61.2: Lupus Erythematosus

Question 1

What are the laboratory testing indicators for Acute Cutaneous Lupus Erythematosus (ACLE)?

Answer 1

High-titer ANA, anti-dsDNA, anti-Smith antigen, hypocomplementemia, hypergammaglobinemia, cytopenias, decreased kidney function, and urine changes reflect disease activity.

Question 2

What are the laboratory testing indicators for Subacute Cutaneous Lupus Erythematosus (SCLE)?

Answer 2

Indicators include anti-Ro/SS-A (70-90%), anti-La/SS-B (30-50%), ANA (60-80%), rheumatoid factor (approximately 33%), false-positive VDRL and RPR (7-33%), anticardiolipin (10-16%), antithyroid (18-44%), anti-Smith antigen (10%), anti-dsDNA (10%), and anti-U1 ribonucleoprotein (10%).

Question 3

What is the significance of anti-Ro/SS-A antibodies in SCLE?

Answer 3

Anti-Ro/SS-A antibodies are present in 70-90% of SCLE cases and are associated with the disease’s pathogenesis.

Question 4

What are the laboratory findings in SCLE?

Answer 4

Findings include anti-Ro/SS-A (70-90%), anti-La/SS-B (30-50%), ANA (60-80%), rheumatoid factor (33%), false-positive VDRL and RPR (7-33%), anticardiolipin (10-16%), and antithyroid antibodies (18-44%).

Question 5

What are the key laboratory tests and their significance in diagnosing Acute Cutaneous Lupus Erythematosus (ACLE)?

Answer 5

Key laboratory tests for ACLE include high-titer ANA, anti-dsDNA, anti-Smith antigen, hypocomplementemia, hypergammaglobinemia, and indicators of kidney function and urine changes reflect disease activity.

Question 6

What laboratory findings are typically associated with Subacute Cutaneous Lupus Erythematosus (SCLE) and their prevalence?

Answer 6

Typical laboratory findings in SCLE include anti-Ro/SS-A (70-90%), anti-La/SS-B (30-50%), ANA (60-80%), rheumatoid factor (approximately 33%), false-positive VDRL and RPR (7-33%), anticardiolipin (10-16%), antithyroid (18-44%), anti-Smith antigen (10%), anti-dsDNA (10%), and anti-U1 ribonucleoprotein (10%).

Question 7

How do the laboratory findings differ between ACLE and SCLE in terms of disease activity indicators?

Answer 7

ACLE shows high-titer ANA, anti-dsDNA, anti-Smith antigen, hypocomplementemia, hypergammaglobinemia, and indicators of kidney function reflecting disease activity, while SCLE has specific autoantibodies like anti-Ro/SS-A and anti-La/SS-B with less emphasis on kidney function changes.

Question 8

What are the common laboratory findings in patients with Discoid Lupus Erythematosus (DLE)?

Answer 8

Common findings include low-titer ANA in 30-40% of patients, higher ANA levels in 5% of patients with overt SLE, anti-ssDNA antibodies, distinctly uncommon anti-dsDNA antibodies, precipitating antibodies to U1 ribonucleoprotein, low levels of anti-Ro/SS-A antibodies, low-grade anemia, and modest leukopenia.

Question 9

What histopathological features are characteristic of Subacute Cutaneous Lupus Erythematosus (SCLE)?

Answer 9

Histopathological features include interface dermatitis with vacuolar alteration of basal keratinocytes, pronounced epidermal atrophy, dermal edema, prominent mucin deposition, and sparse mononuclear cell infiltration.

Question 10

What is the significance of the lupus band test in diagnosing cutaneous lupus erythematosus?

Answer 10

The lupus band test detects immunoglobulins and complement at the dermal-epidermal junction. In ACLE, 60-100% show a lupus band; in SCLE, a ‘dust-like particle’ pattern of IgG deposition is more specific.

Question 11

What is the significance of the lupus band test in diagnosing ACLE?

Answer 11

The lupus band test shows IgG, IgA, IgM, C3, and other complement components deposited at the dermal-epidermal junction, positive in 60-100% of ACLE cases.

Question 12

What are the histopathological features of CCLE?

