eMedicine Specialties > Dermatology > Connective Tissue Diseases
Lupus Erythematosus, Discoid: Treatment & Medication
Updated: Nov 9, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- The goals of discoid lupus erythematosus (DLE) management are to improve the patient's appearance, to control existing lesions and limit scarring, and to prevent the development of further lesions.
- Advise patients that the risk of serious systemic disease is possible, although rare. Regular repeat clinical evaluation accompanied by simple laboratory studies usually is sufficient to evaluate the possible progression from the primary cutaneous disorder to the disorder accompanied by systemic involvement.
- Therapy begins with sun-protective measures, including sunscreens, protective clothing, and behavior alteration. Cosmetic measures, such as cover-up makeup or wigs, may be suggested for appropriately selected patients. Makeup used for camouflage includes Covermark and Dermablend.6
- Smoking appears to decrease the efficacy of antimalarial agents, and efforts regarding smoking cessation are advisable in patients who smoke or are exposed to secondary smoke.7,8,9
- Standard medical therapy includes corticosteroids (topical or intralesional) and antimalarials. Antimalarials appear less effective in patients who smoke; however, discoid lupus erythematosus possibly is worse in these patients. Alternative therapies include auranofin, thalidomide,10,11,12,13 oral or topical retinoids, and immunosuppressive agents.14,15 Thalidomide is regularly used in antimalarial-resistant patients. In most patients, the antimalarial should be continued during thalidomide therapy, unless a complication due to the antimalarial occurs. In addition, lenalidomide may be useful in some patients.16
- Topical corticosteroids are selected for the type of lesion under treatment and for the site of involvement. For example, lotions or foams are preferred for the scalp, weaker agents are used on the face, and superpotent agents are used for hypertrophic lesions.
- Topical calcineurin inhibitors have also been used in patients with cutaneous lesions of LE. In addition, topical retinoids have been reported to be helpful. Lastly, topical imiquimod has been reported to be effective in one patient.
- Intralesional injection of corticosteroids (typically, this author uses triamcinolone acetonide 3 mg/mL) is useful as adjunctive therapy for individual lesions. Potential for atrophy relates to the amount of corticosteroid injected in any one area; therefore, dilute concentrations are preferred. In addition, the treating physician must take care to limit the total dose of the injections at any given office/clinic visit to avoid systemic toxicity from the steroids eg, if a patient is given 10 mL of triamcinolone 3 mg/mL, this means that the patient has received a total of 30 mg, and toxicity is the same as if it had been delivered orally or by intramuscular injection.
- Among immunosuppressives, methotrexate (MTX) may be considered.17 In this author's experience, azathioprine and, recently, mycophenolate mofetil, have been more successful than MTX, while systemic corticosteroids are rarely effective.18
Surgical Care
- Excision of burned-out scarred lesions is possible; however, reactivation of inactive lesions has been reported in some patients.
- Laser therapy may be useful for lesions with prominent telangiectases. Reactivation also is a consideration with this form of therapy.
- An open trial in a small group of patients has demonstrated efficacy of pulsed dye laser therapy for discoid lupus erythematosus lesions. However, before using this therapy in additional patients, at a minimum, a test area should be treated to make certain that the discoid lupus erythematosus does not flare.19
Consultations
- Rheumatologist - For joint involvement
- Nephrologist - For renal involvement
- Internist - To evaluate systemic involvement
- Ophthalmologist - To monitor therapy with hydroxychloroquine or chloroquine
Diet
- No special diet is recommended.
Activity
- Since chronic CLE is exacerbated by sunlight or other UV exposure, advise patients to take precautions, eg, to limit exposure to sunlight to early morning or late afternoon when the sun is less intense. Advise patients to avoid artificial light sources such as tanning beds.
Medication
The goals of pharmacotherapy are to reduce morbidity and to prevent complications. Hydroxychloroquine and chloroquine phosphate have shown beneficial effects in treating DLE. Alternative therapies, anecdotal reports, and small open-label trials (as reported by Callen20 ) suggest that the following agents may be useful in some patients: dapsone, auranofin, quinacrine, thalidomide, isotretinoin,21 acitretin, azathioprine, mycophenolate mofetil, phenytoin, interferon, and chimeric monoclonal antibody.22
Antimalarial agents
May have immunomodulatory properties. Hydroxychloroquine is DOC when a systemic agent is needed for DLE. Chloroquine is second-line therapy.
Hydroxychloroquine (Plaquenil)
For treatment of DLE and SLE. 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/d PO; not to exceed 6.5 mg/kg/d; 310 mg PO qd or bid for several wk depending on response; 155-310 mg/d for prolonged maintenance therapy
Pediatric
6.5 mg/kg/d PO; 3-5 mg base/kg/d PO qd or divided bid; not to exceed 7 mg/kg/d
Penicillamine levels may increase; serum levels increase with cimetidine; magnesium trisilicate may decrease absorption
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 the fetus; do not use if breast-feeding; limit pediatric use to established safe doses to avoid potential fatality; ocular toxicity is possible for hydroxychloroquine and chloroquine but not quinacrine; perform regular ophthalmologic examinations during therapy
Chloroquine (Aralen)
Inhibits chemotaxis of eosinophils, locomotion of neutrophils, and impairs complement-dependent antigen-antibody reactions.
