eMedicine Specialties > Dermatology > Connective Tissue Diseases
Lupus Erythematosus, Bullous: Treatment & Medication
Updated: Jun 18, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Bullous systemic lupus erythematosus (BSLE) generally responds well to medical therapy, and treatment with dapsone is particularly effective. Although type 1 bullous systemic lupus erythematosus and epidermolysis bullosa acquisita are both characterized by antibodies targeting type VII collagen, epidermolysis bullosa acquisita differs considerably in its marked resistance to therapy.
- Dapsone is the initial treatment of choice for bullous systemic lupus erythematosus. The response is usually dramatic, with cessation of new blister formation within 1-2 days and rapid healing of existing lesions. Low doses (25-50 mg/d) are often effective, although a higher dosage is sometimes required. Rapid recurrences may occur upon withdrawal of dapsone, with prompt remission after reinstitution of therapy. However, discontinuance of dapsone therapy is usually possible within a year.
- Prednisone may be effective in patients who cannot tolerate dapsone (eg, those with glucose-6-phosphate dehydrogenase [G-6-PD] deficiency), have a poor response to dapsone, or require treatment of concurrent systemic manifestations of systemic lupus erythematosus. Combination therapy with prednisone and dapsone can also be beneficial.
- Methotrexate (MTX), azathioprine, and mycophenolate mofetil represent additional therapeutic options.21
- Extensive eruptions of toxic epidermal necrolysis–like lupus erythematosus require prompt institution of therapy with intravenous immunoglobulin and/or systemic corticosteroids. Less fulminant manifestations of erythema multiforme–like lupus erythematosus can be treated with antimalarials, corticosteroids (topical or systemic, depending on the severity and presence of systemic disease), and other agents in the therapeutic armamentarium for lupus erythematosus. See Lupus Erythematosus, Subacute Cutaneous.
Consultations
- Dermatologist - For evaluation and management of bullous systemic lupus erythematosus, toxic epidermal necrolysis– or erythema multiforme–like lupus erythematosus, and other cutaneous manifestations of lupus erythematosus
- Internist/rheumatologist - For evaluation and management of extracutaneous (eg, renal, cardiac, pulmonary) manifestations of systemic lupus erythematosus
Medication
Bullous systemic lupus erythematosus (BSLE) generally responds well to medical therapy. Dapsone represents the mainstay of treatment, typically leading to rapid clearance of lesions.
As in drug-induced toxic epidermal necrolysis, intravenous immunoglobulin represents an important therapeutic option for the Fas-mediated massive epidermal necrosis of fulminant toxic epidermal necrolysis–like lupus erythematosus.
Antimycobacterial agents
Therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.
Dapsone (Avlosulfon)
The general mechanism of action is similar to that of sulfonamides, with which competitive antagonism of PABA prevents formation of folic acid, inhibiting bacterial growth. The anti-inflammatory mechanism of dapsone is believed to result from suppression of neutrophils by inhibiting neutrophil myeloperoxidase and inflammation-inducing oxygen intermediates. Shown to inhibit some forms of neutrophil chemotaxis, suppress leukocyte integrin function, and decrease attachment of neutrophils to endothelial cell junctions.
Adult
25-200 mg/d PO
Pediatric
1-2 mg/kg/d PO; not to exceed 100 mg/d
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, amprenavir, saquinavir, and zidovudine may increase toxicity; trimethoprim with dapsone may increase toxicity of both drugs; because of increased renal clearance, levels may decrease significantly 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 cell counts (first mo), then monthly WBC counts (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; may cause hepatotoxicity
Corticosteroids
Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.
Prednisone (Deltasone, Orasone, Sterapred)
Decreases inflammation; in particular, suppresses neutrophil presence and activity at sites of inflammation by inhibition of endothelial cell adhesion molecule expression and chemoattractant production. Also inhibits antigen presentation, T lymphocyte activity, and (at higher doses) antibody production.
