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Dermatology > CONNECTIVE TISSUE DISEASES
Lupus Erythematosus, Bullous
Article Last Updated: Jul 13, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Hillary D Johnson, MD, PhD, Staff Physician, Department of Dermatology, New York University School of Medicine
Hillary D Johnson is a member of the following medical societies: Medical Society of the State of New York
Coauthor(s):
Michael Girardi, MD, Program Director, Assistant Professor, Department of Dermatology, Yale University School of Medicine;
Julie V Schaffer, MD, Assistant Professor, Departments of Dermatology and Pediatrics, New York University School of Medicine
Editors: Kathleen David-Bajar, MD, Former Consultant to the Army Surgeon General, Department of Dermatology, Brooke Army Medical Center; Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center; Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine; Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Author and Editor Disclosure
Synonyms and related 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
Background
Bullous systemic lupus erythematosus (BSLE) is an autoantibody-mediated subepidermal blistering disease that occurs in patients with systemic lupus erythematosus (SLE). The diagnosis of BSLE requires the following elements:
- Fulfillment of the American College of Rheumatology criteria for SLE (see Systemic Lupus Erythematosus)
- An acquired vesiculobullous eruption
- Histologic evidence of a subepidermal blister and a predominantly neutrophilic dermal infiltrate
- Direct immunofluorescence (DIF) microscopy demonstrating immunoglobulin G (with or without immunoglobulin A [IgA] and immunoglobulin M) deposits at the basement membrane zone (BMZ)
- Evidence of antibodies to type VII collagen via DIF or indirect immunofluorescence (IIF) on salt-split skin, immunoblotting, immunoprecipitation, enzyme-linked immunosorbent assay (ELISA), or immunoelectron microscopy
All 5 criteria are needed for a diagnosis of type 1 BSLE, whereas only the first 4 criteria are needed to diagnose type 2 (undetermined location of antigen or dermal antigen other than type VII collagen) and type 3 (epidermal antigen) BSLE. Type VII collagen, a component of anchoring fibrils, is also targeted in epidermolysis bullosa acquisita (EBA). However, unlike EBA, BSLE tends to respond dramatically to treatment with dapsone.
Not all blistering eruptions that occur in patients with lupus erythematosus (LE) represent BSLE as defined above. Vesiculobullous skin lesions can also develop as a result of extensive damage to the epidermal basal layer (and even suprabasal keratinocytes) due to an intense interface dermatitis in the setting of LE-specific skin disease. Such patients may present with a severe form of acute or subacute cutaneous LE (SCLE) that resembles erythema multiforme (Rowell syndrome) or toxic epidermal necrolysis (TEN). Because EBA and BSLE share the same target antigen, distinguishing between the 2 may be difficult.
Pathophysiology
The production of autoantibodies represents a central feature of SLE. For example, antinuclear antibodies (ANAs) are detected in almost all affected individuals. In addition to this general tendency, injury to the dermoepidermal junction by the interface dermatitis of cutaneous LE might expose new epitopes and precipitate the development of antibodies that specifically target the BMZ.
The autoantibody repertoire of SLE can include nonpathogenic and pathogenic anti-BMZ antibodies. Patients with nonbullous SLE often have circulating antibodies to various components of the BMZ (including bullous pemphigoid antigens 1 and 2), which may have a role in formation of the lesional lupus band (ie, granular antibody deposition at the BMZ in normal-appearing skin). Although the lupus band appears to co-localize with type VII collagen, the noncollagenous (NC1) 1 domain of this protein does not represent the target antigen for circulating antibodies in SLE patients without clinical blistering.
In patients with BSLE, antibodies directed at the BMZ likely mediate the blistering phenotype by directly interfering with adhesive connections at the dermoepidermal junction and through induction of complement-dependent inflammation that leads to tissue injury and dermoepidermal separation. Proteolytic damage caused by recruited neutrophils contributes to the latter process.
