p.1
Antinuclear Antibodies (ANA) Testing Methods
What is considered a positive ANA titer?
A titer greater than 1:40.
p.7
Treatment Approaches for Cutaneous Lupus
What is the first-line treatment for Subacute Cutaneous Lupus Erythematosus (SCLE)?
Antimalarials and sun protection.
p.10
Connective Tissue Diseases (CTDs) Overview
What is the relationship between SLE and pregnancy?
The course may be stable, worsen, or improve; patients with lupus nephritis are at increased risk of complications, especially during the postpartum period.
p.8
Neonatal Lupus Erythematosus (NLE) Epidemiology and Pathogenesis
What is the prognosis for children with Neonatal Lupus Erythematosus (NLE)?
Children with NLE may be at increased risk of developing Systemic Lupus Erythematosus (SLE) or autoimmunity later in life.
p.9
Chronic Cutaneous Lupus Erythematosus (CCLE) Characteristics
What is the histopathological feature of Acute Cutaneous Lupus Erythematosus (ACLE)?
Vacuolar interface dermatitis, dermal edema, and sparse perivascular lymphocytic infiltrate limited to upper dermis.
p.10
Treatment Approaches for Cutaneous Lupus
What is the first-line treatment for bullous SLE?
Dapsone is the treatment of choice and shows a dramatic response within 1-2 days.
p.1
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What is the significance of high titers of RNP (U1 RNP) antibodies?
They correlate with MCTD (100%).
p.5
Forms of Cutaneous Lupus and Their Associations
What is the overlap between discoid lupus and lichen planus?
Coexisting DLE and lichen planus with overlap lesions, characterized by palmoplantar involvement.
p.2
Chronic Cutaneous Lupus Erythematosus (CCLE) Characteristics
What are the clinical features of chilblain lupus erythematosus?
Chilblain lupus erythematosus presents as red or dusky purple papules/plaques on fingertips, rims of ears, calves, and heels.
p.6
Histopathological Features of Cutaneous Lupus
What are the typical histopathological features of mucosal lupus erythematosus?
Hyperkeratosis, atrophy of rete pegs, vacuolar-to-lichenoid interface dermatitis, and superficial and deep perivascular lymphocytic infiltrate.
p.8
Neonatal Lupus Erythematosus (NLE) Epidemiology and Pathogenesis
What are the common hematologic abnormalities seen in NLE?
Common hematologic abnormalities include thrombocytopenia, neutropenia, lymphopenia, and hemolytic anemia.
p.7
Neonatal Lupus Erythematosus (NLE) Epidemiology and Pathogenesis
What are the clinical features of Neonatal Lupus Erythematosus (NLE)?
Lesions arise within the first weeks of life, often with photosensitivity and periorbital erythema (raccoon eyes).
p.10
Connective Tissue Diseases (CTDs) Overview
What are the key epidemiological features of SLE?
80% of SLE patients will have skin findings, with a female predominance and a higher incidence in African Americans.
p.5
Lupus Band Test (LBT) and Its Variants
What is the significance of DIF in diagnosing discoid lupus?
DIF shows positive LBT on lesional skin in 75%, more likely to be positive on head/neck and extremities compared with trunk.
What are the laboratory abnormalities associated with complement levels in SLE?
Complement abnormalities include decreased total complement levels and autoantibodies against C1q.
p.10
Forms of Cutaneous Lupus and Their Associations
What are some cutaneous manifestations specific to SLE?
Lupus-specific skin findings include ACLE, SCLE, CCLE, Rowell syndrome, TEN-like LE, and Bullous SLE.
p.9
Forms of Cutaneous Lupus and Their Associations
What are the common locations for Bullous SLE lesions?
Face, neck, upper trunk, proximal extremities, and mucosa.
p.2
Forms of Cutaneous Lupus and Their Associations
What are the three major forms of cutaneous lupus?
The three major forms of cutaneous lupus are acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), and chronic cutaneous lupus erythematosus (CCLE).
p.2
Chronic Cutaneous Lupus Erythematosus (CCLE) Characteristics
What is the typical location for hypertrophic lupus erythematosus lesions?
Hypertrophic lupus erythematosus lesions typically occur on sun-exposed sites such as the extensor forearms, face, and upper trunk.
p.3
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What condition is associated with high titers of U1RNP antibodies?
