Acute cutaneous lupus erythematosus (ACLE) is the most common form of cutaneous lesions of lupus associated with systemic lupus erythematosus (SLE).[1] It can predict the recurrence of systemic disease or prognosis of the disease in some cases.
Serologic investigations are indicated, along with the clinical picture, to confirm the diagnosis. Sun protection, smoking cessation, and topical therapy are first-line treatment, followed by oral systemic therapy. Additionally, therapies used to treat the systemic disease help in controlling ACLE lesions. Per the Systemic Lupus International Collaborating Clinics (SLICC) group, 4 of the 11 clinical criteria used for SLE classification are mucocutaneous in nature and include ACLE (also including subacute cutaneous lupus erythematosus [SCLE]), chronic cutaneous lupus erythematosus (CCLE), oral or nasal ulcers, and nonscarring alopecia.[2]
Lupus erythematosus is a heterogeneous connective-tissue disease associated with polyclonal B-cell activation and is believed to result from the interplay of genetic, environmental, and hormonal factors. Studies show that B-cell–activating factor (BAFF) is a member of the tumor necrosis receptor superfamily (TNRSF) involved in many immune and inflammatory processes, including SLE.[3] Other immune cells, including T cells, neutrophils, and plasmacytoid dendritic cells, have also been shown to play a role in the disease process. The spectrum of disease involvement can vary from limited cutaneous involvement to devastating systemic disease.
From a dermatologic standpoint, the type of skin involvement can prove to be a good barometer of the pattern of underlying systemic activity. Lupus erythematosus–specific skin diseases are classified into three subsets according to Gilliam and Sontheimer's classification: (1) ACLE, (2) SCLE, and (3) CCLE. See the diagram below. Over the years, intermittent cutaneous lupus erythematosus (ICLE), also known as lupus tumidus, has been introduced as a distinct entity and is included in the Dusseldorf classification by Kuhn and Landmann.[4] Clinical characteristics of each group are unique, although histopathologically, only subtle differences are identified. The major clinical variants of ACLE include localized ACLE, generalized ACLE, and toxic epidermal necrolysis (TEN)–like ACLE. The focus of this article is on ACLE.[5, 6]
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Relationship of acute cutaneous lupus erythematosus (ACLE) to systemic disease. LE is lupus erythematosus. CCLE is chronic cutaneous lupus erythematos....
Patients with the localized ACLE present with the classic malar eruption in a "butterfly" pattern localized to the central portion of the face. (See the image below.) Less commonly, patients may present with a generalized maculopapular eruption in photosensitive areas. Rarely, patients may present with TEN-like ACLE featuring vesiculobullous lesions with epidermal sloughing commonly on sun-exposed sites and may involve one or more mucous membranes.[7]
ACLE has a strong association with the systemic disease for which patients present to rheumatologists and internists. Of patients with a new diagnosis of cutaneous lupus erythematosus, 24% have already been diagnosed with SLE and about 18% subsequently are diagnosed with SLE.[8]
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Erythema involving the malar area, forehead, and neck. Note sparing of some of the creases.
Localized ACLE can be transient, lasting for several days to weeks. Lesions wax and wane with sun exposure over a period of several hours; however, some patients experience prolonged disease activity.
Generalized ACLE may present with maculopapular erythema of the hands, specifically over the interphalangeal joints. However, this should be distinguished from Gottron papules of dermatomyositis, which present with an erythematous rash of the metacarpophalangeal joints or knuckles.
TEN-like ACLE presents similarly to Stevens-Johnson syndrome/TEN (SJS/TEN), which is a severe mucocutaneous skin disease associated with high mortality. SJS/TEN is often triggered by use of certain medications, and, therefore, a careful medication history, clinical evaluation, laboratory investigation, and pathological investigation must be performed to distinguish between the two.
Resolution of lesions in ACLE may result in postinflammatory hyperpigmentation, especially in patients with darkly pigmented skin. Usually, the lesions are nonscarring.
The etiology of lupus erythematosus is believed to be multifactorial, involving genetic, environmental, and hormonal factors. An association with human leukocyte antigen DR2 and human leukocyte antigen DR3 has been identified. Concordance in monozygotic twins and familial associations support a genetic basis in acute cutaneous lupus erythematosus.
More than 25 genes have been identified as contributing to the mechanisms that predispose patients to lupus. They include alleles in the major histocompatibility complex region (multiple genes): IRF5, ITGAM, STAT4, BLK, BANK1, PDCD1, PTPN22, TNFSF4, TNFA1P3, SPP1, some fc gene receptors, and deficiency in several complement components, including C1qC4+C2.
In patients who are predisposed genetically, exposure to natural ultraviolet radiation is a frequent precipitating factor for lupus erythematosus. In addition, certain viruses (eg, Epstein-Barr virus, cytomegalovirus, human immunodeficiency virus [HIV]) have been implicated in precipitating or exacerbating lupus erythematosus in genetically predisposed individuals.
Smoking has been associated with lesions of cutaneous lupus erythematosus, and continued smoking is associated with more severe disease and decreased response to medications. Alcohol consumption has not been reported to affect cutaneous lupus erythematosus. Sex hormones are thought to play a role, as in other female-predominant autoimmune diseases.[9]
Chemicals such as L-canavanine, which is present in alfalfa sprouts, have been known to induce SLE-like illness. Drugs implicated in inducing a lupus erythematosus–like illness (eg, procainamide, isoniazid, hydralazine) typically do not induce ACLE.
Studies have found that microbes can trigger cutaneous lupus erythematosus. Staphylococcus aureus is a common skin pathogen that can colonize the skin following interferon-mediated barrier disruption and is associated with cutaneous lupus erythematosus skin lesions.[10]
See also Bullous Lupus Erythematosus, Discoid Lupus Erythematosus, Drug-Induced Lupus Erythematosus, and Subacute Cutaneous Lupus Erythematosus.
Immunopathology
Data concerning direct immunofluorescence in ACLE are sparse. In one study, the results of 5 (100%) of 5 skin biopsy specimens were reported as positive for the lupus band test. The lupus band test reveals the presence of immunoglobulins and C3 complement components along the dermoepidermal junction. All three immunoglobulin classes (IgG, IgM, IgA) and a variety of complement components have been identified at the dermoepidermal junction.
Research has shown that 60% of patients with a malar eruption of lupus erythematosus have positive lupus band test results. In nonlesional skin, positive lupus band test results correlate strongly with an aggressive course of systemic disease.
Histology
Localized and generalized ACLE show histological evidence of interface dermatitis with basal-layer vacuolization with superficial perivascular lymphocytic infiltrate in the upper half to the deep dermis and mucin deposits in the reticular dermis.[11] TEN-like ACLE presents histologically with epidermal necrosis with basal vacuolization and necrotic keratinocytes with sparse lymphohistiocytic infiltrate.[12]
In the United States, the malar rash has been reported in 20-60% of patients in large lupus erythematosus cohorts, while limited data suggest that the maculopapular eruption is present in 35% of patients with SLE. The malar rash is believed to be associated with a younger age of disease onset. The incidence of cutaneous lupus erythematosus in Sweden and the United States has been estimated at 4 cases per 100,000 inhabitants.[13]
Race-related demographics
Precise data concerning the prevalence of ACLE in specific racial groups are not available; however, since photosensitivity is observed more frequently in White persons than in Black persons, the same prevalence for ACLE may be inferred. Estimates suggest that 1 in 250 Black women in the United States and the Caribbean and 1 in 1000 Chinese persons have SLE.
Although lupus erythematosus may be rare in most parts of Africa, data concerning this finding conflict. A retrospective study in South Africa found that ACLE is more common in non-Black patients with SLE than in Black patients.[14]
Data concerning ACLE are difficult to interpret, since a lack of conformity is found in the description of lesions and biopsy data are lacking for skin lesions observed in patients with systemic disease.
Significant morbidity and potential mortality are associated with SLE, of which ACLE is a manifestation.
The localized malar eruption tends to wax and wane with systemic activity; however, whether the presence of malar rash indicates a worse overall outlook for patients has not yet been determined.
No definite correlation has been identified between ACLE and nephritis; however, localized lesions of ACLE are believed to tend to wax and wane, paralleling the underlying systemic disease. Postinflammatory hyperpigmentation may occur in dark-skinned patients following resolution.
Women with SLE who are pregnant and have the Ro/La antibodies should be advised on the risk of developing a fetus with neonatal lupus erythematosus.
Complications
Unlike discoid lupus lesions, lesions of ACLE do not scar with healing. Transient hyperpigmentation is seen during the healing phase. Oral lesions heal without scarring. Very rarely, hypopigmentation can be seen after healing of the malar rash.
Acute cutaneous lupus erythematosus (ACLE) can be classified into the following three categories:
Localized ACLE
Generalized ACLE
Toxic epidermal necrolysis (TEN)–like ACLE
Primary lesions in ACLE therefore include the classic facial malar rash, confluent erythema and edema, erythematous macules and papules that eventually become confluent, morbilliform macules and papules in a generalized photo-distributed pattern, bullous lesions resembling TEN, and erythema multiforme–like lesions (Rowell syndrome).[15]
The facial rash of ACLE includes the malar area and the cheeks and always spares the nasolabial folds. Other sites of involvement include the forehead, periorbital area, and sides of the neck, essentially all the areas that are exposed to the sun.