In type 1 BSLE (which accounts for most cases), antibodies against type VII collagen may weaken or block anchoring fibril-mediated connections between the lamina densa of the basement membrane and the papillary dermis. In both EBA and BSLE, antigenic epitopes reside within the NC1 and NC2 domains of type VII collagen, which are localized to the lamina densa and the underlying dermis, respectively. Subepidermal blisters can be induced in mice by the passive transfer of such autoantibodies from EBA patients, but not the Fab fragments alone. This demonstrates that human EBA autoantibodies are pathogenic and that complement-mediated inflammation (which requires the Fc fragment) has an important role in the blistering process. Antibodies recognizing bullous pemphigoid antigen 1, laminin-5, and laminin-6 have also been described in patients with BSLE.
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 LE, acute graft versus host disease, pseudoporphyria, and the classic drug-hypersensitivity syndrome. Fas-Fas ligand interactions have been implicated in the massive keratinocyte apoptosis that characterizes ASAP. TEN-like cutaneous LE must be differentiated from drug-induced TEN occurring in a patient with LE.
Frequency
International
BSLE accounts for 2-3% of cases of autoimmune subepidermal blistering disease, with an estimated incidence of fewer than 0.5 cases per million population per year.
Mortality/Morbidity
- The development of BSLE in patients with SLE does not typically lead to increased mortality. Morbidity depends on the extent of the eruption and the response to therapy. Fortunately, unlike EBA, treatment with dapsone is successful in most cases of BSLE.
- TEN-like LE can result in considerable morbidity and even mortality if extensive areas of skin are denuded, especially in the context of severe systemic manifestations of LE.
Race
Persons of any race can develop BSLE, but it occurs most frequently in African Americans.
Sex
BSLE affects women more often than men, reflecting the female preponderance in SLE.
Age
BSLE most often manifests in the second through fourth decades of life, but it has been reported in children and older adults.
History
- BSLE is characterized by the rapid development of a widespread vesiculobullous eruption. The blistering activity does not necessarily correlate with that of the patient's LE-specific skin or systemic disease, but parallel exacerbations (often involving lupus nephritis) have been described. BSLE occasionally represents the initial clinical manifestation of SLE.
- BSLE may be accompanied by pruritus of variable intensity. Mucosal lesions are often painful.
- Patients can exhibit any of the symptoms associated with SLE. These can include fever; weight loss; fatigue; photosensitivity; arthralgias; arthritis; and manifestations of renal, pulmonary, cardiac, and/or central nervous system disease. See eMedicine's articles in the Internal Medicine section (Systemic Lupus Erythematosus) and Neurology section (Systemic Lupus Erythematosus) for details on the clinical manifestations of SLE.
- Patients with TEN-like acute cutaneous LE often have significant systemic disease activity (eg, lupus nephritis or cerebritis).
Physical
- Morphology of BSLE skin lesions
- Blisters range from large, tense bullae (resembling bullous pemphigoid) to small, grouped vesicles (resembling dermatitis herpetiformis). They often arise on erythematous skin and may be preceded by urticarial papules and plaques. An annular or figurate configuration is occasionally observed. Bullae contain clear or hemorrhagic fluid. Rupture results in erosions and crusts, which typically heal with postinflammatory hyperpigmentation or hypopigmentation.
- Although BSLE was initially defined as a nonscarring bullous disease, a mechanobullous presentation resembling classic EBA has been reported. In such patients, vesicles and bullae arise within noninflamed skin of trauma-prone sites (reflecting skin fragility) and heal with milia and scarring.
- Distribution of BSLE
- BSLE frequently manifests as a widespread, symmetric distribution of skin lesions. The eruption favors the upper part of the trunk, proximal upper extremities (flexural and extensor aspects), neck, and face, but blisters can occur anywhere on the cutaneous surface. Usually, no clear association exists between sun exposure and the development of lesions, which involve both sun-protected and sun-exposed sites.
- Lesions of classic EBA-like BSLE are localized to trauma-prone areas such as the dorsal hands, feet, elbows, and knees.
- Blisters and erosions can also affect the oral, pharyngeal, nasal, and vulvar mucous membranes.