Mixed Connective Tissue Disease (MCTD)
p.6
Subacute Cutaneous Lupus Erythematosus (SCLE) Pathogenesis
What is the prognosis for patients with SCLE regarding progression to SLE?
5%–20% progress to SLE, with increased risk in widespread DLE and childhood DLE.
p.9
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What is the significance of anti-C1q autoantibodies in SLE patients?
They arise in 30%-50% of SLE patients and are associated with lupus nephritis.
p.5
Forms of Cutaneous Lupus and Their Associations
What findings are associated with lupus erythematosus panniculitis?
May have histologic findings of overlying DLE without clinical findings of DLE, including dermal mucin deposition and lymphocytic lobular panniculitis.
p.2
Chronic Cutaneous Lupus Erythematosus (CCLE) Characteristics
What are the typical clinical features of discoid lupus erythematosus (DLE)?
DLE begins with red macules or plaques that develop scale, atrophy, and scarring, with central hypopigmentation and peripheral hyperpigmentation.
p.11
Treatment Approaches for Cutaneous Lupus
What is the treatment for severe active SLE with renal involvement?
The treatment includes high-dose prednisone and pulsed IV cyclophosphamide or MMF.
p.1
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
Which ANA pattern is associated with anti-dsDNA antibodies?
Peripheral (rim) pattern.
p.10
Connective Tissue Diseases (CTDs) Overview
What laboratory tests are commonly performed for SLE?
Common tests include ANA, ELISA for autoantibodies to collagen VII, and salt-split skin testing for dermal reactivity.
p.1
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What is the association of Scl-70 antibodies?
Most strongly associated with diffuse SSc (60%).
p.2
Lupus Band Test (LBT) and Its Variants
What is the significance of the Lupus Band Test (LBT)?
The LBT shows a granular continuous band of immunoglobulin deposits at the dermal-epidermal junction in patients with SLE, helping to differentiate from other rashes.
p.2
Chronic Cutaneous Lupus Erythematosus (CCLE) Characteristics
What triggers chronic cutaneous lupus erythematosus (CCLE)?
Ultraviolet radiation (UVR), particularly UVB, is an important trigger for all CCLE subtypes.
p.11
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What is the significance of anti-dsDNA antibodies in SLE?
Anti-dsDNA antibodies are not sensitive but highly specific for SLE and are useful in monitoring disease activity.
p.7
Neonatal Lupus Erythematosus (NLE) Epidemiology and Pathogenesis
What is the epidemiology of Neonatal Lupus Erythematosus (NLE)?
There is a female predominance for NLE of skin (3:1) and cardiac NLE (2:1).
p.7
Neonatal Lupus Erythematosus (NLE) Epidemiology and Pathogenesis
What is the primary cause of Neonatal Lupus Erythematosus (NLE)?
Transplacental passage of maternal autoantibodies, most importantly anti-Ro/SS-A (99%).
p.2
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What are the autoantibody associations for nucleolar antibodies?
Nucleolar antibodies are associated with RNA processing molecules (fibrillarin/U3RNP) and are linked to systemic sclerosis (SSc) and polymyositis-SSc overlap.
p.2
Chronic Cutaneous Lupus Erythematosus (CCLE) Characteristics
What is the association of smoking with discoid lupus erythematosus (DLE)?
Smoking is a risk factor for DLE, and cessation may help resolve recalcitrant lesions.
p.11
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What is the role of antiphospholipid antibodies in SLE?
Antiphospholipid antibodies include anti-β2-glycoprotein and anti-anticardiolipin antibodies, which are significant in SLE.
p.7
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What are the common antibodies associated with drug-induced Subacute Cutaneous Lupus Erythematosus (SCLE)?
Anti-Ro/SS-A (80%) and anti-La/SS-B.
p.9
Forms of Cutaneous Lupus and Their Associations
What is the typical clinical presentation of Bullous SLE?
Widespread, symmetric eruption of tense, subepidermal bullae on an erythematous-to-urticarial base.
p.5
Forms of Cutaneous Lupus and Their Associations
What characterizes Chilblain lupus erythematosus?
