Approach

The primary goals of treatment are to improve physical appearance, control existing lesions and limit scarring, and prevent the development of new lesions.

Lifestyle changes

Lifestyle changes should be discussed with patients.[12][15] Patients are advised to limit their exposure to the sun, cover up exposed areas of skin, and apply a broad-spectrum (both ultraviolet A and ultraviolet B) high-SPF sunscreen every 2 hours.[12][15][16] All patients who use long-term sun protection should consider vitamin D supplementation.[12][15][16]

Smoking cessation is highly recommended, as smoking is associated with more severe disease and decreases the efficacy of antimalarials.[7][12][15][17]

Cosmetic camouflage may be used to improve the appearance of lesions.

Nonsevere localized/limited disease

Localized/limited disease involves only the head and neck.

Topical corticosteroids are used as first-line treatment for patients with localized disease.[12][15][16]

Calcineurin inhibitors (e.g. pimecrolimus, tacrolimus) are recommended as an alternative first-line option if topical corticosteroids are contraindicated in patients with concomitant acne/rosacea.[12][15][16]

Initial treatment with a potent/very potent topical corticosteroid for severe disease is recommended for 4 weeks (stepped down once symptoms are controlled), or 12 weeks with a calcineurin inhibitor.[15] Topical corticosteroids of different potencies may then be used in combination depending on the patient's symptoms. Potent corticosteroids (e.g., betamethasone valerate 0.1%) and very potent corticosteroids (e.g., clobetasol propionate 0.05%) are often used to treat the trunk and limbs including the hands, as well as the scalp. Potent or very potent formulations can be considered for severe disease on the head and neck for short periods. Moderate-potency corticosteroids (e.g., triamcinolone acetonide 0.1%) are used in areas more prone to atrophy, such as the face and neck. Mild-potency corticosteroids (e.g., hydrocortisone 1%) are typically reserved for the eyelids. Scalp involvement may be treated with foam or lotion formulations.

The effectiveness of calcineurin inhibitors as an alternative treatment of cutaneous lupus erythematosus (CLE), including DLE, has been assessed in randomized trials with variable results. One vehicle-controlled trial of topical tacrolimus in patients with CLE (14 with DLE, 4 with subacute CLE) found that tacrolimus significantly improved skin lesions at 4 and 6 weeks, but not at 12 weeks, compared with vehicle.[18] However, when pimecrolimus was compared to betamethasone in a small randomized controlled trial, no significant difference was found in efficacy between the treatments for patients with DLE at 8 weeks. An 86% decrease in clinical severity score was seen for pimecrolimus, compared with a 73% decrease with betamethasone (P=0.043).[19] Small uncontrolled studies have shown topical tacrolimus or pimecrolimus to be effective alternatives in the treatment of cutaneous lupus, including DLE.[20][21][22]

Intralesional injection of a corticosteroid may be considered for individual lesions and is recommended for sites at higher risk of atrophy in patients with localized DLE, or as an adjunct treatment for persistent lesions.[12][15]

Disseminated disease or severe or refractory localized/limited disease

First-line therapy

For patients with disseminated disease or severe or refractory localized/limited DLE, an antimalarial drug (e.g., hydroxychloroquine or chloroquine), either as monotherapy or with adjunctive topical corticosteroids, is recommended as first-line treatment.[12][15][16] Adjunctive topical corticosteroid treatment may be stopped, or used as required, once the antimalarial is fully effective.

Chloroquine should only be considered as an option when hydroxychloroquine is ineffective or not tolerated.[12]

Patients with DLE are at a greater risk of scarring than patients with other subtypes of CLE; therefore, the following options should be considered for initial treatment:[12][15]

  • A higher dose of an antimalarial drug

  • Short-term concomitant use, or tapering courses, of systemic corticosteroids.

Patients who receive long-term oral corticosteroids (>3 weeks' duration), or those who require frequent courses (3-4 per year), should be monitored regularly to prevent corticosteroid-induced osteoporosis and adrenal insufficiency.[12]

Treatment with antimalarials is recommended for a period of 1-2 years to fully suppress cutaneous lupus activity.[23]

Second-line therapy

If antimalarials are ineffective, immunosuppressant therapy (e.g., methotrexate, mycophenolate) should be considered.[12][15][16][24][25][26]

Combination treatment with methotrexate or mycophenolate plus an antimalarial may be considered in patients with a partial response to an antimalarial plus topical therapy.[12]

A retinoid (e.g., acitretin) or dapsone may be considered as monotherapy (or in addition to an antimalarial for patients with refractory DLE).[12][15]

One randomized double-blind study comparing acitretin with hydroxychloroquine for the treatment of facial lesions in 58 patients with CLE reported similar rates of complete clearing or marked improvement in both groups at 8 weeks.[27] The rate of adverse effects was higher in the acitretin group, leading to discontinuation of treatment in 4 patients.

Evidence is limited for the treatment of DLE with dapsone. One small study reported that out of 11 patients with DLE, 9 patients showed improvement in skin disease at 16 weeks.[28] One retrospective review of 33 patients with DLE who received dapsone for 1-27 months found that treatment gave excellent results in 8 patients (24%), and some effect in 8 patients (24%), while no response was seen in 17 patients (52%).[29]

Consultation with a specialist should be sought before initiating immunosuppressant therapy.

Third-line therapy

Thalidomide should be considered as treatment for cutaneous lupus, including DLE, that has not responded to other treatments.[12][15][16][30][31]

Due to the high incidence of neurotoxicity associated with thalidomide, which does not appear to be dose dependent, it has been suggested that thalidomide should be used as a remission-inducing drug only for patients with severely refractory CLE or who are at high risk for severe scarring.[32][33] However, in clinical practice, low doses of thalidomide can be effective without significant risk of neurotoxicity.

Laser or surgical treatment

Pulsed dye laser treatment is generally not recommended but may be considered as an adjunct treatment for telangiectasia.[15][16]

A small study involving patients with active chronic DLE lesions concluded that pulsed dye laser treatment is an effective and safe therapy for patients with refractory chronic DLE.[34]

Surgical excision followed by skin graft may be considered for patients with refractory DLE with localized lesions in cosmetically unacceptable areas when topical and systemic treatments have failed or are not tolerated.[15] Burned-out scarred lesions may be excised surgically, although reactivation of inactive lesions following surgical excision has been reported.[35] Therefore, surgical intervention should be combined with medical treatment with antimalarials and/or systemic corticosteroids.[15]

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