Acute Cutaneous Lupus Erythematosus (ACLE) Medication

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

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

Adjuvant therapy for systemic disease can be provided with methotrexate, azathioprine, cyclophosphamide, and thalidomide. Hydroxychloroquine, administered as first-line therapy to most patients with systemic disease, has particularly beneficial effects on skin lesions. Intravenous IgG has become important in controlling recalcitrant disease. [25]



Class Summary

Corticosteroids have anti-inflammatory properties and cause profound and varied metabolic effects. These agents modify the body's immune response to diverse stimuli.


Prednisone is a glucocorticoid (adrenocortical steroid) that is absorbed easily into the gastrointestinal (GI) tract. An immunosuppressant, it is used for the treatment of autoimmune disorders. Prednisone may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear (PMN)-cell activity. It stabilizes lysosomal membranes and also suppresses lymphocytes and antibody production.



Class Summary

These agents are used for immunosuppression and, ultimately, disease control.

Azathioprine (Imuran, Azasan)

Azathioprine antagonizes purine metabolism and inhibits the synthesis of DNA, ribonucleic acid (RNA), and proteins. It may decrease the proliferation of immune cells, in that way lowering autoimmune activity. For dermatomyositis/polymyositis, respiratory and muscular symptoms respond, but skin lesion response has not been consistent.

Azathioprine is slow acting, with its therapeutic effect not being seen for 6-8 weeks. Metabolites accumulate slowly, and maximal immunosuppression is not reached until after 8-12 weeks. The drug is available in 25-, 50-, 75-, and 100-mg tablets or in a 100-mg vial.


Cyclophosphamide is chemically related to nitrogen mustards. It is an alkylating agent, and the mechanism of action of its active metabolites may involve cross-linking of DNA, which may interfere with the growth of normal and neoplastic cells.

Thalidomide (Thalomid)

Thalidomide is an immunomodulatory agent that may suppress the excessive production of tumor necrosis factor-alpha and may down-regulate selected cell-surface adhesion molecules involved in leukocyte migration. In patients weighing less than 50 kg (110 lb), start at low end of dose regimen.

Hydroxychloroquine (Plaquenil)

This agent inhibits chemotaxis of eosinophils and the locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions. Hydroxychloroquine sulfate 200 mg is equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate.

Immune globulin IV (Gammagard, Gamunex, Octagam, Gammaplex )

Immunoglobulin neutralizes circulating myelin antibodies through anti-idiotypic antibodies; down-regulates proinflammatory cytokines, including interferon gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; promotes remyelination; and may increase cerebrospinal fluid IgG (10%).

Methotrexate (Otrexup, Rasuvo, Trexall)

Methotrexate was introduced in 1965. It is considered second-line therapy, especially in acute cutaneous lupus erythematosus (ACLE) and chronic cutaneous lupus erythematosus (CCLE). It is also used in lesions refractory to antimalarials and as a corticosteroid-sparing agent. Its mechanism of action includes action on adenosine, which is a purine nucleoside and has potent anti-inflammatory effects. It induces apoptosis in CD4+Tcells.

Dose ranges from 7.5-25 mg once per week orally, intravenously, or subcutaneously. Adverse effects include GI complaints, which can be alleviated with administration of folic acid prior to or after methotrexate administration. Hepatotoxicity, nephrotoxicity, and bone marrow suppression are other known adverse effects.


Monoclonal Antibody

Class Summary

These drugs restore the potential to minimize self-immunity. Monoclonal antibodies can induce cytotoxicity after binding to specific antigens that may regulate cell cycle initiation. This then results in the inhibition of cell growth and differentiation.

Rituximab (Rituxan)

Rituximab is a murine/human chimeric anti-CD20 monoclonal antibody. CD20 is expressed early in pre ̶ B cell development. Binding induces complement-dependent B-cell cytotoxicity along with antibody-dependent cellular toxicity. Rituximab is a murine/human chimeric anti-CD20 monoclonal antibody, US FDA approved for the treatment of refractory low-grade or follicular non-Hodgkin lymphoma and severe rheumatoid arthritis. It is available as an injectable solution of 10 mg/mL.

Belimumab (Benlysta)

Belimumab is a human recombinant IgG1-gamma monoclonal antibody that is used in the treatment of systemic lupus erythematosus (SLE). The monoclonal antibody specifically targets B lymphocytes stimulator (BLyS), thereby inhibiting autoimmune B cells to live longer. Belimumab was approved by the FDA for use in SLE in 2011 and is given as an intravenous administration of 10 mg/kg every 2 weeks for three doses, followed by the same dose every 4 weeks. This drug was approved for use in lupus nephritis in 2020.



Lenalidomide (Revlimid)

Lenalidomide is an immunomodulatory drug with potent antineoplastic, antiangiogenic, and anti-inflammatory properties. It is an analogue of thalidomide; however, it is a much safer drug with fewer adverse effects. Lenalidomide is preferably used in the treatment of refractory cutaneous lupus erythematosus. Lenalidomide is approved by the FDA for the treatment of several conditions, including multiple myeloma, myelodysplastic syndromes, mantle cell lymphoma, follicular lymphoma, and marginal zone lymphoma.