Answer 12

CCLE shows hyperkeratosis, variable atrophy, thickened basement membrane, dense mononuclear cell infiltrate, and melanophages.

Question 13

What are the histopathological features of SCLE?

Answer 13

SCLE presents as interface dermatitis with vacuolar alteration of basal keratinocytes, pronounced epidermal atrophy, dermal edema, prominent mucin deposition, and sparse mononuclear cell infiltration.

Question 14

What are the histopathological differences between SCLE and CCLE?

Answer 14

SCLE shows interface dermatitis and pronounced epidermal atrophy, while CCLE shows hyperkeratosis and dense mononuclear cell infiltrate extending into the deeper dermis.

Question 15

What are the histopathological features of ACLE?

Answer 15

ACLE shows sparse lymphohistiocytic infiltrate, increased neutrophils, focal vacuolar alteration of basal keratinocytes, telangiectases, extravasation of erythrocytes, and individually necrotic keratinocytes.

Question 16

What are the clinical features of LE profundus?

Answer 16

LE profundus shows immunoglobulin and complement deposits in blood vessel walls of the deep dermis and subcutis, with ANA positive in 70-75% of cases, but anti-dsDNA is rare.

Question 17

What is the significance of the lupus band test in CCLE?

Answer 17

In CCLE, the lupus band test is more frequently positive in lesions on the head, neck, and arms (80%) than on the trunk (20%).

Question 18

What are the laboratory findings in DLE?

Answer 18

Findings include low-titer ANA in 30-40%, anti-ssDNA (not uncommon), anti-dsDNA (distinctly uncommon), and sometimes precipitating antibodies to U1 ribonucleoprotein.

Question 19

What are the common laboratory findings in patients with Discoid Lupus Erythematosus (DLE)?

Answer 19

Common findings include low-titer ANA in 30-40% of patients, higher ANA levels in 5% of patients with overt SLE, anti-ssDNA antibodies, distinctly uncommon anti-dsDNA antibodies, precipitating antibodies to U1 ribonucleoprotein, low levels of anti-Ro/SS-A antibodies, and low-grade anemia.

Question 20

How does the histopathological presentation of Subacute Cutaneous Lupus Erythematosus (SCLE) differ from that of Discoid Lupus Erythematosus (DLE)?

Answer 20

SCLE shows pronounced epidermal atrophy and sparse mononuclear cell infiltration, while DLE shows hyperkeratosis and dense mononuclear cell infiltration.

Question 21

What is the clinical significance of the presence of a lupus band in patients with Cutaneous Lupus Erythematosus (CLE)?

Answer 21

The presence of a lupus band indicates strong association with the disease, being positive in 60-100% of ACLE patients, and more specific patterns in SCLE.

Question 22

What is the significance of the presence of 3 or more immunoreactants in non-lesional skin for SLE diagnosis?

Answer 22

The presence of 3 or more immunoreactants correlates positively with the risk for developing lupus nephritis and indicates a high specificity for SLE.

Question 23

What are the ominous prognostic signs associated with SCLE?

Answer 23

Ominous prognostic signs for SCLE include hypertension, nephritis, systemic vasculitis, and CNS disease.

Question 24

What is the typical clinical course of ACLE?

Answer 24

ACLE can be localized or generalized, with flares and remissions associated with underlying SLE. The survival rate is approximately 80-95% at 5 years and 70-90% at 10 years.

Question 25

What percentage of patients with SCLE develop active SLE, and what are some associated risk factors?

Answer 25

Approximately 10% of patients with SCLE develop active SLE, including lupus nephritis. Risk factors include papulosquamous SCLE, localized ACLE, high-titer ANA, leukopenia, and/or antibodies to dsDNA.

Question 26

What are the recommended local therapy measures for sun protection in patients with CLE?

Answer 26

Recommended measures include wearing tightly woven clothing, broad-brimmed hats, and using broad-spectrum, water-resistant sunscreens (SPF ≥30) with an efficient ultraviolet A blocking agent.

Question 27

What are the ominous prognostic signs in ACLE?

Answer 27

Ominous signs include hypertension, nephritis, systemic vasculitis, and CNS disease.

Question 28

What are the clinical features of ACLE?

