Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ONGOING

non-severe localised/limited disease

1st line – topical treatment

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ONGOING
|
non-severe localised/limited disease
1st line – 

topical treatment

Localised/limited disease involves only the head and neck.

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

Initial treatment with a potent/very potent topical corticosteroid 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% or betamethasone valerate 0.025%) 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.

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]

The effectiveness of calcineurin inhibitors as an alternative treatment of cutaneous lupus erythematosus (CLE), including DLE, has been assessed in randomised 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 randomised 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]

Primary options

hydrocortisone topical: (1%) apply to the affected area(s) once or twice daily

More

OR

triamcinolone topical: (0.1%) apply to the affected area(s) once or twice daily

More

OR

betamethasone valerate topical: (0.025%) apply to the affected area(s) once or twice daily

More

OR

betamethasone valerate topical: (0.1%) apply to the affected area(s) once or twice daily

More

OR

clobetasol topical: (0.05%) apply to the affected area(s) twice daily

More

OR

tacrolimus topical: (0.1%) apply to the affected area(s) twice daily

OR

pimecrolimus topical: (1%) apply to the affected area(s) twice daily

Plus – lifestyle changes

Back
ONGOING
|
non-severe localised/limited disease
Plus – 

lifestyle changes

Treatment recommended for ALL patients in selected patient group

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.

Consider – intralesional corticosteroid injection

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ONGOING
|
non-severe localised/limited disease
Consider – 

intralesional corticosteroid injection

Additional treatment recommended for SOME patients in selected patient group

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

Primary options

triamcinolone acetonide: consult specialist for guidance on intralesional dose

Consider – pulsed dye laser

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ONGOING
|
non-severe localised/limited disease
Consider – 

pulsed dye laser

Additional treatment recommended for SOME patients in selected patient group

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]

severe or refractory localised/limited disease; disseminated disease

1st line – antimalarial

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ONGOING
|
severe or refractory localised/limited disease; disseminated disease
1st line – 

antimalarial

For patients with disseminated disease or severe or refractory localised/limited DLE, an antimalarial drug (e.g., hydroxychloroquine, chloroquine), either as monotherapy or with adjunctive topical corticosteroids, is recommended as first-line treatment.[12][15][16]

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, a higher dose of an antimalarial should be considered for initial treatment.[12][15]

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

Hydroxychloroquine and chloroquine are associated with retinal toxicity. Regular screening for retinal changes is required according to local guidelines. Maximum doses of <5 mg/kg/day (hydroxychloroquine) or <2.3 mg/kg/day (chloroquine) are recommended (based on real body weight) to reduce the risk of retinal toxicity.[16]

Primary options

hydroxychloroquine: 200-400 mg/day orally given in 1-2 divided doses, maximum 5 mg/kg/day

Secondary options

chloroquine phosphate: consult specialist for guidance on dose

Plus – lifestyle changes

Back
ONGOING
|
severe or refractory localised/limited disease; disseminated disease
Plus – 

lifestyle changes

Treatment recommended for ALL patients in selected patient group

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.

Consider – topical corticosteroid

Back
ONGOING
|
severe or refractory localised/limited disease; disseminated disease
Consider – 

topical corticosteroid

Additional treatment recommended for SOME patients in selected patient group

May be used with initial antimalarial treatment. Adjunctive topical corticosteroid treatment may be stopped, or used as required, once the antimalarial is fully effective.

Topical corticosteroids of different potencies may 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% or betamethasone valerate 0.025%) 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.

Primary options

hydrocortisone topical: (1%) apply to the affected area(s) once or twice daily

More

OR

triamcinolone topical: (0.1%) apply to the affected area(s) once or twice daily

More

OR

betamethasone valerate topical: (0.025%) apply to the affected area(s) once or twice daily

More

OR

betamethasone valerate topical: (0.1%) apply to the affected area(s) once or twice daily

More

OR

clobetasol topical: (0.05%) apply to the affected area(s) twice daily

More

Consider – systemic corticosteroid

Back
ONGOING
|
severe or refractory localised/limited disease; disseminated disease
Consider – 

systemic corticosteroid

Additional treatment recommended for SOME patients in selected patient group

Patients with DLE are at a greater risk of scarring than patients with other subtypes of CLE; therefore, a short-term concomitant use, or tapering courses, of a systemic corticosteroid (e.g., intravenous methylprednisolone or oral prednisolone) should be considered for initial treatment.[12][15]

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]

Primary options

methylprednisolone sodium succinate: 1 g intravenously once daily for 3-5 days

OR

prednisolone: 0.5 to 1.5 mg/kg/day orally for 2-4 weeks, then taper gradually

2nd line – immunosuppressant therapy

Back
ONGOING
|
severe or refractory localised/limited disease; disseminated disease
2nd line – 

immunosuppressant therapy

If antimalarials are ineffective or not tolerated, immunosuppressant monotherapy (e.g., methotrexate, mycophenolate) should be considered.[12][15][16][24][25][26]

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

One randomised 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.

Primary options

methotrexate: 7.5 mg orally once weekly on the same day each week initially, increase by 2.5 mg/week increments at monthly intervals according to response, maximum 20 mg/week

OR

mycophenolate mofetil: 0.25 to 1 g orally twice daily

OR

acitretin: consult specialist for guidance on dose

OR

dapsone: consult specialist for guidance on dose

Plus – lifestyle changes

Back
ONGOING
|
severe or refractory localised/limited disease; disseminated disease
Plus – 

lifestyle changes

Treatment recommended for ALL patients in selected patient group

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.

Consider – antimalarial

Back
ONGOING
|
severe or refractory localised/limited disease; disseminated disease
Consider – 

antimalarial

Additional treatment recommended for SOME patients in selected patient group

Combination treatment with an antimalarial plus methotrexate or mycophenolate may be considered in patients with a partial response to antimalarials and topical therapy.[12] An antimalarial may also be considered in combination with a retinoid (e.g., acitretin) or dapsone for patients with refractory DLE.[12][15]

Hydroxychloroquine and chloroquine are associated with retinal toxicity. Regular screening for retinal changes is required according to local guidelines. Maximum doses of <5 mg/kg/day (hydroxychloroquine) or <2.3 mg/kg/day (chloroquine) are recommended (based on real body weight) to reduce the risk of retinal toxicity.[16]

Primary options

hydroxychloroquine: 200-400 mg/day orally given in 1-2 divided doses, maximum 5 mg/kg/day

Secondary options

chloroquine phosphate: consult specialist for guidance on dose

3rd line – thalidomide

Back
ONGOING
|
severe or refractory localised/limited disease; disseminated disease
3rd line – 

thalidomide

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 seem 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.

Primary options

thalidomide: consult specialist for guidance on dose

Plus – lifestyle changes

Back
ONGOING
|
severe or refractory localised/limited disease; disseminated disease
Plus – 

lifestyle changes

Treatment recommended for ALL patients in selected patient group

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.

4th line – surgical excision

Back
ONGOING
|
severe or refractory localised/limited disease; disseminated disease
4th line – 

surgical excision

Surgical excision followed by skin graft may be considered for patients with refractory DLE with localised 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]

Plus – lifestyle changes

Back
ONGOING
|
severe or refractory localised/limited disease; disseminated disease
Plus – 

lifestyle changes

Treatment recommended for ALL patients in selected patient group

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.

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Choose a patient group to see our recommendations

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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