Adult
250-500 mg PO qd
Pediatric
10 mg/kg PO d 1, then 5 mg/kg 6 h later, followed by 5 mg/kg d 2 and 3
Cimetidine may increase serum levels of chloroquine (possibly other 4-aminoquinolones); magnesium trisilicate may decrease absorption of 4-aminoquinolones
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
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
Leprostatic agents
May modulate the immune system.
Dapsone (Avlosulfon)
Mechanism of action is similar to sulfonamides where competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth. The anti-inflammatory action may relate to suppression of neutrophil function by inhibition of the halide-myeloperoxidase system.
Adult
100-200 mg PO qd
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; 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 or biweekly 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
Gold compounds
Have proven effective in the treatment of inflammation with autoimmune etiology.
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 divided tid; then, if no response, discontinue drug
Pediatric
Initial dose: 0.1 mg/kg/d PO divided bid
Maintenance dose: 0.15 mg/kg/d PO qd or divided bid
Penicillamine, hydroxychloroquine, and antimalarials may increase toxicity
Documented hypersensitivity; renal impairment; history of blood dyscrasias, exfoliative dermatitis, congestive heart failure, necrotizing enterocolitis
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Discontinue therapy if platelet counts fall <100,000/µL, WBC count <4,000/µL, granulocyte count <1,500/µL
Immunomodulators
Affect factors that regulate the immune system.
Methotrexate (Rheumatrex, Trexall)
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
Oral aminoglycosides may decrease absorption and blood levels of concurrent oral MTX; charcoal lowers levels; coadministration with etretinate may increase hepatotoxicity; 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); has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems; discontinue if significant drop in blood counts occurs; fatal reactions reported when administered concurrently with NSAIDs
Thalidomide (Thalomid)
Immunomodulatory agent that may suppress excessive production of 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.
Adult
100-300 mg PO hs aq, and >1 h pc
Pediatric
Not established
May increase sedation effects of alcohol, barbiturates, chlorpromazine, and reserpine; because of teratogenic effects, women must use 2 additional methods of contraception or abstain from intercourse
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)
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
Documented hypersensitivity
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Increases risk of neoplasia; caution with liver disease and renal impairment; hematologic toxicities may occur
Interferon alfa-2a and alfa-2b (Roferon and Intron A)
Protein product manufactured by recombinant DNA technology. Mechanism of antitumor activity is not clearly understood; however, direct antiproliferative effects against malignant cells and modulation of host immune response may be important factors. Has antiviral, antitumor, and immunomodulatory actions.
Adult
2 million U/m2 SC 3 times/wk for 30 d
Pediatric
Not established
Theophylline may increase toxicity; cimetidine may increase antitumor effects; zidovudine and vinblastine may increase toxicity
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
Caution in brain metastases, severe hepatic or renal insufficiencies, seizure disorders, multiple sclerosis, or compromised CNS
Mycophenolate (CellCept)
Inhibits inosine monophosphate dehydrogenase and suppresses de novo purine synthesis by lymphocytes, thereby inhibiting their proliferation. Inhibits antibody production.
Adult
1 g PO bid
Pediatric
Not established; 15-23 mg/kg PO bid suggested
May elevate levels of acyclovir and ganciclovir; antacids and cholestyramine decrease absorption, reducing levels (do not administer together); probenecid may increase levels of mycophenolate; salicylates may increase toxicity of mycophenolate
Documented hypersensitivity
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
Corticosteroids
Anti-inflammatory agents that suppress the immune system at several levels including inhibition of inflammatory cells and the production of antibodies.
Triamcinolone (Aristocort)
Can be administered intralesionally in a concentration of 3-5 mg/mL. Amounts injected should be recorded. Systemic adverse effects are uncommon with low doses. Atrophy is possible and is dose dependent.
Adult
3-5 mg/mL; not to exceed 2 mL at any single setting
Pediatric
Not established
Rare for intralesional, but if administered IM or in sufficient dosage, potential adverse effects may occur with coadministration with barbiturates, phenytoin, and rifampin, which decrease effects of triamcinolone
Documented hypersensitivity; fungal, viral, and bacterial skin 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
Adverse effects of intralesional corticosteroids include atrophy and hypopigmentation; significant systemic exposure to corticosteroids may result in multiple complications (eg, severe infections, hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, and growth suppression); abrupt discontinuation of glucocorticoids may cause adrenal crisis
Retinoids
Have the ability to regulate cell proliferation and regulate immune system.
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 therapy 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 intake, and continue contraceptive measures for a minimum of 3 y following cessation of therapy; perform AST, ALT, and LDH tests prior to initiation of acitretin therapy at 1- to 2-wk intervals until stable and thereafter, at intervals as indicated clinically
Isotretinoin (Accutane)
Synthetic 13-cis isomer of naturally occurring tretinoin (trans -retinoic acid). Both agents are structurally related to vitamin A.
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
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; inflammatory bowel disease may occur; 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 exposure to UV light or sunlight until tolerance achieved; discontinue treatment if rectal bleeding, abdominal pain, or severe diarrhea occur
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| Overview: Lupus Erythematosus, Discoid |
| Differential Diagnoses & Workup: Lupus Erythematosus, Discoid |
 Treatment & Medication: Lupus Erythematosus, Discoid |
| Follow-up: Lupus Erythematosus, Discoid |
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References
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Further Reading
Keywords
chronic cutaneous lupus erythematosus, discoid lupus erythematosus