Answer 28

ACLE can be localized or generalized, flaring and abating with underlying SLE. Five-year survival rates are 80-95%, and 10-year rates are 70-90%.

Question 29

A patient with SCLE presents with intermittent recurrences and long-term remissions. What are the risk factors for developing active SLE in such patients?

Answer 29

Risk factors include papulosquamous SCLE, localized ACLE, high-titer ANA, leukopenia, and/or antibodies to dsDNA.

Question 30

What is the recommended sun protection strategy for patients with CLE?

Answer 30

Patients should wear tightly woven clothing, broad-brimmed hats, and regularly use broad-spectrum, water-resistant sunscreens (SPF ≥30) with an efficient ultraviolet A blocking agent.

Question 31

What are the clinical features of reticulated erythematosus mucinosis?

Answer 31

It presents as a reticulated array of macules and/or papules on the upper chest and back and is related to LE tumidus.

Question 32

What are the potential complications of untreated classic DLE?

Answer 32

Untreated classic DLE can lead to indolent progression to large areas of cutaneous dystrophy, scarring alopecia, and occasionally squamous cell carcinoma.

Question 33

What are the clinical features of SCLE?

Answer 33

SCLE is characterized by intermittent recurrences, long-term remissions, or unremitting cutaneous disease. About 10% of patients develop active SLE, including lupus nephritis.

Question 34

What are the clinical features of CCLE?

Answer 34

CCLE can lead to large areas of cutaneous dystrophy, scarring alopecia, and occasionally squamous cell carcinoma. Spontaneous remission is rare.

Question 35

What are the clinical implications of the presence of 3 or more immunoreactants in nonlesional skin for patients with SLE?

Answer 35

The presence of 3 or more immunoreactants correlates positively with the risk for developing lupus nephritis, indicating a higher likelihood of systemic involvement in SLE.

Question 36

What is the significance of the survival rates for ACLE and SCLE in terms of prognosis?

Answer 36

The survival rates for ACLE are 80-95% at 5 years and 70-90% at 10 years, while SCLE patients may experience intermittent recurrences and have a 10% chance of developing active SLE.

Question 37

How does the management of ACLE differ from that of SCLE and CCLE?

Answer 37

Management of ACLE typically involves systemic immunosuppressive measures, while SCLE and CCLE prefer nonimmunosuppressive treatment modalities.

Question 38

What are the prognosis rates for ACLE and SCLE?

Answer 38

ACLE are 80-95% at 5 years and 70-90% at 10 years, while SCLE patients may experience intermittent recurrences and have a 10% chance of developing active SLE, including lupus nephritis.

Question 39

What are the recommended local therapy strategies for sun protection in patients with CLE?

Answer 39

Recommended strategies include wearing tightly woven clothing, broad-brimmed hats, and using broad-spectrum, water-resistant sunscreens (SPF ≥30) with effective ultraviolet A blocking agents such as avobenzone or micronized zinc oxide.

Question 40

What are the recommended topical glucocorticoids for treating Cutaneous Lupus Erythematosus (CLE)?

Answer 40

- **Triamcinolone acetonide 0.1%** for the face, applied 2x daily for 2 weeks followed by a 2-week rest. - **Superpotent topical agents** like clobetasol propionate 0.05% or betamethasone dipropionate 0.05% are also effective. - **Topical calcineurin inhibitors** such as pimecrolimus 1% cream and tacrolimus 0.1% ointment can be used.

Question 41

What is the role of antimalarials in the treatment of Cutaneous Lupus Erythematosus (CLE)?

Answer 41

- **Hydroxychloroquine sulfate (Plaquenil)**: 6 to 6.5 mg/kg of lean body mass daily; may take 2-3 months for therapeutic benefit. - If no response after 8-12 weeks, add **quinacrine 100mg/day** (no risk for retinopathy). - **Chloroquine diphosphate (Aralen)**: 3 to 4 mg/kg/day, more retinotoxic than HCQ. - **Quinacrine**: 100mg/day, with side effects including headache and hematologic toxicity.

Question 42

What are the potential side effects of quinacrine when used for CLE treatment?

