eMedicine Specialties > Dermatology > Connective Tissue Diseases
Lupus Erythematosus, Subacute Cutaneous: Treatment & Medication
Updated: Apr 13, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- The goals of management are to improve the patient's appearance and to prevent the development of further lesions.
- Counsel patients regarding the risk of serious systemic disease. The frequency and severity of systemic disease in patients with subacute cutaneous lupus erythematosus (SCLE) is controversial. Traditionally, it was suggested that although many patients fulfilled the criteria for SLE, the severity of systemic manifestations was mild, and renal and CNS disease rarely occurred. Cohen and Crosby found no appreciable difference between SCLE and SLE patients seen in a rheumatology practice,8 whereas Black et al found significant differences between these 2 populations.9 Inform patients that although they probably will not have serious systemic disease, follow-up evaluation and treatment will be performed for manifestations that may occur.
- Therapy begins with sun-protective measures, including sunscreens, protective clothing, and behavior alteration. One sunscreen has been tested in a randomized placebo-controlled trial and was demonstrated to prevent the development of UV-induced cutaneous lesions among patients who used a sunscreen that contained Mexoryl SX and Mexoryl XL.10,11 Some patients with cutaneous LE have been found to be vitamin D deficient; therefore, the use of vitamin D and calcium should be considered following an assessment of vitamin D levels.12
- Cosmetic measures are less important in patients with SCLE than in patients with DLE.
- Standard therapy includes corticosteroids (topical, intralesional) and antimalarials. Additional therapies that may be considered in selected patients include auranofin, dapsone, thalidomide, retinoids, interferon, and immunosuppressive agents.13,14,15,16 Avoid systemic corticosteroids except for acute short-term usage or when the presence of systemic disease warrants use. Patients who smoke appear to respond less well to antimalarial therapy. Note that clofazimine is primarily beneficial for cutaneous, not systemic, disease.
Surgical Care
Surgical approaches rarely are needed in SCLE patients.
Consultations
- Rheumatologist - When joints are involved
- Nephrologist - When renal involvement is present
- Neurologist - When CNS disease is present
- Internal medicine specialist - For systemic involvement
Diet
No special diet is required with SCLE.
Activity
Since SCLE is exacerbated by sunlight or other UV light exposure, advise patients to take precautions. One precaution is to discourage exposure to sunlight between the hours of 10 am and 4 pm. While this helps some patients, many are so exquisitely photosensitive, that this alteration does not help. In addition, advise patients to avoid artificial light sources such as tanning beds.
Medication
The basic therapy of skin disease uses sun-protection methods, such as sunscreens, sun-protective clothing, and alteration of exposure by decreasing activities during times of high intensity UV light. Topical corticosteroids are used and selected by the appropriate strength for the area of the body. Intralesional injection of triamcinolone acetonide is useful for individual recalcitrant lesions. Antimalarials are the mainstay of systemic therapy.
Anecdotal reports or small open-label trials, as reported by Callen,17,18,19 suggest that the following agents may be of use in some patients: dapsone, quinacrine, auranofin, thalidomide, isotretinoin, acitretin, azathioprine, methotrexate (MTX), mycophenolate mofetil, interferon alfa, chimeric monoclonal antibody, and phenytoin.20,21,22,23
Antimalarials
Have immunomodulatory effects that may improve symptoms of the disease. Hydroxychloroquine is DOC for systemic therapy of SCLE. Chloroquine is second line. The lowest possible dose needed to control their disease should be used.
Hydroxychloroquine (Plaquenil)
Inhibits chemotaxis of eosinophils, 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.