Demonstrates features of both chilblains (papillary edema, perivascular and dermal lymphohistiocytic infiltration) and DLE. DIF shows positive LBT.
p.3
Connective Tissue Diseases (CTDs) Overview
What is the most common pattern of ANA in Systemic Sclerosis?
Speckled, nucleolar, centromere (CREST)
p.2
Chronic Cutaneous Lupus Erythematosus (CCLE) Characteristics
What is the female predominance in chronic cutaneous lupus erythematosus (CCLE)?
CCLE shows a female predominance, with discoid lupus erythematosus (DLE) accounting for the majority of cases.
What laboratory findings are associated with SLE?
Routine labs may show increased inflammatory markers, hemolytic anemia, leukopenia, thrombocytopenia, proteinuria, and hematuria.
p.3
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What is the most sensitive serologic test for Systemic Lupus Erythematosus (SLE)?
ANA (Antinuclear Antibodies)
p.9
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What laboratory tests are recommended for ACLE?
Perform the same lab studies as in SLE to search for end-organ damage.
p.5
Forms of Cutaneous Lupus and Their Associations
What are the histologic features of Hypertrophic (verrucous) lupus erythematosus (LE)?
Similar histologic features as DLE but with greater orthohyperkeratosis and endophytic buds of hyperplastic follicular epithelium. Pseudoepitheliomatous hyperplasia is often mistaken for SCC.
p.7
Forms of Cutaneous Lupus and Their Associations
What are the typical locations for lesions in Subacute Cutaneous Lupus Erythematosus (SCLE)?
Sun-exposed areas of the lateral face, neck, V-chest, and upper back/extremities.
p.9
Chronic Cutaneous Lupus Erythematosus (CCLE) Characteristics
What is the relationship between cutaneous flares and systemic disease in ACLE?
Cutaneous flares tend to correlate with systemic disease activity.
p.5
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What laboratory findings are associated with discoid lupus?
(+/-) ANA, leukopenia, and increased ESR; serologic abnormalities are more common in patients with widespread DLE.
p.7
Subacute Cutaneous Lupus Erythematosus (SCLE) Pathogenesis
What are the histopathological features of Subacute Cutaneous Lupus Erythematosus (SCLE)?
Compact hyperkeratosis, prominent epidermal atrophy, vacuolar interface dermatitis with pigment incontinence, BMZ thickening, PV/PA lymphoid aggregates limited to superficial dermis with scattered plasma cells, and mucin deposition. Lacks eosinophils and follicular plugging.
p.8
Forms of Cutaneous Lupus and Their Associations
How do skin lesions associated with NLE typically resolve?
Skin lesions resolve without scarring by approximately 6 months as maternal antibodies clear from neonatal circulation.
p.6
Treatment Approaches for Cutaneous Lupus
What is the recommended treatment for severe and widespread skin manifestations of SCLE?
Topical treatment combined with hydroxychloroquine or chloroquine.
p.10
Connective Tissue Diseases (CTDs) Overview
What genetic factors are associated with SLE?
Strong genetic components include susceptibility loci such as genes encoding early complement components (C1, C2, C4), TREX1, and ITGAM.
p.5
Forms of Cutaneous Lupus and Their Associations
What are the typical lesions seen in mucosal lupus erythematosus?
Classic plaque with central erythema and surrounding white keratotic border, usually on hard palate, and discoid lesions most commonly on the lip.
p.11
Connective Tissue Diseases (CTDs) Overview
What are the criteria for a malar rash in SLE classification?
A malar rash is defined as fixed erythema over the malar eminences, tending to spare the nasolabial folds.
p.4
Forms of Cutaneous Lupus and Their Associations
What is the characteristic feature of lupus erythematosus panniculitis/profundus?
Indurated, non-tender, subcutaneous nodules or plaques that heal with atrophy
p.10
Connective Tissue Diseases (CTDs) Overview
What are the ACR criteria for diagnosing SLE?
Need to satisfy four items (at least one clinical and one immunologic item) OR have biopsy-proven nephritis compatible with SLE in the presence of ANA or anti-dsDNA antibodies.
p.10
Forms of Cutaneous Lupus and Their Associations
What are non-specific skin findings suggestive of SLE?