Answer 42

- **Headache** - **GI intolerance** - **Hematologic toxicity** - **Pruritus** - **Lichenoid drug eruptions** - **Mucosal or cutaneous pigmentary deposition** - **Yellow discoloration** of the skin and sclera in fair-skinned individuals (reversible) - **Significant hemolysis** in G6PD deficient patients.

Question 43

What precautions should be taken before starting antimalarial therapy for CLE?

Answer 43

- A **pretreatment ophthalmologic examination** should be performed due to the risk of antimalarial retinopathy. - **CBC**, liver, and renal function tests should be conducted before treatment and repeated 4 to 6 weeks after therapy initiation, and every 4 to 6 months thereafter.

Question 44

What are the recommended dosages for dapsone in the treatment of refractory CLE?

Answer 44

- **Dapsone**: Start with 25 mg by mouth twice daily, which can be increased up to **200 to 400 mg/day**. - Monitor for dose-related hemolysis and methemoglobinemia, especially in G6PD deficient patients.

Question 45

What are the adverse effects of chloroquine in CLE treatment?

Answer 45

Chloroquine is more retinotoxic than hydroxychloroquine and should not be used simultaneously with it. Doses must be adjusted for decreased renal or hepatic function.

Question 46

What are the systemic side effects of thalidomide in CLE treatment?

Answer 46

Side effects include teratogenicity, sensory neuropathy, somnolence, constipation, and thromboembolism. Neuropathy correlates with total treatment time.

Question 47

How should hydroxychloroquine sulfate be administered for CLE, and what are the precautions?

Answer 47

Hydroxychloroquine sulfate should be given at 6 to 6.5 mg/kg of lean body mass daily, either once daily or in two divided doses to prevent GI side effects. Pretreatment ophthalmologic examination is required due to the rare risk of antimalarial retinopathy.

Question 48

A patient with CLE is unresponsive to hydroxychloroquine after 8-12 weeks. What is the next step in treatment?

Answer 48

Add quinacrine 100 mg/day, as it has no risk for retinopathy. If no control is achieved after 4-6 weeks with HCQ+Quinacrine, consider switching to chloroquine.

Question 49

What are the potential side effects of quinacrine in CLE treatment?

Answer 49

Side effects include headache, GI intolerance, hematologic toxicity, pruritus, lichenoid drug eruptions, mucosal or cutaneous pigmentary deposition, yellow discoloration of the skin and sclera (reversible), and significant hemolysis in G6PD-deficient patients.

Question 50

What is the role of topical calcineurin inhibitors in CLE treatment?

Answer 50

- **Pimecrolimus 1% cream** and **tacrolimus 0.1% ointment** have demonstrated efficacy in treating ACLE, DLE, and SCLE. - Pimecrolimus 1% cream has shown equal efficacy with betamethasone valerate 0.1% cream in treating facial DLE.

Question 51

What are the risks associated with thalidomide in CLE treatment?

Answer 51

Risks include teratogenicity, sensory neuropathy (25-75% of patients develop peripheral neuropathy), somnolence, constipation, and thromboembolism. Neuropathy correlates with total treatment time, so short courses are preferred.

Question 52

What are the systemic side effects of isotretinoin and acitretin in CLE treatment?

Answer 52

Side effects include teratogenicity, dryness, hyperlipidemia, and potential breakthrough of CLE activity.

Question 53

What is the role of topical tacrolimus in CLE treatment?

Answer 53

Topical tacrolimus 0.3% compounded in clobetasol propionate 0.05% is used for recalcitrant CLE.

Question 54

What are the potential side effects of dapsone in CLE treatment?

Answer 54

Side effects include dose-related hemolysis, methemoglobinemia (especially in G6PD-deficient patients), and the need for regular CBC and liver function tests.

Question 55

What are the recommended topical glucocorticoids for treating Cutaneous Lupus Erythematosus (CLE) and their application frequency?

Answer 55

- **Triamcinolone acetonide 0.1%**: Apply 2 times daily for 2 weeks followed by a 2-week rest. - **Superpotent topical agents** (e.g., clobetasol propionate 0.05%, betamethasone dipropionate 0.05%): Produce the greatest benefit in CLE.

Question 56

What is the role of topical calcineurin inhibitors in the treatment of CLE?

