Highly associated with systemic LE (almost 100%)

SUBACUTE: This variant is psoriasiform or annular ~50% of these patients will meet criueria for SLE

CHRONIC: ie Discoid Lupus Most patients (85-90% never develop systemic lupus)

Systemic Lupus Erythematosus Chronic

autoimmune disease Most common form

of lupus Autoantibodies

produced by own immune system

recognize own DNA as foreign

Lupus “wolf”

Understanding the causes:

Unknown Possible Factors:

genetics environmental hormonal

May explain why lupus occurs more frequently in females than in males

NOT infectious

Screening and Diagnosis:

Difficult Usually takes months to even years Laboratory tests:

Antinuclear antibody (ANA) test that detects the presence of autoantibodies that attack your own cells

blood tests for anemia, low white-cell count, abnormalities in organ function

urinalysis electrocardiogram or echocardiogram to check the

heart chest x-ray

Eleven Criteria Used for the Diagnosis of Lupus:

Malar Rash Rash over cheeks

Discoid Rash Red raised patches

Photosensitivity Reaction to sunlight

Oral Ulcers Ulcers in nose or mouth

Arthritis Two or more joints

Serositis Pleuritis or pericarditis

Eleven Criteria cont…

Renal Disorder Excessive protein in the urine or cast.

Neurologic Disorder Seizures

Hematologic Disorder Hemolytic anemia or leukopenia

Immunologic Disorder Positive anti-double stranded anti-DNA test

Antinuclear Antibody Positive test

Why organs are attacked:

Due to autoantibodies Also referred to as anti-nuclear antibodies Antibodies produced by the immune system Attack the RNA and DNA in the nucleus of

own cells

Systems Affected

Musculoskeletal system -- avascular necrosis -- muscle inflammationKidney systemNervous system -- seizures -- nerve paralysis -- severe depression -- psychosis -- strokesBlood and Lymph system -- anemia -- thrombocytopenia -- leucopenia

Systems Affected

Stomach, Intestines, Liver, and Associated Organs -- ulcers -- abdominal painsSkin and Hair -- rash and alopeciaHeart and Blood Vessels -- pericarditis -- arthrosclerosis -- spasms of the arteryLungs -- pleurisy, pneumonia, and pleural effusionEyes -- rarely involved except for retina

The simplest of treatments include:

1- Anti-inflammatory drugs like Aspirin.

2- Anti- malarial drugs.

3- Immunosuppressive medications.

4- Corticosteroids.

Subacute Cutaneous Lupus Erythematosus

Widespread, non-scarring but often photosensitive rash.

Annular or papulosquamous morphology.Mild systemic disease common but renal

involvement rare.Positive ANA in most patients, but anti-nDNA

uncommon.Anti-Ro in two thirds patients.

Subacute Cutaneous Lupus

Discoid Lupus Erythematous (DLE)

Most scarring and chronic form of cutaneous lupus.

Discoid shaped plaques with white scale, with time, lesions become atrophic.

Can lead to scarring alopecia.Few patients meet criteria for SLE (6%).

The characteristics of DLE lesions:

1. Persistent localized erythema.

2. Adherent scales related to the dilated follicles.

3. Follicular plugging.

4. Redness & telangiectasia of the border.

5. Atrophy & scarring of the center.

Follicular Plugging

Diagnosis = lupus band test

Presence of IgG & C in linear pattern at dermo-epidermal junction below lamina densa in involved sun-exposed skin only.

Treatment:

Treat with intralesional or topical steroids, sun avoidance & antimalarial if severe or large areas involved.