Adult
200-400 mg sulfate/d PO; not to exceed 6.5 mg/kg/d; 310 mg base PO qd/bid for several wk depending on response; 155-310 mg base/d for prolonged maintenance therapy
Pediatric
Up to 6.5 mg sulfate/kg/d PO; 3-5 mg base/kg/d PO qd or divided bid; not to exceed 7 mg/kg/d
May increase penicillamine levels; serum levels of hydroxychloroquine may increase with cimetidine; magnesium trisilicate may decrease absorption; concurrent use of aurothioglucose and antimalarial agents may induce blood dyscrasias and may also result in additive risk of this effect; concurrent digoxin may result in increased serum digoxin concentrations
Concomitant therapy with astemizole, cisapride, arsenic trioxide, TCAs, or other agents may prolong QT interval; concurrent administration with mefloquine may produce electrocardiographic abnormalities and increase risk of convulsions; rabies vaccine (HDCV) administered intradermally should be completed 1-2 mo before hydroxychloroquine administration begins (if not possible, check serologic tests several weeks after vaccination to determine degree of antibody response; if HDCV given intramuscularly, chloroquine may be given concomitantly)
Documented hypersensitivity; psoriasis; retinal and visual field changes attributable to 4-aminoquinolones
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Crosses placenta and may cause ocular, CNS, or ototoxicity in fetus; do not use in breast-feeding; limit pediatric use to established safe doses to avoid potential fatality; perform regular ophthalmologic exams (including visual acuity, slit lamp, funduscopic, and visual field tests); caution in patients with G-6-PD deficiency; check blood counts periodically (perhaps biannually); hemolysis, aplastic anemia, agranulocytosis, and leukopenia can occur; test for muscle weakness; ECG changes (eg, T-wave inversion, T-wave flattening, QT interval prolongation) can occur following therapeutic doses
Chloroquine (Aralen)
Inhibits effects of immune cells, impairing complement-dependent antigen-antibody reactions.
Adult
250-500 mg/d PO
Pediatric
Up to 3.5 mg base tab/kg/d
Cimetidine may increase serum levels (possibly other 4-aminoquinolones); magnesium trisilicate may decrease absorption of 4-aminoquinolones
Concurrent use with kaolin may decrease plasma concentrations of chloroquine; concurrent administration with aurothioglucose or antimalarial agents may result in additive effect inducing blood dyscrasias; concomitant administration of levothyroxine may decrease effect of levothyroxine and increase serum thyrotropin levels
Concomitant therapy with astemizole, cisapride, arsenic trioxide, tricyclic antidepressants, or other agents may prolong QT interval; concurrent administration of mefloquine and chloroquine may produce electrocardiographic abnormalities and increase risk of convulsions; rabies vaccine (HDCV) administered intradermally should be completed 1-2 mo before chloroquine administration begins (if not possible, check serologic tests several weeks after vaccination to determine degree of antibody response; if HDCV given intramuscularly, chloroquine may be given concomitantly)
Documented hypersensitivity; psoriasis; retinal and visual field changes attributable to 4-aminoquinolones
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Ocular toxicity is possible for hydroxychloroquine and chloroquine; caution in hepatic disease, G-6-PD deficiency, psoriasis, porphyria; not recommended for long-term use in children; perform periodic ophthalmologic examinations; test for muscle weakness; ECG changes (eg, T-wave inversion, T-wave flattening, QT interval prolongation) can occur following therapeutic doses of chloroquine
Leprostatic agents
May have immunomodulatory effects.
Dapsone (Avlosulfon)
Mechanism of action is similar to sulfonamides where competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth.
Adult
100-200 mg/d PO
Pediatric
Not established
May inhibit anti-inflammatory effects of clofazimine; hematologic reactions may increase with folic acid antagonists, eg, pyrimethamine (monitor for agranulocytosis during second and third mo of therapy); probenecid increases dapsone toxicity; trimethoprim with dapsone may increase toxicity of both drugs; because of increased renal clearance, dapsone levels may significantly decrease when administered concurrently with rifampin
Documented hypersensitivity; G-6-PD deficiency
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Perform weekly blood counts (first mo), then perform WBC counts monthly (6 mo), then semiannually; discontinue if significant reduction in platelets, leukocytes, or hematopoiesis is seen; caution in methemoglobin reductase deficiency, G-6-PD deficiency, or hemoglobin M because of high risk for hemolysis and Heinz body formation; caution in patients exposed to other agents or conditions (eg, infection, diabetic ketosis) capable of producing hemolysis; peripheral neuropathy can occur (rare); phototoxicity may occur when exposed to UV light; pancreatitis may occur; various forms of renal complications, including acute renal failure, acute tubular necrosis, and oliguria, have occurred
Clofazimine (Lamprene)
Lipophilic rhimophenazine dye that inhibits template function of DNA by binding to it. Weakly bactericidal and has anti-inflammatory effects. Originally developed to treat tuberculosis. Although mechanism of action unclear, seems to exert main effect upon neutrophils and monocytes in a variety of ways (eg, stimulating phagocytosis and release of lysosomal enzymes).