Answer 56

- **Pimecrolimus 1% cream** and **tacrolimus 0.1% ointment** have shown efficacy in treating ACLE, DLE, and SCLE. - Topical calcineurin can be used daily during rest periods.

Question 57

What are the systemic therapy options for Cutaneous Lupus Erythematosus (CLE) and their potential side effects?

Answer 57

- **Hydroxychloroquine sulfate (Plaquenil)**: 6 to 6.5 mg/kg of lean body mass daily; delayed onset of therapeutic benefit, risk of retinopathy. - **Chloroquine diphosphate (Aralen)**: 3 to 4 mg/kg/day; more retinotoxic than HCQ. - **Quinacrine**: 100 mg/day; side effects include headache, GI intolerance, and hematologic toxicity. - **Dapsone**: 25 mg twice daily, can increase to 200-400 mg/day; risk of hemolysis and methemoglobinemia.

Question 58

What precautions should be taken before starting antimalarial therapy for CLE?

Answer 58

- A **pretreatment ophthalmologic examination** should be performed due to the risk of antimalarial retinopathy. - Regular monitoring of **CBC** and liver/renal function tests is essential.

Question 59

What are the potential adverse effects of Thalidomide when used for refractory CLE?

Answer 59

- **Thalidomide**: 50 to 200 mg/day; potential adverse effects include teratogenicity, sensory neuropathy (25-75% of patients may develop peripheral neuropathy), and thromboembolism.

Question 60

What are the immunosuppressive options for antimalarial refractory disease in patients with cutaneous lupus erythematosus (CLE)?

Answer 60

1. **Systemic Glucocorticoids**: IV methylprednisolone for severe cases. Prednisone 20 to 40 mg/day with gradual reduction to avoid avascular necrosis. 2. **Traditional Immunosuppressives**: Azathioprine (1.5 to 2 mg/kg/day), Mycophenolate mofetil (2.5 to 3 g/day), Methotrexate (7.5 to 25 mg/week). 3. **Biologics**: Anti-TNF medications, B-lymphocyte stimulator inhibitors, Rontalizumab, sifalimumab, Anifrolumab, Tofacitinib, and ruxolitinib.

Question 61

What are the surgical and cosmetic therapy options for patients with cutaneous lupus erythematosus (CLE)?

Answer 61

1. **Hair Transplantation and Dermabrasion**: With risks due to CLE's tendency for nonspecific mechanical trauma. 2. **Scar Revision Techniques**: If on maintenance systemic therapy. 3. **Laser Treatments**: Argon and pulsed dye laser, Erbium:YAG or Fraxel CO2 for resurfacing atrophic scars. 4. **Autologous Fat Transplant**: For atrophic scarring and burned out lupus panniculitis. 5. **Avoid Collagen and Foreign Cosmetic Material Injections**: Due to potential complications.

Question 62

What preventive measures are recommended for patients with cutaneous lupus erythematosus (CLE)?

Answer 62

1. **Physical protection from the sun**: Use of broad spectrum sunscreens. 2. **Cessation of cigarette smoking**: Encouraged to discontinue smoking as it is associated with CLE.

Question 63

What systemic therapy is recommended for severe and symptomatic CLE?

Answer 63

IV methylprednisolone is recommended for severe and symptomatic CLE.

Question 64

What is the role of tofacitinib in CLE treatment?

Answer 64

Tofacitinib (JAK 1/3 inhibitor) has shown clinical benefit in CLE and cutaneous dermatomyositis in case reports.

Question 65

What is the role of belimumab in CLE treatment?

Answer 65

Belimumab is a fully human monoclonal antibody directed against BLyS, which is FDA-approved for SLE.

Question 66

What are the recommended preventive measures for CLE patients?

Answer 66

Preventive measures include physical protection from the sun, use of broad-spectrum sunscreens, and discontinuation of cigarette smoking.

Question 67

What is the role of methotrexate in CLE treatment?

Answer 67

Methotrexate (7.5 to 25 mg orally per week) is effective for severe refractory CLE.

Question 68

What are the adverse effects of systemic glucocorticoids in CLE treatment?

Answer 68

Adverse effects include steroid-induced bone loss, avascular bone necrosis, and adrenal suppression.

Question 69

What is the role of azathioprine in CLE treatment?

