Manifestations and Treatment of Cutaneous Lupus Erythematosus
(Part II of II)
See Part I in Volume 10 Issue 10 (October 2011) of JDD.
Treatment
All patients with CLE should be counseled on proper sun protection and smoking cessation. Treatment of local disease should start with a topical corticosteroid or calcineurin inhibitors. Severe or widespread disease may warrant short-term use of systemic corticosteroids and initiation of an antimalarial. In recalcitrant disease, methotrexate, retinoids, dapsone, thalidomide, mycophenolate mofetil, or a biologic therapy can be considered.24 In general, these therapies are effective for all forms of CLE, with a tendency for DLE to be the least responsive and SCLE to be most responsive to treatment. Laser therapy is an emerging option for treating active and quiescent lesions.
All patients with CLE should be counseled on proper sun protection and smoking cessation. Treatment of local disease should start with a topical corticosteroid or calcineurin inhibitors. Severe or widespread disease may warrant short-term use of systemic corticosteroids and initiation of an antimalarial. In recalcitrant disease, methotrexate, retinoids, dapsone, thalidomide, mycophenolate mofetil, or a biologic therapy can be considered.24 In general, these therapies are effective for all forms of CLE, with a tendency for DLE to be the least responsive and SCLE to be most responsive to treatment. Laser therapy is an emerging option for treating active and quiescent lesions.
Ultraviolet Protection
CLE lesions can be induced or exacerbated by ultraviolet (UV) A or B light.25 Effective UV protection comes from the use of broad spectrum sunscreens and UV protective clothing, as well as from limiting activity in the midday sun.
CLE lesions can be induced or exacerbated by ultraviolet (UV) A or B light.25 Effective UV protection comes from the use of broad spectrum sunscreens and UV protective clothing, as well as from limiting activity in the midday sun.
Avoidance of the sun may adversely affect vitamin D levels. Even in summer months, 85 percent of CLE patients have vitamin D deficiency on laboratory examination.26 The role of vitamin D supplementation in CLE has yet to be defined.
Smoking Cessation
Cigarette smoking is more prevalent in patients with CLE27,28 and SLE28 than in the general population. Furthermore, smoking is associated with poor response to antimalarial drugs.29 Although the exact role of smoking is unknown, patients with LE derive benefit from smoking cessation.
Cigarette smoking is more prevalent in patients with CLE27,28 and SLE28 than in the general population. Furthermore, smoking is associated with poor response to antimalarial drugs.29 Although the exact role of smoking is unknown, patients with LE derive benefit from smoking cessation.
Topical Therapy
Topical steroids are an effective treatment for all subtypes of CLE in a potency-dependent manner.30 Use is limited by side effects. However, in DLE, where scarring and atrophy are part of the natural history of the lesion, the side effects of topical steroids carry less weight. Ointments are preferable to creams because they are more hydrating and contain fewer potential irritants.31 Intralesional injections are effective in 80 percent of cases but should not be used more frequently than every 4-6 weeks.5
Topical steroids are an effective treatment for all subtypes of CLE in a potency-dependent manner.30 Use is limited by side effects. However, in DLE, where scarring and atrophy are part of the natural history of the lesion, the side effects of topical steroids carry less weight. Ointments are preferable to creams because they are more hydrating and contain fewer potential irritants.31 Intralesional injections are effective in 80 percent of cases but should not be used more frequently than every 4-6 weeks.5
Topical application of calcineurin inhibitors (tacrolimus ointment 0.1% and pimecrolimus cream 1%) has been shown to be as efficacious
as topical steroids in small case studies,24,32-34 LET lesions being the most responsive and DLE the least. However, a recent RCT of 30 subjects with steroid-resistant CLE showed that 0.1% tacrolimus accelerated lesion improvement but did not demonstrate
significant long-term effects.35 The appeal of calcineurin inhibitors is the decreased frequency of side effects compared to topical steroids.24 Nonetheless, until more evidence is available to support the use of calcineurin inhibitors in lieu of steroids, they will remain second line topical treatment for CLE.
In addition to steroids and calcineurin inhibitors, R-salbutamol, retinoids, and imiquimod have been used topically for CLE. Topical
R-salbutamol (ASF-1096), a β2-receptor agonist, was shown in a phase II trial to be both effective and safe for the treatment of DLE. Unfortunately, no further trials are currently listed on ClincialTrials.
gov. Both topical retinoids36,37 and imiquimod38,39 have shown beneficial effects on CLE in case reports. Oral retinoid therapy is discussed below.
Systemic Therapy
Antimalarials
Hydroxycloroquine, chloroquine, and quinacrine were developed as antimalarial drugs. However, they have also been used in the treatment of inflammatory processes such as SLE, CLE, and sarcoidosis because of their anti-inflammatory properties. Antimalarials accumulate in lysosomes and inhibit lysosomal proteases in the treatment of malaria, and these drugs also inhibit protein MHC interactions in the lysosome. The end result is a decrease in the presentation of self antigens and a decrease in inflammatory mediators.40
Antimalarials
Hydroxycloroquine, chloroquine, and quinacrine were developed as antimalarial drugs. However, they have also been used in the treatment of inflammatory processes such as SLE, CLE, and sarcoidosis because of their anti-inflammatory properties. Antimalarials accumulate in lysosomes and inhibit lysosomal proteases in the treatment of malaria, and these drugs also inhibit protein MHC interactions in the lysosome. The end result is a decrease in the presentation of self antigens and a decrease in inflammatory mediators.40
The response rate to antimalarials for the treatment of all subtypes
of CLE is 50-90% as demonstrated by observational trials.30 In a randomized control trial, hydroxychloroquine showed similar efficacy to acitretin but had fewer side effects.41 The use of chloroquine
and hydroxychloroquine is limited by its adverse effects of retinopathy. For the most part, retinopathy can be avoided by keeping daily doses of chloroquine below 3.5 mg/kg of ideal body weight and hydroxychloroquine below 5 mg/kg of ideal body