Adult
100 mg/d PO qd
Pediatric
1 mg/kg/d PO qd
Dapsone may inhibit anti-inflammatory activity, although commonly used together; levels may be increased by simultaneous use with isoniazid
Concurrent use with aluminum/magnesium-containing antacids may result in reduced clofazimine bioavailability and should be avoided because of the decreased absorption
Fosphenytoin is a prodrug of phenytoin, and the same interactions that occur with phenytoin are expected to occur with fosphenytoin; concurrent use of phenytoin and clofazimine may result in reduced phenytoin efficacy; when clofazimine is added to or withdrawn from therapy, phenytoin dose adjustments may be needed; monitor patients for reduced phenytoin efficacy; also prudent to monitor phenytoin serum concentrations; alterations in fosphenytoin dosing may be required when clofazimine is given concomitantly
In healthy, fasted subjects, concurrent administration of small quantities of orange juice with clofazimine resulted in modest reduction of clofazimine relative bioavailability; avoid concurrent administration of clofazimine and orange juice when in a fasted state
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Severe abdominal symptoms may require exploratory laparotomies; caution in patients with GI problems (eg, abdominal pain, diarrhea); skin discoloration due to drug may result in depression or suicide; apply oil to skin for dryness and ichthyosis; monitor liver function if dose >100 mg/d
Immunomodulators
Are effective in the treatment of diseases with autoimmune etiology.
Azathioprine (Imuran)
Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity.
Adult
1 mg/kg/d PO for 6-8 wk; increase by 0.5 mg/kg q4wk until response is seen or dose reaches 2.5 mg/kg/d
Pediatric
Initial dose: 2-5 mg/kg/d PO/IV
Maintenance dose: 1-2 mg/kg/d PO/IV
Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of MTX metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine; coadministration with mycophenolate may increase toxicity; alfalfa, black Cohosh, and echinacea may reduce immunosuppressive drug effectiveness
Documented hypersensitivity
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Increases risk of neoplasia; caution in liver disease and renal impairment; hematologic toxicities may occur; hepatotoxicity and pancreatitis reported
Thalidomide (Thalomid)
May suppress excessive production of tumor necrosis factor alpha (TNF-alpha), and may down-regulate selected cell-surface adhesion molecules involved in leukocyte migration.
If <50 kg (110 lb), start at low end of dose regimen.
Can cause severe, life-threatening birth defects and is contraindicated in pregnant women. Also contraindicated in women of childbearing potential unless using 2 forms of reliable contraception and complying with serial pregnancy testing while on therapy.
Also contraindicated in sexually active men not using latex condom as barrier contraception. Drug available only under special restricted distribution program called STEPS (System for Thalidomide Education and Prescribing Safety) Program; only prescribers and pharmacists registered with this program may prescribe and dispense thalidomide. For more information, contact the Celgene Corporation at 1-888-423-5436.
Adult
100-300 mg/d PO qd with water, preferably hs and >1 h pc
Pediatric
Not established
May increase sedation effects of alcohol, barbiturates, chlorpromazine, and reserpine; increases thromboembolic risk of erythropoietic proteins such as Darbepoetin Alfa in myelodysplastic syndrome (MDS) patients
Coadministration with dexamethasone increases risk of developing toxic epidermal necrolysis;
risk of renal dysfunction may be increased when zoledronic acid used in combination with thalidomide in multiple myeloma patients
Documented hypersensitivity
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Perform pregnancy test within 24 h prior to initiating therapy (weekly during first mo, followed by monthly tests in women with regular menstrual cycles or q2wk with irregular menstrual cycles); bradycardia may occur; use protective measures (eg, sunscreens, protective clothing) against exposure to sunlight or UV light (eg, tanning beds); as result of teratogenic effects, women must use 2 additional methods of contraception or abstain from intercourse; adverse effects include neutropenia, peripheral neuropathy, pulmonary embolism, seizure, and thrombotic disorder
Mycophenolate (CellCept)
Inhibits inosine monophosphate dehydrogenase (IMPDH) and suppresses de novo purine synthesis by lymphocytes, thereby inhibiting their proliferation. Inhibits antibody production.