- LE-specific vesiculobullous skin disease
- These vesiculobullous lesions are distinct from BSLE, representing severe variants of acute, subacute, or (rarely) discoid cutaneous LE. The eruptions can develop rapidly or evolve over several weeks.
- In TEN-like acute cutaneous LE, photodistributed diffuse or patchy erythema evolves (usually rapidly) into flaccid bullae (positive Nikolsky sign, unlike BSLE) and widespread sheetlike, full-thickness epidermal detachment.
- TEN-like SCLE manifests as widespread blistering and full-thickness epidermal detachment in the context of preexisting photodistributed annular or papulosquamous skin lesions and anti-Ro/SS-1 and/or anti-La/SS-2 antibody production.
- Involvement the oral, conjunctival, and genital mucosae occurs in some cases of TEN-like LE.
- Erythema multiforme–like lesions occurring in the context of acute, subacute, or (as initially described by Rowell and colleagues in 1963) discoid cutaneous LE have been referred to as Rowell syndrome. The development of these targetoid erythematous plaques with central (or, in the setting of SCLE, peripheral) blistering and erosions is thought to represent a limited form of TEN-like LE. Mucosal involvement is often observed.
- Vesicles or erosions at the advancing edge of active annular subacute LE lesions represent a relatively common observation.
- Other mucocutaneous findings
- Specific cutaneous lesions of acute, subacute, and chronic (discoid) cutaneous lupus are observed in a minority of patients with BSLE. See Lupus Erythematosus, Acute; Lupus Erythematosus, Subacute Cutaneous; and Lupus Erythematosus, Discoid for information on these forms of cutaneous LE.
- Nonspecific cutaneous findings of LE, such as periungual telangiectasias, livedo reticularis, and nonscarring alopecia, also may be present. See Lupus Erythematosus, Acute; Lupus Erythematosus, Subacute Cutaneous; and Lupus Erythematosus, Discoid for details.
- Chilblain lupus can occur in association with Rowell syndrome.
- General examination: Extracutaneous findings of SLE may include joint tenderness and swelling, pallor or petechiae (reflecting hematologic abnormalities), and auscultation evidence of a pulmonary or pericardial effusion. See Systemic Lupus Erythematosus in eMedicine's Internal Medicine section for details.
Causes
- Certain individuals may have a genetic predisposition to develop autoimmunity to BMZ antigens and to SLE. For example, EBA, BSLE, and SLE are all associated with an increased prevalence of the HLA class II DR2 haplotype. The antigen-presenting protein encoded by the DR2-associated DRB1*1501 allele (found in both EBA and BSLE patients) has been postulated to be involved in presenting type VII collagen epitopes to T lymphocytes.
- TEN-like LE can be triggered by intensive ultraviolet exposure.
Bullous Pemphigoid
Dermatitis Herpetiformis
Drug-Induced Bullous Disorders
Epidermolysis Bullosa
Epidermolysis Bullosa Acquisita
Erythema Multiforme
Graft Versus Host Disease
Hydroa Vacciniforme
Linear IgA Dermatosis
Porphyria Cutanea Tarda
Pseudoporphyria
Other Problems to be Considered
The clinical, histological, and immunohistochemical features of BSLE can resemble EBA (the inflammatory more often than the classic mechanobullous form), dermatitis herpetiformis, and bullous pemphigoid.
TEN-like LE demonstrates clinical and histologic overlap with drug-induced TEN and TEN-like variants of acute graft versus host disease and pseudoporphyria. A similar clinical presentation is occasionally observed in patients with fulminant linear IgA bullous dermatosis (especially the drug-induced variety).
Lab Studies
- BSLE occurs in the setting of SLE; thus, ANA test results generally are positive. Anti-dsDNA, anti-Sm, anti-Ro/SS-A, anti-La/SS-B, and anticardiolipin antibodies may also be detected.