Generalized ACLE, although less common, presents as a erythematous morbilliform rash on exposed parts of the body, such as the extensor surfaces of the hand. Classically, it spares the knuckles.
Sometimes, vesicles and bullae on erythematous skin resembling Stevens-Johnson syndrome (SJS)/TEN (SJS/TEN) can be seen because the inflammatory infiltrate is so severe.[16]
Superficial oral ulcers of the posterior surface of the hard palate are seen most commonly. Occasionally, buccal and gingival mucosae and the tongue may be involved.
Note that ACLE may coexist with other lupus erythematosus–specific skin diseases. In about 20% of cases, ACLE and subacute cutaneous lupus erythematosus (SCLE) coexist. However, the occurrence of ACLE with chronic cutaneous lupus erythematosus (CCLE) is unusual.
Erythema multiforme–like lesions may be seen with ACLE and SCLE and is often referred to as Rowell syndrome when associated with immunologic serum abnormalities such as a speckled antinuclear antibody (ANA) pattern and positive rheumatoid factor.[17]
The most common presentation of ACLE is a red macular eruption involving the malar area (see image below). The forehead, periorbital area, and neck also may be involved, representing a photodistribution. Occasionally, unilateral involvement may occur.
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Erythema involving the malar area, forehead, and neck. Note sparing of some of the creases.
Less commonly, ACLE presents as a generalized photosensitive eruption, while more rarely, patients present with widespread blistering simulating SJS)/TEN. SJS/TEN-like cutaneous lupus erythematosus is due to extensive epidermal necrosis, which is believed to be a phototoxic reaction and may be triggered by intensive ultraviolet exposure, and must be differentiated from drug-induced TEN occurring in a patient with lupus erythematosus. The combination of recent lupus exacerbation; photodistribution; annular lesions; absent or mild focal erosive mucosal involvement; and histological changes including junctional vacuolar alteration, solitary necrotic keratinocytes at lower epidermal levels, dense periadnexal and perivascular lymphocytic infiltrates, and mucin favor lupus erythematosus over SJS or TEN.[18]
The term acute syndrome of apoptotic pan-epidermolysis (ASAP) has been proposed for the TEN-like cutaneous injury pattern that can occur in settings of lupus erythematosus, where Fas-Fas ligand interactions are implicated in the massive keratinocyte apoptosis.[19] See the image below.
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Toxic epidermal necrolysis–like eruption.
Patients with ACLE frequently experience superficial ulceration of the oral and nasal mucosae. These lesions may produce extreme discomfort in some patients, although the lesions may be entirely painless in others. The posterior surface of the hard palate is the site affected most frequently; however, the gingival, buccal, and lingual mucosae also may be involved.
An unusual reported cutaneous presentation is the presence of erythematous, slightly scaly, pruritic papules and plaques on the elbows.[20]
Because acute cutaneous lupus erythematosus (ACLE) and systemic lupus erythematosus (SLE) are associated closely, it is safe to assume that the laboratory findings in SLE closely mirror the findings in ACLE.
Diagnostic data from laboratory tests are supported by histopathologic examination of the skin. Further diagnostic substantiation is obtained by performing immunofluorescent examination of skin lesions.
The most striking histologic change in ACLE is the presence of edema involving upper dermis and focal liquefactive degeneration of the basal cell layer. Cellular dermal infiltrate is sparse and consists of lymphocytes. In extreme cases, dissolution of the basal layer occurs secondary to extensive vacuolization, forming a subepidermal bulla.[22]
Antinuclear antibody (ANA) assay results invariably are positive in patients with SLE and, therefore, in patients with ACLE. The peripheral rim pattern is associated most strongly with lupus erythematosus, although other patterns commonly are present. ANA results are less likely to be positive in dermatomyositis, which mimics lupus erythematosus both clinically and histologically.
Anti–double-stranded deoxyribonucleic acid (DNA) antibody (anti-dsDNA) assay is specific for SLE and is present in 60-80% of patients with ACLE, often in high titers.
Complement levels usually are depressed in patients with ACLE.
Anti-Sm antibody assay has a strong specificity for SLE; therefore, perform this assay to exclude underlying systemic involvement. This is particularly relevant in patients in whom anti-dsDNA results are negative.