Adult
1-1.5 g PO bid
Pediatric
Not established; 15-23 mg/kg PO bid suggested
May elevate levels of acyclovir and ganciclovir; antacids and cholestyramine decreases absorption, reducing levels (do not administer together); probenecid may increase levels of mycophenolate; salicylates may increase toxicity of mycophenolate; concomitant use with azathioprine not recommended; use cautiously in elderly patient and with drugs that affect enterohepatic recirculation; live attenuated vaccines should not be used during treatment and other vaccines may be less effective
Documented hypersensitivity; hypersensitivity to polysorbate 80 (IV formulation)
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Increases risk for infection; increases toxicity in patients with renal impairment; caution in active peptic ulcer disease
Increases risk for infection; increases toxicity in patients with renal impairment; caution in active peptic ulcer disease
Bone marrow suppression may occur, including severe neutropenia; due to increased risk of skin cancer, limit exposure to sunlight and UV light; increased risk for developing lymphomas or other malignancies; concomitant use with azathioprine is not recommended; oral susp contains aspartame so should be used with caution in patients with phenylketonuria; use cautiously in elderly patient and with drugs that affect enterohepatic recirculation; live attenuated vaccines should not be used during treatment and other vaccines may be less effective; avoid in patients with hereditary deficiency of hypoxanthine-guanine phosphoribosyl-transferase
Avoid in pregnant women unless benefit clearly outweighs risk; negative pregnancy test should be obtained in women of childbearing potential; contraception should be used during treatment and for 6 wk after stopping treatment
Serious adverse effects may include confusion, GI hemorrhage, hypertension, increased frequency of cough, infectious disease, myelosuppression, peripheral edema, sepsis, and tremor
Methotrexate (Rheumatrex, Trexall)
This drug reversibly inhibits dihydrofolate reductase; limits the availability of 1-carbon fragments necessary for synthesis of purines and the conversion of deoxyuridylate to thymidylate in the synthesis of DNA and cell reproduction. Extensively used for cancer treatment, rheumatoid arthritis, psoriasis, and as a steroid-sparing agent in various autoimmune conditions.
Adult
In autoimmune conditions: 7.5-25 mg/wk as a single dose PO/SC
Folic acid supplementation is usually given concomitantly
Pediatric
5-15 mg/m2/wk as a single dose PO/SC
Children receiving 20-30 mg/m2 may have better absorption and fewer adverse GI effects if administered IM or SC
Safety and effectiveness in pediatric patients only established for cancer chemotherapy and polyarticular-course juvenile rheumatoid arthritis; when oral MTX is indicated for polyarticular-course juvenile rheumatoid arthritis recommended initial dose is 10 mg/m2 once weekly, with gradual dosage adjustments to achieve optimal response
Oral aminoglycosides may decrease absorption and blood levels of concurrent oral MTX; charcoal lowers MTX levels; coadministration with etretinate may increase hepatotoxicity of MTX; folic acid or its derivatives contained in some vitamins may decrease response to MTX
Probenecid, NSAIDs, salicylates, procarbazine, and sulfonamides, including TMP-SMZ, can increase MTX plasma levels; may decrease phenytoin plasma levels; may increase plasma levels of thiopurines
Documented hypersensitivity; alcoholism; hepatic insufficiency; documented immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia); renal insufficiency
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Monitor CBC counts monthly, and liver and renal function q1-3mo during therapy (monitor more frequently during initial dosing, dose adjustments, or when risk of elevated MTX levels, eg, dehydration); MTX has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems; discontinue if significant drop in blood counts occur; fatal reactions reported when administered concurrently with NSAIDs
Increased incidence of serious toxic reactions, especially bone marrow suppression, in adults at doses >20 mg/wk
Efalizumab (Raptiva)
Recombinant humanized monoclonal IgG1 antibody directed against LFA-1, which interferes with T-lymphocyte activation, trans tissue trafficking, and T-lymphocyte reactivation. Efalizumab is being withdrawn from the US market and will no longer be available after June 8, 2009, because of potential risk for progressive multifocal leukoencephalopathy (PML). PML is a rapidly progressive infection of the central nervous system caused by the JC virus that leads to death or severe disability. Demyelination associated with PML is a result from the JC virus infection. JC virus belongs to the genus Polyomavirus of the Papovaviridae. PML should be considered in any patient presenting with new-onset neurologic manifestations who have taken efalizumab. For more information, see the Food and Drug Administration MedWatch Safety Alert.