- Other laboratory abnormalities related to SLE can include low levels of complement (ie, C3, C4, CH50), anemia, leukopenia, thrombocytopenia, proteinuria or cellular casts upon urinalysis, and an elevated erythrocyte sedimentation rate. See Systemic Lupus Erythematosus or Nephritis, Lupus for details.
- Laboratory findings in persons with TEN-like LE vary depending on the underlying type of LE present.
- Although the LE patients with erythema multiforme–like skin lesions who were originally described by Rowell and colleagues had immunologic features that included a speckled pattern of ANAs, anti-Ro/SS-A and/or La/SS-B antibodies, and a positive rheumatoid factor, these findings are not consistently observed in LE patients with this clinical presentation. Anticardiolipin antibodies and lupus anticoagulant have also been reported in individuals with Rowell syndrome.
Procedures
- Diagnosis of BSLE requires skin biopsy specimens for both routine light microscopy (preferably from the edge of a fresh blister, including adjacent clinically unblistered skin) and DIF microscopy (from perilesional skin).
Histologic Findings
Light microscopy in BSLE lesions
Histologically, BSLE is characterized by dermoepidermal separation beneath an intact epidermis. A predominantly neutrophilic infiltrate is observed in an edematous upper dermis, either concentrated in the dermal papillae (similar to dermatitis herpetiformis) or distributed in a continuous band beneath the BMZ (similar to linear IgA bullous dermatosis). Nuclear dust is often apparent, and a variable number of eosinophils and mononuclear cells are present within the dermal infiltrate. Basal keratinocyte vacuolization and other histologic features of cutaneous LE are typically absent.
Immunofluorescence microscopy in BSLE lesions
DIF microscopy of perilesional skin reveals deposition of immunoglobulin G (with and without immunoglobulin M and IgA) and complement in a linear or granular bandlike pattern at the BMZ.
In type 1 BSLE, DIF of salt-split perilesional skin and/or IIF microscopy following incubation of the patient's serum with salt-split normal human skin reveals immunoglobulin deposits localized to the dermal floor of the split. Immunoblot and immunoprecipitation studies using the patient's serum can confirm that these antibodies recognize the 290- and 145-kd proteins of type VII collagen; an ELISA for rapid detection of anti–type VII collagen NC1 domain antibodies has also been developed. In addition, direct immunoelectron microscopy can be used to demonstrate that the immunoglobulin deposits are co-distributed with anchoring fibrils/type VII collagen within and just below the lamina densa.
Cases with negative IIF and an undetermined sublocalization of anti-BMZ antibodies (or antibodies recognizing a dermal antigen other than collagen VII) are referred to as type 2 BSLE. Of note, circulating antibodies to type VII collagen are less likely to be present in patients with a granular pattern of fluorescence on DIF. BSLE characterized by immunoglobulin binding the epidermal roof (type 3 BSLE) or both the roof and the dermal floor of salt-split skin has also been observed. Antigens in such patients have included bullous pemphigoid antigen 1, laminin-5, and laminin-6.
Histologic findings in TEN- and erythema multiforme–like LE lesions
The histologic features of TEN-like LE consist of full-thickness epidermal necrosis and a sparse lymphocytic infiltrate in the upper dermis, as is seen in drug-induced TEN. Rowell syndrome is characterized by a vacuolar interface dermatitis with lymphocyte exocytosis, prominent necrotic keratinocytes in all layers of the epidermis, a mild superficial perivascular lymphocytic infiltrate, and dermal edema, features suggestive of erythema multiforme but also compatible with early, hyperacute cutaneous LE. DIF findings are usually negative.
Medical Care
- BSLE generally responds well to medical therapy, and treatment with dapsone is particularly effective. Although type 1 BSLE and EBA are both characterized by antibodies targeting type VII collagen, EBA differs considerably in its marked resistance to therapy.
- Dapsone is the initial treatment of choice for BSLE. 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 SLE. Combination therapy with prednisone and dapsone can also be beneficial.
- Methotrexate (MTX), azathioprine, and mycophenolate mofetil represent additional therapeutic options.
