What is Chronic Cutaneous Lupus Erythematus / Discoid Lupus?

Discoid lupus erythematosus (DLE) is cutaneous manifestation of the autoimmune spectrum of lupus erythematosus.  It is further classified as a form of chronic cutaneous lupus erythematosus which primarily affects the skin and hair follicles.   DLE is not considered a primary cicatricial alopecia, because the inflammation surrounds not only the hair follicle but many other structures in the skin.   If treated early, hair loss in DLE it is potentially reversible.  If not treated early, over half the patients will develop scarring alopecia.

Cause
Like many autoimmune disorders, the true cause is unknown.  It likely is caused by a combination of genetic susceptibility, environmental triggers and other factors including medications and other medical comorbidities.

Signs & Symptoms
DLE is more common in women, especially women of African ancestry.  It may occur at any age, but onset is frequently in persons between age 20 and 40 years.  Few patients (5 to 10%) with DLE progress to SLE, although in patients with classic SLE, DLE occurs in about 20% of patients.

DLE most commonly affects the scalp, face, and ears.  It starts as a well-defined scaly purplish patch and enlarges into a discoid (coin-shaped) patch of hair loss. Decreased or increased pigmentation, increased blood vessels, and follicular plugging are characteristically seen in the center of the of discoid lupus lesions.  Another helpful finding is the “carpet tack” sign that may be elicited by lifting the adherent scale and revealing spikey keratin on the underside.  External ear involvement is common in patients with discoid lupus.  Symptoms may include itching, pain, burning, and tenderness.

Diagnosis & Treatments
Detailed history, thorough physical examination of the scalp and scalp biopsies are critical in diagnosing DLE.

Early diagnosis and therapeutic intervention can often prevent permanent damage to existing hair follicles. Once a definitive diagnosis is made, we can determine the best treatment plan for each individual. Some regrowth is even possible with treatment.

If DLE is treated early, the hair loss is potentially reversible.  If not treated early, over half the patients will develop scarring alopecia.  The end stage of DLE is clinically distinct, just as the early phase is distinct, with striking hypo- and hyperpigmentation.  Treatment is case-dependent, based on severity of symptoms (itching and inflammation), findings on biopsy and extent of hair loss. Response to treatment varies from individual to individual.

In general, very mild disease may be managed by topical corticosteroid solutions or foams, topical calcineurin inhibitors (tacrolimus). However, in most cases, topical treatments alone are not sufficient.  The next step could be the addition of corticosteroid injections to the involved areas of the scalp, if the disease is localized to small areas.  For more diffuse or severe cases, oral anti-inflammatory medications such hydroxychloroquine (Plaquenil) or other anti-malarials, or methotrexate are necessary to control the inflammation and destructive process.  Rarely, immune suppressant drugs such as azathioprine (Imuran), mycophenolate mofetil (Cellcept) and cyclosporine are needed to achieve control.

Most forms of chronic cutaneous lupus erythematosus (including DLE) are triggered or exacerbated by exposure to UV sunlight.  Therefore, it is imperative to reduce or avoid sun exposure whenever possible, and to wear a broad-spectrum SPF 30+ sunblock if sun exposure cannot be avoided.

We encourage you to discuss these treatment options with your dermatologist.

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