Non-specific findings include diffuse non-scarring alopecia, periungal telangiectasias, non-specific mucosal ulcers, and various forms of vasculitis.
p.6
Treatment Approaches for Cutaneous Lupus
What topical agents are included in the treatment algorithm for cutaneous lupus erythematosus?
Topical steroids, calcineurin inhibitors, and retinoids.
p.8
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What is the risk of developing SLE in individuals with homozygous C2 deficiency?
Only 10%–20% of individuals with homozygous C2 deficiency will develop SLE, indicating a low risk.
p.8
Treatment Approaches for Cutaneous Lupus
What treatment is recommended for skin disease in NLE?
Sun protection and topical corticosteroids are recommended for skin disease.
p.5
Histopathological Features of Cutaneous Lupus
What histopathological features are characteristic of discoid lupus?
Compact orthokeratosis, vacuolar interface dermatitis with necrotic keratinocytes, pigment incontinence, epidermal atrophy, BMZ thickening, and follicular plugging.
p.11
Connective Tissue Diseases (CTDs) Overview
What is the definition of serositis in the context of SLE classification?
Serositis can be pleuritis or pericarditis, documented by history or ECG.
p.1
Antinuclear Antibodies (ANA) Testing Methods
What are the two methods used to detect ANAs?
ELISA (newer, cheaper method) and IIF (older but more sensitive method).
p.6
Subacute Cutaneous Lupus Erythematosus (SCLE) Pathogenesis
What is a proposed mechanism for the pathogenesis of SCLE?
UVR-induced apoptosis leading to release of proinflammatory cytokines and production of ANAs, particularly anti-Ro/SS-A autoantibodies.
p.8
Neonatal Lupus Erythematosus (NLE) Epidemiology and Pathogenesis
What is the mortality rate associated with cardiac disease in NLE?
Cardiac NLE has a mortality rate of 20%–30%.
p.8
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What is the most common hereditary complement disorder associated with SLE?
Primary C2 deficiency is the most common hereditary complement disorder.
p.5
Forms of Cutaneous Lupus and Their Associations
What are the histological features of Tumid lupus erythematosus?
No significant epidermal changes, shares characteristic dermal features of DLE, including PV/PA lymphoid aggregates and massive mucin deposition. DIF shows positive LBT in 50%.
p.10
Connective Tissue Diseases (CTDs) Overview
What environmental triggers are known to affect SLE?
Environmental triggers include sunlight, cigarettes, infections, vitamin D deficiency, and estrogen.
p.11
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What is the specificity and sensitivity of Anti-Smith antibodies for SLE?
Anti-Smith antibodies are not sensitive, but highly specific for SLE.
p.11
Antinuclear Antibodies (ANA) Testing Methods
What is the significance of the ANA test in SLE?
ANA is positive in 99% of SLE cases, indicating an autoimmune response.
p.9
Forms of Cutaneous Lupus and Their Associations
What characterizes the butterfly rash in ACLE?
Localized erythema on the nasal bridge and bilateral malar eminence, sparing the melolabial folds.
p.8
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What are the clinical findings associated with C2 deficiency?
C2 deficiency is associated with adult onset SLE, prominent photosensitivity, and increased bacterial infections with encapsulated bacteria.
p.8
Neonatal Lupus Erythematosus (NLE) Epidemiology and Pathogenesis
What prenatal treatment may reduce the risk of congenital heart block in NLE?
Prenatal systemic corticosteroids may reduce the risk of developing congenital heart block.
p.11
Treatment Approaches for Cutaneous Lupus
What is the treatment for mild active SLE without life-threatening organ involvement?
The treatment includes hydroxychloroquine and NSAIDs.
p.7
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
What systemic manifestations are common in Subacute Cutaneous Lupus Erythematosus (SCLE)?
Systemic manifestations are common, but only 30%-50% fully meet criteria for SLE, with arthritis/arthralgias being the most common finding (up to 70%).
p.9
Neonatal Lupus Erythematosus (NLE) Epidemiology and Pathogenesis
What is the typical demographic for Bullous SLE?
Predominantly affects females and African Americans.
p.11
Autoantibody Associations in Systemic Lupus Erythematosus (SLE)
Which autoantibody is most important in association with Mixed Connective Tissue Disease (MCTD)?
Anti-U1RNP antibodies are associated with MCTD and have lower titers in SLE.
