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Acute Cutaneous Lupus Erythematosus (ACLE) Treatment & Management

  • Author: Ivan D Camacho, MD; Chief Editor: William D James, MD  more...
 
Updated: Nov 12, 2015
 

Approach Considerations

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

Additional immunosuppressive agents, such as azathioprine, cyclophosphamide, and thalidomide, are used as adjuvant therapy to treat systemic disease because of steroid-sparing effects.[10, 11]

Hydroxychloroquine 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.

Intravenous IgG and rituximab have been used in controlling recalcitrant disease.[12, 13, 14, 15, 16]

Mycophenolate mofetil has shown poor results in disease refractory to multiple treatment modalities.[17]

One case reports describes the effectiveness of plasmapheresis for refractory TEN-like acute cutaneous lupus erythematosus.[18]

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.[19]

Recently, Díez et al described the positive clinical, histopathologic, and immunohistolgic effect of pulsed dye laser on cutaneous lupus erythematosus lesions, with clinical improvement in 8 of 9 patients (88.9%), reduction of the dermal lymphocytic infiltrate, basal layer damage, mucin deposition, and intracellular adhesion molecule (ICAM) and vascular cell adhesion molecule (VCAM) expression.[20]

Photoprotection

Cutaneous lupus erythematosus lesions may be induced and exacerbated by ultraviolet radiation exposure, and patients with cutaneous manifestations of lupus erythematousus are generally considered photosensitive. Patients with acute cutaneous lupus erythematous require extensive education about avoidance of sun exposure, photoprotection through physical barriers such as protective clothing, and daily application of broad-spectrum sunscreens.[21]

Diet and activity

Dietary restrictions may be necessary in the presence of renal compromise. In terms of activity, advise patients with acute cutaneous lupus erythematosus to avoid activities involving excessive exposure to the sun.

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

An investigational drug, belimumab, is currently undergoing phase III clinical trials for the potential treatment of systemic lupus erythematosus. It is a neutralizing B-lymphocyte stimulator monoclonal antibody that inhibits the biologic activity of the soluble form of essential B cells.[22]

Doses used in the phase II 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 (ANA ≥1:80 or anti-dsDNA >30 IU) and the physician global assessment score.

Epratuzumab is also investigational. It is a humanized anti-CD22 monoclonal antibody that partially depletes B cells. Treatment is shown to decrease disease activity but not autoantibody levels in patients with moderately active systemic lupus erythematosus.

In an open-label, single-case study of 14 patients with systemic lupus erythematosus, patients received intravenous epratuzumab at 360 mg/m2 every 2 weeks for 4 doses, with analgesic/antihistamine premedication prior to each dose. Total British Isle Lupus Assessment Group (BILAG) scores were decreased by greater than or equal to 50% in all 14 patients at 6 weeks.

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Contributor Information and Disclosures
Author

Ivan D Camacho, MD Dermatologist, Private Practice; Voluntary Assistant Professor of Dermatology, Department of Dermatology and Cutaneous Surgery, University of Miami, Leonard M Miller School of Medicine

Ivan D Camacho, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Florida Medical Association, International Society of Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

References
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Relationship of acute cutaneous lupus erythematosus (ACLE) to systemic disease. LE is lupus erythematosus. CCLE is chronic cutaneous lupus erythematosus. SCLE is subacute cutaneous lupus erythematosus.
Erythema involving the malar area, forehead, and neck. Note sparing of some of the creases.
Toxic epidermal necrolysis–like eruption.
 
 
 
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