Adult
0.5-1.5 mg/kg/d PO for several wk, with slow tapering
Pediatric
Administer as in adults
Live or attenuated vaccines; coadministration with estrogens may decrease clearance
Coadministration with estrogens may decrease clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase the metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics; coadministration with ritonavir may significantly increase serum concentrations of prednisone; concomitant therapy with montelukast may result in severe peripheral edema; clarithromycin may increase risk of psychotic symptoms
Postmarketing surveillance reports indicate that risk of tendon rupture may be increased in patients receiving concomitant fluoroquinolones and corticosteroids, especially elderly patients; administration of asparaginase concurrently with or before prednisone therapy may result in increased toxicity
Documented hypersensitivity; active viral, fungal, or mycobacterial infections; peptic ulcer disease, hepatic dysfunction
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
Short-term adverse effects include mood changes, insomnia, gastritis, salt and water retention, increased appetite/weight gain, hyperglycemia, and acneiform eruptions; long-term use may also lead to Cushing syndrome, HPA-axis suppression, hypertension, hypokalemic alkalosis, peptic ulcer disease, osteoporosis, myopathy, posterior subcapsular cataracts, glaucoma, and growth retardation; regardless of dosing schedule, avascular necrosis of long bones, usually the femoral head, can occur
Antirheumatic agents
Have anti-inflammatory effects.
Methotrexate (Folex PFS, Rheumatrex)
Antimetabolite that inhibits dihydrofolate reductase, thereby hindering DNA and RNA synthesis in lymphocytes and other immune cells. Anti-inflammatory effects also result from inhibition of AICAR transformylase (increasing tissue concentrations of the anti-inflammatory mediator adenosine) and methionine synthetase (reducing production of the proinflammatory mediator S-adenyl methionine).
Adult
7.5-25 mg/wk, PO/IM; folic acid 1 mg/d is given concomitantly
Pediatric
0.2-0.6 mg/kg/wk PO/IM/SC; folic acid 1 mg/d is given concomitantly
Oral aminoglycosides may decrease absorption and blood levels of concurrent oral MTX; charcoal lowers 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 count 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); although low-dose MTX is generally well-tolerated, MTX can have toxic effects on hematologic, hepatic, renal, GI, pulmonary, and neurologic systems; discontinue if significant drop in blood cell counts occurs; fatal reactions have been reported when administered concurrently with NSAIDs; serious adverse effects include atrophy of liver, cirrhosis, lung disease, GI hemorrhage, hepatic fibrosis, hyperuricemia, elevated liver function test results, inflammatory disease of mucous membranes, interstitial pneumonia (acute and chronic), kidney disease, liver failure, myelosuppression, renal failure, skin ulcer, and arachnoiditis (with intrathecal administration)
Blood products
Used to improve clinical and immunologic aspects of the disease. May decrease autoantibody production and increase solubilization and removal of immune complexes.
Immune globulin intravenous (Gammar-P, Sandoglobulin, Gammagard)
Antibody-mediated blockade of Fas-Fas ligand interactions involved in the epidermal necrosis of toxic epidermal necrolysis–like cutaneous lupus erythematosus.
Adult
0.75-1 g/kg/d IV for 3-7 d
Pediatric
Administer as in adults
Globulin preparation may interfere with immune response to live virus vaccine (MMR) and reduce efficacy (do not administer within 3 mo of vaccine)
Documented hypersensitivity; IgA 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
Check serum IgA level before use (use an IgA-depleted product, eg, Gammagard S/D) if positive ; infusions may increase serum viscosity and thromboembolic events; infusions may increase risk of migraine attacks, aseptic meningitis (10%), urticaria, pruritus, or petechiae (2-30 d postinfusion)
Increases risk of renal tubular necrosis in elderly patients and in patients with diabetes, volume depletion, and preexisting kidney disease; laboratory result changes associated with infusions include elevated antiviral or antibacterial antibody titers for 1 mo, 6-fold increase in ESR for 2-3 wk, and apparent hyponatremia
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| Overview: Lupus Erythematosus, Bullous |
| Differential Diagnoses & Workup: Lupus Erythematosus, Bullous |
 Treatment & Medication: Lupus Erythematosus, Bullous |
| Follow-up: Lupus Erythematosus, Bullous |
| Multimedia: Lupus Erythematosus, Bullous |
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References
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Further Reading
Keywords
bullous systemic lupus erythematosus, BSLE, bullous eruption of systemic lupus erythematosus, vesiculobullous systemic lupus erythematosus; lupus erythematosus-specific vesiculobullous skin disease, Rowell's syndrome, Rowell syndrome, toxic epidermal necrolysis-like acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus
