Acute Cutaneous Lupus Erythematosus (ACLE) Treatment & Management

Updated: Jul 20, 2021
  • Author: Fnu Nutan, MD, FACP; Chief Editor: William D James, MD  more...
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Treatment

Approach Considerations

Cutaneous lupus erythematosus therapy is aimed at preventing recurrences and scarring of the skin. Local treatments using topical steroids or calcineurin inhibitors can be used for mild cases.

Systemic corticosteroids usually are the mainstay of therapy for systemic disease. Skin changes tend to respond in tandem with the systemic response to treatment.

Additional immunosuppressive agents, such as methotrexate, azathioprine, cyclophosphamide, and thalidomide, are used as adjuvant therapy to treat systemic disease because of steroid-sparing effects. [22, 23]

Hydroxychloroquine (antimalarial) also has been shown to have steroid-sparing effects and is administered as first-line therapy to most patients with systemic disease. The effects of hydroxychloroquine on skin lesions are especially beneficial. A 2017 meta-analysis found that antimalarials were 2.5 times more effective in lesions of acute cutaneous lupus erythematosus (ACLE) compared with other lupus cutaneous skin lesion types. [24] However, an inherent bias could be that patients with ACLE are on other systemic treatments for the associated systemic lupus erythematosus (SLE).

Intravenous IgG (IVIG) and rituximab have been used in controlling recalcitrant disease. [25, 26, 27, 28, 29]

Mycophenolate mofetil has shown poor results in disease refractory to multiple treatment modalities. [30]

One case reports describes the effectiveness of plasmapheresis for refractory toxic epidermal necrolysis (TEN)–like ACLE. [31]

Topical tacrolimus 0.1% ointment has shown to provide at least temporary benefit, especially in acute, edematous, nonhyperkeratotic lesions of cutaneous lupus erythematosus, in a small multicenter, randomized, double-blind, vehicle-controlled trial. [32]

In 2011, Díez et al described the positive clinical, histopathologic, and immunohistological effect of pulsed-dye laser on cutaneous lupus erythematosus lesions, with clinical improvement in 8 (88.9%) of 9 patients, reduction of the dermal lymphocytic infiltrate, basal layer damage, mucin deposition, and intracellular adhesion molecule (ICAM) and vascular cell adhesion molecule (VCAM) expression. [33]

In 2017, transplantation of in vivo–harvested epidermal cell suspension was reported to successfully treat depigmentation associated with malar rash. [34]

Belimumab is a neutralizing B-lymphocyte stimulator monoclonal antibody that inhibits the biologic activity of the soluble form of essential B cells. [35] Doses used in the phase 2 trials were randomized to 1, 4, or 10 mg/kg intravenously on days 1, 14, and 28, and then every 28 days for 76 weeks. The treatment significantly improved the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores in seropositive patients (antinuclear antibody [ANA] ≥1:80 or anti-dsDNA >30 IU) and the physician global assessment score. Currently, belimumab is the only biological agent approved for the treatment of adults and pediatric patients with SLE and has been shown to reduce autoantibody levels in people with SLE and to help control disease activity. [36]

Lenalidomide (analogue of thalidomide) is an immunomodulatory drug with antineoplastic, anti-inflammatory, and antiangiogenic properties and is approved for use in many cancers. However, in recent years, multiple small, open-label trials have shown lenalidomide to be both safe and efficacious in the treatment of cutaneous lupus erythematosus. [37]

Photoprotection

Cutaneous lupus erythematosus lesions may be induced and exacerbated by ultraviolet radiation exposure, and patients with cutaneous manifestations of lupus erythematosus are generally considered photosensitive. Patients with ACLE require extensive education about avoidance of sun exposure, photoprotection through physical barriers such as protective clothing, and daily application of broad-spectrum sunscreens. [38]

Diet and activity

Dietary restrictions may be necessary in the presence of renal compromise. In terms of activity, advise patients with ACLE to avoid activities involving excessive exposure to the sun. Advice to stop smoking is essential.

Consultations

Refer patients with clinical and serologic evidence of lupus erythematosus to a rheumatologist for further treatment. Refer patients with red blood cell casts, significant proteinuria (>0.5 g/mL/24h), and a diastolic blood pressure of more than 90 mm Hg to a nephrologist.

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Precautions in Corticosteroid Use

When administering systemic corticosteroids, address adverse effects such as diabetes mellitus, hypertension, osteonecrosis, the stigmata of Cushing syndrome, and the risk of osteoporosis.

Perform a baseline bone densitometry scan, and, if normal, repeat the scan at 6 months. If osteoporosis is present, refer the patient to an osteoporosis specialist for consideration of treatment with bisphosphonates.

Ideally, perform 24-hour urine collection to check calcium levels, since steroids enhance renal excretion of calcium, thereby increasing the patient's susceptibility to developing renal stones. If the results are normal, administer cholecalciferol (400-800 IU/d) and calcium (1500 mg/d). If evidence of hypercalciuria is present, administer thiazide diuretics until levels return to normal.

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Investigational Drugs

Epratuzumab is an investigational drug. It is a humanized anti-CD22 monoclonal antibody that partially depletes B cells. Treatment has been shown to decrease disease activity but not autoantibody levels in patients with moderately active SLE. In an open-label, single-case study of 14 patients with SLE, patients received intravenous epratuzumab at 360 mg/m2 every 2 weeks for four doses, with analgesic/antihistamine premedication prior to each dose. Total British Isle Lupus Assessment Group (BILAG) scores were decreased by 50% or more in all 14 patients at 6 weeks. A meta-analysis of several randomized controlled trials using epratuzumab has shown that it may be an effective option for the treatment of moderately to severely active SLE. [39]

Ustekinumab is a human monoclonal antibody that binds to interleukins 12 and 23, thereby preventing the activation of TH17 cells. It has been approved for the treatment of psoriasis and is also used for ACLE, hypertrophic cutaneous lupus, and chronic cutaneous lupus erythematosus (CCLE). [40] However, a phase 3 LOTUS study in SLE was discontinued because of a to lack of efficacy in SLE.

Anifrolumab, a human monoclonal antibody to the type I interferon receptor subunit 1 was previously investigated for the treatment of SLE. Although the clinical trial investigating anifrolumab failed to meet its primary endpoint in a phase 3 trial, a pooled analysis of phase 2 and 3 trials has shown efficacy of anifrolumab in adults with moderate-to-severe SLE. Monthly intravenous anifrolumab at 300 mg is generally well tolerated over 52 weeks, with an acceptable safety profile. [41]

Baricitinib is an oral selective inhibitor of the Janus kinase (JAK) 1 and JAK2 and is a new potential oral therapy for SLE. Baricitinib is currently under phase 3 study investigation to evaluate long-term safety and efficacy. The 4-mg dose of baricitinib significantly improved the signs and symptoms of active SLE. [42] However, further study is needed to evaluate its efficacy in cutaneous lupus erythematosus disease.

New immunomodulating drugs such as iberdomide, a derivative of lenalidomide, is currently under phase 2 clinical trial for the treatment of both SLE and cutaneous lupus erythematosus (A Pilot Study of CC-220 to Treat Systemic Lupus Erythematosus).

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