Answer 69

Azathioprine (1.5 to 2 mg/kg per day orally) can play a glucocorticoid-sparing role in CLE treatment.

Question 70

What are the adverse effects of lenalidomide in CLE treatment?

Answer 70

Lenalidomide has a lower rate of peripheral neuropathy compared to thalidomide but shares similar efficacy in CLE treatment.

Question 71

What is the role of mycophenolate mofetil in CLE treatment?

Answer 71

Mycophenolate mofetil (2.5 to 3 g divided twice daily orally) provides more specific inhibition of the de novo pathway in lymphocytes.

Question 72

What are the risks of using anti-TNF medications in CLE treatment?

Answer 72

Anti-TNF medications are used for recalcitrant CLE, particularly SCLE, but they are also known to induce both SLE and CLE.

Question 73

What are the recommended immunosuppressive options for patients with antimalarial refractory cutaneous lupus erythematosus (CLE)?

Answer 73

1. **Systemic Glucocorticoids**: IV methylprednisolone for severe cases. Prednisone (20-40 mg/day) with gradual reduction. 2. **Traditional Immunosuppressives**: Azathioprine, Mycophenolate mofetil, Methotrexate. 3. **Biologics**: Anti-TNF medications, B-lymphocyte stimulator inhibitors, Rontalizumab, sifalimumab, Anifrolumab, Tofacitinib, and ruxolitinib.

Question 74

What is the purpose of fat transplant in lupus patients?

Answer 74

For atrophic scarring and burned out lupus panniculitis.

Question 75

What should be avoided in cosmetic treatments for lupus patients?

Answer 75

Collagen and foreign cosmetic material injections should be avoided due to potential complications.

Question 76

What preventive measures should be taken for patients with cutaneous lupus erythematosus (CLE)?

Answer 76

1. Physical Protection: Use broad spectrum sunscreens and protective clothing against UV light. 2. Cigarette Smoking: Patients are encouraged to discontinue smoking as it is associated with CLE and less so with SLE. 3. Awareness of Antimalarial Treatment: Smoking is not responsive to antimalarials, hence cessation is crucial for management.

Question 77

What conditions should always be ruled out when diagnosing Acute Cutaneous Lupus Erythematosus (ACLE)?

Answer 77

The conditions that should always be ruled out for ACLE include: - Cancer - Toxic epidermal necrolysis - Chronic Cutaneous Lupus Erythematosus (CCLE) - Tinea imbricata - Cutaneous T-cell lymphoma - Lupus panniculitis - Infectious granulomatous (like fungal/ atypical/mycobacterial organisms) - Calciphylaxis

Question 78

What are the first-line therapeutic options for lupus erythematosus-specific skin disease?

Answer 78

The first-line therapeutic options include: - Topical glucocorticoids: Class I topical steroid daily to twice daily for 2 weeks, alternating with pimecrolimus 1% or tacrolimus 0.1% twice daily for 2 weeks. - Intralesional triamcinolone acetonide: 2.5-10 mg. ## Footnote Dosage for topical glucocorticoids is based on the severity of the condition and patient response.

Question 79

What are the key differential diagnoses for suspected Acute Cutaneous Lupus Erythematosus (ACLE)?

Answer 79

The key differential diagnoses for ACLE include: - Localized: Acne rosacea, Dermatitis (contact/irritant), Drug-induced hypersensitivity reaction - Consider: Isomorphic response (Koebner phenomenon), Psoriasis, Erythema multiforme - Always Rule Out: CLE, Toxic epidermal necrolysis, DLE, Cutaneous T-cell lymphoma, Infectious processes (e.g., fungal, mycobacterial)

Question 80

What are the first-line therapeutic options for managing skin disease in patients with Lupus Erythematosus?

Answer 80

The first-line therapeutic options for skin disease in Lupus Erythematosus include: - Topical glucocorticoids: Class I topical steroids daily to twice daily for 2 weeks, alternating with pimecrolimus 1% or tacrolimus 0.1% twice daily for 2 weeks. - Intralesional triamcinolone acetonide: 2.5-10 mg. - Hydroxychloroquine: 6.5 mg/kg/day based on ideal body weight. - Chloroquine: 3.5 mg/kg/day based on ideal body weight.