What is the appropriate management for a patient with cutaneous manifestations of systemic lupus erythematosus presenting with inflammatory plaques on the face, back, and legs, including ulcerative lesions?

Management of Cutaneous Manifestations in Systemic Lupus Erythematosus

For a patient with inflammatory plaques on the face, back, and legs including ulcerative lesions, initiate mandatory photoprotection with SPF 60+ sunscreen, enforce smoking cessation, start hydroxychloroquine as first-line systemic therapy, and add moderate-dose glucocorticoids for initial disease control while antimalarials reach therapeutic effect. 1

Immediate Non-Pharmacologic Interventions

Photoprotection is mandatory for all SLE patients due to high photosensitivity and must include SPF 60+ sunscreen containing zinc oxide or titanium dioxide, strict sun avoidance, and protective clothing. 1, 2 UV exposure directly triggers and aggravates cutaneous lupus lesions. 3

Smoking cessation must be strongly counseled because smoking impairs response to antimalarial therapy and is linked to more severe forms of skin disease and comorbidity. 1, 3

Diagnostic Confirmation Before Treatment

Perform a skin biopsy from the most active erythematous or ulcerative portion of the lesion while clinically visible, as sampling after healing markedly reduces diagnostic yield. 1 The specimen should be processed for light microscopy and direct immunofluorescence to detect immunoglobulin and complement deposits. 1

Classify lesions into acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), or chronic cutaneous LE (CCLE) based on morphology and distribution, as this guides prognosis and systemic risk. 1, 4

First-Line Systemic Therapy

Hydroxychloroquine is the first-line systemic treatment for all types of cutaneous lupus not controlled with topical measures. 1, 4 Antimalarials are appropriate for widespread lesions, severe disease, and cases resistant to topical treatment. 4

Glucocorticoids should be used judiciously at the lowest effective dose for the shortest duration to achieve initial disease control while hydroxychloroquine reaches therapeutic effect (typically 6-12 weeks). 1 For severe inflammatory plaques with ulceration, consider initial pulses of intravenous methylprednisolone for 1-3 days followed by moderate oral doses. 5

Topical corticosteroids can be initiated simultaneously for localized lesions while systemic therapy takes effect. 1

Escalation Strategy for Refractory Disease

If the patient fails to respond adequately to hydroxychloroquine plus glucocorticoids, or if glucocorticoids cannot be tapered below acceptable doses for chronic use, add immunosuppressive agents. 1

Second-line choices include:

• Methotrexate (MTX)
• Azathioprine (AZA) - particularly compatible with pregnancy contemplation 5
• Thalidomide
• Retinoids
• Dapsone 4

Third-line treatment is mycophenolate mofetil (MMF), which is a potent immunosuppressant with efficacy in non-renal lupus, though its teratogenic potential (requires discontinuation at least 6 weeks before conceiving) and higher cost pose limitations in women of reproductive age. 5

Fourth-line options for refractory disease:

• Belimumab for widespread CLE lesions in patients with active SLE or recurrence during corticosteroid tapering 4
• Pulsed-dye laser or surgery for localized, refractory CCLE lesions in cosmetically unacceptable areas 4

Critical Assessment for Systemic Involvement

The presence of ulcerative lesions raises concern for thrombotic vasculopathy, which indicates increased cardiovascular risk and worse overall prognosis. 6 If ulceration, necrosis, or other signs of thrombotic vasculopathy are present, patients should be treated with antiplatelet drugs in addition to immunosuppressive therapy. 6

Obtain comprehensive laboratory workup:

• Complete autoantibody panel: ANA, anti-dsDNA, anti-Ro/SSA, anti-La/SSB, anti-RNP, anti-Sm 1
• Antiphospholipid antibodies 5
• Complement levels (C3, C4) 1
• CBC, ESR, CRP, serum albumin, creatinine, urinalysis with urine protein/creatinine ratio 7

Monitor for organ-threatening manifestations including lupus nephritis (serum creatinine, urine sediment, proteinuria, blood pressure), neuropsychiatric lupus, and hematologic complications, as cutaneous disease may herald systemic involvement. 7, 2

Ongoing Monitoring Protocol

Monitor patients every 6-12 months with CBC, ESR, CRP, serum albumin, creatinine, urinalysis, anti-dsDNA, C3, and C4 levels. 7, 1 Use the Cutaneous Lupus Disease Area and Severity Index (CLASI) to quantify disease activity and damage at baseline and follow-up. 1

Repeat anti-dsDNA and complement levels at follow-up visits even if previously negative or normal, as some patients develop these markers during flares. 1

Comorbidity Prevention During Treatment

Prescribe calcium and vitamin D supplementation for all patients on chronic glucocorticoid therapy to prevent bone loss. 1

Consider low-dose aspirin in patients receiving corticosteroids, those with antiphospholipid antibodies, or those with traditional cardiovascular risk factors. 1

Screen for and aggressively manage hypertension, dyslipidemia, and diabetes, as SLE patients have markedly increased cardiovascular disease risk. 1

Critical Pitfalls to Avoid

Do not rely on serological activity alone to intensify therapy without clinical correlation, as this risks overtreatment. 1

Avoid prolonged high-dose glucocorticoids due to increased infection risk, osteoporosis, avascular necrosis, and cardiovascular complications. 1

Do not delay skin biopsy until lesions have completely healed, as nonspecific histologic findings may fail to confirm the diagnosis. 1

If there is a change in clinical morphology of lesions or lack of response to treatment, perform a repeat biopsy to reassess the diagnosis. 8