- Extensive eruptions of TEN-like LE require prompt institution of therapy with intravenous immunoglobulin and/or systemic corticosteroids. Less fulminant manifestations of erythema multiforme–like LE 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 LE. See Lupus Erythematosus, Subacute Cutaneous.
Consultations
- Dermatologist - For evaluation and management of BSLE, TEN- or erythema multiforme–like LE, and other cutaneous manifestations of LE
- Internist/rheumatologist - For evaluation and management of extracutaneous (eg, renal, cardiac, pulmonary) manifestations of SLE
BSLE generally responds well to medical therapy. Dapsone represents the mainstay of treatment, typically leading to rapid clearance of lesions.
As in drug-induced TEN, intravenous immunoglobulin represents an important therapeutic option for the Fas-mediated massive epidermal necrosis of fulminant TEN-like LE.
Drug Category: Antimycobacterial agents
Therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.
| Drug Name | Dapsone (Avlosulfon) |
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| Description | 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. |
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| Adult Dose | 25-200 mg/d PO |
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| Pediatric Dose | 1-2 mg/kg/d PO; not to exceed 100 mg/d |
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| Contraindications | Documented hypersensitivity; G-6-PD deficiency |
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| Interactions | 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 |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
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| 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 |
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Drug Category: Corticosteroids
Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.
| Drug Name | Prednisone (Deltasone, Orasone, Sterapred) |
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| Description | 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. |
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| Adult Dose | 0.5-1.5 mg/kg/d PO for several wk, with slow tapering |
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| Pediatric Dose | Administer as in adults |
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| Contraindications | Documented hypersensitivity; active viral, fungal, or mycobacterial infections; peptic ulcer disease, hepatic dysfunction |
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| Interactions | Live or attenuated vaccines; coadministration with estrogens may decrease clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
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| Pregnancy | B - Usually safe but benefits must outweigh the risks.
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| 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 |
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Drug Category: Antirheumatic agents
Have anti-inflammatory effects.
| Drug Name | Methotrexate (Folex PFS, Rheumatrex) |
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| Description | 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). |
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| Adult Dose | 7.5-25 mg/wk, PO/IM; folic acid 1 mg/d is given concomitantly |
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| Pediatric Dose | 0.2-0.6 mg/kg/wk PO/IM/SC; folic acid 1 mg/d is given concomitantly |
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| Contraindications | Documented hypersensitivity; alcoholism; hepatic insufficiency; documented immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia); renal insufficiency |
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| Interactions | 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 |
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| Pregnancy | X - Contraindicated in pregnancy
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| 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) |
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Drug Category: Blood products
Used to improve clinical and immunologic aspects of the disease. May decrease autoantibody production and increase solubilization and removal of immune complexes.
| Drug Name | Immune globulin intravenous (Gammar-P, Sandoglobulin, Gammagard) |
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| Description | Antibody-mediated blockade of Fas-Fas ligand interactions involved in the epidermal necrosis of TEN-like cutaneous LE. |
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| Adult Dose | 0.75-1 g/kg/d IV for 3-7 d |
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| Pediatric Dose | Administer as in adults |
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| Contraindications | Documented hypersensitivity; IgA deficiency |
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| Interactions | Globulin preparation may interfere with immune response to live virus vaccine (MMR) and reduce efficacy (do not administer within 3 mo of vaccine) |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
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| 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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Prognosis
- The course of BSLE is often remitting, and it tends to respond well to therapy with dapsone. The disorder frequently resolves spontaneously in less than 1 year.
Patient Education
Medical/Legal Pitfalls
- Appropriate diagnosis of BSLE requires that patients with subepidermal blistering disorders characterized by a primarily neutrophilic infiltrate and DIF/IIF findings suggestive of EBA are evaluated for clinical and laboratory signs of SLE.
| Media file 1:
Tense vesiculobullous lesions on the neck of a patient with bullous systemic lupus erythematosus. |
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Media type: Photo
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Lupus Erythematosus, Bullous excerpt Article Last Updated: Jul 13, 2006
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