Adult
0.7 mg/kg SC first dose, followed by 1 mg/kg/wk SC
Pediatric
Not established
Concurrent administration of live vaccinations may increase risk of disseminated infection; administration with other immunosuppressive agents (eg, corticosteroids, cyclosporine) may cause excessive immunosuppression
Documented hypersensitivity; thrombocytopenia; concomitant immunosuppressant use; serious infections
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Associated with increased risk for life threatening infections (eg, JC virus), resulting in progressive multifocal leukoencephalopathy, bacterial sepsis, viral meningitis, or invasive fungal disease; flulike symptoms may occur (transient); adverse effects may include thrombocytopenia, hemolytic anemia, pancytopenia, and peripheral demyelination; patients may experience rebound effects with abrupt cessation of drug (rarely)
Gold compounds
May regulate immune cell function.
Auranofin (Ridaura)
Gold is taken up by macrophages, which in turn inhibit phagocytosis and lysosomal membrane stabilization. Alters immunoglobulins, decreasing prostaglandin synthesis and lysosomal enzyme activity.
Adult
6 mg/d PO qd or divided bid; after 3 mo, may increase to 9 mg/d tid; then, if no response, discontinue drug
Pediatric
Initial: 0.1 mg/kg/d PO qd or divided bid
Maintenance: 0.15 mg/kg/d PO qd or divided bid
Penicillamine, hydroxychloroquine, and antimalarials may increase toxicity of auranofin
Documented hypersensitivity; renal impairment; history of blood dyscrasias, exfoliative dermatitis, congestive heart failure, necrotizing enterocolitis, bone marrow aplasia, pulmonary fibrosis
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Discontinue therapy if platelet counts fall <100,000/µL; WBC <4,000/µL, granulocytes <1,500/µL
Adverse effects include gastrointestinal hemorrhage, hepatotoxicity, nephrotoxicity, hematuria, proteinuria, and pneumonitis; caution in compromised cerebral or cardiovascular circulation
Retinoids
Play a role in cell growth and differentiation.
Acitretin (Soriatane)
Retinoic acid analog similar to etretinate and isotretinoin. Etretinate is main metabolite, and acitretin has demonstrated clinical effects close to those seen with etretinate. Mechanism of action is unknown.
Adult
Initial dose: 25 or 50 mg/d PO single dose with main meal
Maintenance dose: 25-50 mg/d PO after initial response; terminate when lesions have resolved sufficiently
Pediatric
Not established
Increases toxicity of MTX (avoid concomitant use); interferes with effects of microdosed progestin minipill; coadministration with alcohol may enhance synthesis of etretinate, which has much longer half-life than acitretin (>120 d)
Documented hypersensitivity
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Do not use in severe obesity; women of childbearing age must be able to comply with effective contraceptive measures, abstain from alcohol, and continue contraceptive measures for a minimum of 3 y following cessation of therapy; perform AST, ALT, and LDH tests prior to initiating acitretin at 1- to 2-wk intervals until stable and thereafter, at intervals as indicated clinically
Isotretinoin (Accutane)
Decreases sebaceous gland size and sebum production. May inhibit sebaceous gland differentiation and abnormal keratinization.
A US Food and Drug Administration–mandated registry is now in place for all individuals prescribing, dispensing, or taking isotretinoin. For more information on this registry, see iPLEDGE. This registry aims to further decrease the risk of pregnancy and other unwanted and potentially dangerous adverse effects during a course of isotretinoin therapy.
Adult
40-60 mg/d PO for 4 mo; alternatively, 1-2 mg/kg/d for up to 20 wk
Pediatric
Not established
Toxicity may occur with vitamin A coadministration; pseudotumor cerebri or papilledema may occur when coadministered with tetracyclines; may reduce plasma levels of carbamazepine
Documented hypersensitivity
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
May decrease night vision; may be associated with development of hepatitis; occasional exaggerated healing response of acne lesions (excessive granulation with crusting) may occur; patients with diabetes may experience problems in controlling blood sugar; avoid excessive exposure to UV light or sunlight
More on Lupus Erythematosus, Subacute Cutaneous |
| Overview: Lupus Erythematosus, Subacute Cutaneous |
| Differential Diagnoses & Workup: Lupus Erythematosus, Subacute Cutaneous |
 Treatment & Medication: Lupus Erythematosus, Subacute Cutaneous |
| Follow-up: Lupus Erythematosus, Subacute Cutaneous |
| Multimedia: Lupus Erythematosus, Subacute Cutaneous |
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References
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Further Reading
Keywords
subacute cutaneous lupus erythematosus, SCLE, systemic lupus erythematosus, SLE, lupus, autoimmune disease, discoid lupus erythematosus, Sjogren syndrome