Ro (SS-A) antibodies are often correlated with cutaneous involvement in subacute cutaneous lupus erythematosus (SCLE). However, almost a third of Ro antibody–positive patients with ACLE present with kidney involvement, particularly young female patients.[23]
A positive rheumatoid factor and speckled ANA pattern may be seen in association with Rowell syndrome.[17]
Low-specificity tests include the following:
U1 ribonucleoprotein antibody assay - Results are positive in mixed connective-tissue disease, which sometimes manifests as a malar eruption
Complete blood cell (CBC) count - Anemia, leukopenia, and/or thrombocytopenia may be seen in patients with ACLE who have systemic involvement
Erythrocyte sedimentation rate - Although a nonspecific marker, marked elevations in levels indicate possible systemic involvement
Urinalysis - Proteinuria, hematuria, and urine casts are indicative of underlying nephritis
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.[24]
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.[25, 26]
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 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.[27] 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.[28, 29, 30, 31, 32]
Mycophenolate mofetil has shown poor results in disease refractory to multiple treatment modalities.[33]
One case reports describes the effectiveness of plasmapheresis for refractory toxic epidermal necrolysis (TEN)–like ACLE.[34]
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.[35]
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.[36]
Transplantation of in vivo–harvested epidermal cell suspension was reported to successfully treat depigmentation associated with malar rash.[37]
Belimumab is a neutralizing B-lymphocyte stimulator monoclonal antibody that inhibits the biologic activity of the soluble form of essential B cells.[38] 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. Belimumab is 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.[39]
Anifrolumab, a human monoclonal antibody to the type I interferon receptor subunit 1, is approved by the US Food and Drug Administration (FDA) for adults with moderate to severe SLE.[40] In the phase 3 trials for anifrolumab in SLE, this medication also demonstrated benefit in lupus-associated skin disease, with 49% of patients in the anifrolumab group having a 50% or more reduction in CLASI (Cutaneous Lupus Erythematosus Disease Area and Severity Index) activity score versus 25% in the placebo group.[41, 42, 43]
Lenalidomide (analogue of thalidomide) is an immunomodulatory drug with antineoplastic, anti-inflammatory, and antiangiogenic properties and is approved for use in many cancers. Multiple small, open-label trials have shown lenalidomide to be both safe and efficacious in the treatment of cutaneous lupus erythematosus.[44, 45]
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.[46]
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.
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.
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.[47]
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).[48] However, a phase 3 LOTUS study in SLE was discontinued because of a to lack of efficacy in SLE.
Baricitinib is an oral selective inhibitor of the Janus kinase (JAK) 1 and JAK2 and is a potential oral therapy for SLE. Baricitinib is 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.[49] However, further study is needed to evaluate its efficacy in cutaneous lupus erythematosus disease.
Immunomodulating drugs such as iberdomide, a derivative of lenalidomide, are currently in phase 2 clinical trials for the treatment of both SLE and cutaneous lupus erythematosus.
The tyrosine kinase 2 (TYK2) inhibitor deucravacitinib, is approved by the FDA to treat psoriasis. A phase 2 study focused on deucravacitinib for relief of SLE. Improvements in cutaneous disease were a secondary endpoint. The trial showed that the patients who received deucravacitinib achieved a 56-70% CLASI-50 response, depending on dosing, compared with a 17% response among those on placebo at week 48.[50]
The following organizations have released guidelines for the management of acute cutaneous lupus erythematosus. Key diagnostic and treatment recommendations have been reviewed and integrated throughout the article.
British Association of Dermatologists. Guidelines for the management of people with cutaneous lupus erythematosus. 2021[1]
European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV). S2k guideline for treatment of cutaneous lupus erythematosus. 2017[24]
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.[28]
Clinical Context:
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.
Corticosteroids have anti-inflammatory properties and cause profound and varied metabolic effects. These agents modify the body's immune response to diverse stimuli.
Clinical Context:
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.
Clinical Context:
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.
Clinical Context:
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.
Clinical Context:
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%).
Clinical Context:
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.
Clinical Context:
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.
Clinical Context:
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.
Clinical Context:
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.
Clinical Context:
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.
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.
Fnu Nutan, MD, FACP, Assistant Professor, Department of Dermatology, Virginia Commonwealth University School of Medicine
Disclosure: Nothing to disclose.
Coauthor(s)
Urmila Sivagnanalingam, MS, MD Candidate, Virginia Commonwealth University School of Medicine
Disclosure: Nothing to disclose.
Chief Editor
William D James, MD, Emeritus Professor, Department of Dermatology, University of Pennsylvania School of Medicine
Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.
Additional Contributors
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
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
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
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.
Erythema involving the malar area, forehead, and neck. Note sparing of some of the creases.
Toxic epidermal necrolysis–like eruption.
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.
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