What are the clinical features and recommended management for scalp discoid lupus erythematosus (DLE) in a post‑menopausal woman?

Discoid Lupus Erythematosus of the Scalp: Clinical Features and Management

Clinical Features

Scalp DLE presents as inflammatory, erythematous plaques with adherent scales that progress to permanent scarring alopecia in 34% of patients, particularly those with prolonged disease duration and early scalp involvement. 1

Key Diagnostic Features

• Morphology: Inflammatory plaques with follicular keratotic plugs, perifollicular scaling, and white structureless areas representing scarring 2
• Distribution: Face (81.1%), limbs (71.7%), and scalp (48.4%) are most commonly affected 3
• Dermoscopic findings in scalp DLE:
  • White structureless areas (62%) indicating fibrosis 2
  • Arborizing vessels (57.8%) 2
  • White scales (54.2%) and follicular keratotic plugs (47%) 2
  • Absent follicular openings (45.8%) signaling permanent hair loss 2
  • Perifollicular scaling (43.9%) 2
  • Pink-white background (40.4%) and speckled brown pigmentation (38%) 2

Pathophysiology of Scarring

• Perifollicular lymphocytic inflammation targets the mid-follicle at the sebaceous gland level, causing destruction of mid-follicular stem cells and resulting in irreversible scarring alopecia 1
• Significant reduction in sebaceous gland size occurs in affected scalp 1
• Histopathology shows periadnexal and perivascular dermal infiltrates, basal cell vacuolization, epidermal atrophy, hyperkeratosis, and follicular plugging 3

Laboratory Evaluation

• ANA positive in 56.6% of patients 3
• Anti-dsDNA positive in 45.3% of patients 3
• Direct immunofluorescence shows IgM and IgG deposits at the dermoepidermal junction 3

Management Algorithm

First-Line Treatment (Initiate Immediately)

All patients with scalp DLE should receive hydroxychloroquine (not exceeding 5 mg/kg real body weight) combined with high-potency topical corticosteroids and strict photoprotection. 4

• Hydroxychloroquine: Cornerstone of therapy, reduces disease activity and mortality 4
• Ophthalmologic screening: Baseline, after 5 years, then yearly to monitor for retinal toxicity 4
• Topical corticosteroids: High-potency agents (clobetasol) applied to scalp lesions twice daily 4
• Topical calcineurin inhibitors: Tacrolimus as alternative to avoid skin atrophy 4

Essential Preventive Measures

• Strict photoprotection: Broad-spectrum sunscreens and protective headwear, as UV exposure is the leading trigger (86.8% of patients) 3, 4
• Smoking cessation: Critical, as smoking reduces antimalarial efficacy and worsens cutaneous lupus 4
• Stress management: Stress triggers flares in 26.4% of patients 3

Second-Line Treatment (For Inadequate Response After 8-12 Weeks)

For refractory scalp DLE, add immunomodulatory agents to facilitate glucocorticoid tapering and prevent further scarring. 4

• Methotrexate: Effective for various cutaneous manifestations 4
• Mycophenolate mofetil: Effective for refractory cutaneous disease 4
• Dapsone: Particularly useful for bullous variants 4
• Retinoids: For hyperkeratotic and hypertrophic lesions 4

Systemic Glucocorticoids (Short-Term Only)

• For widespread or severe disease: Short-term systemic prednisone 4
• Taper rapidly to ≤7.5 mg/day prednisone equivalent once disease control achieved 4
• Goal: Minimize to <7.5 mg/day for chronic maintenance and withdraw when possible 4

Third-Line Treatment (Refractory Cases)

For cases unresponsive to standard therapies, biologics should be considered. 4

• Belimumab or rituximab: For refractory disease 4
• Anifrolumab: Emerging evidence shows efficacy in severe lupus skin manifestations with dermoscopic and histological remission 5

Monitoring Protocol

Regular Assessment

• Clinical evaluation: Assess rash extent and severity at each visit 4
• Laboratory monitoring every 3-6 months: Complete blood count, anti-dsDNA, and complement levels (C3, C4) to detect systemic disease progression 4
• Dermoscopy: Valuable tool for monitoring therapeutic response, tracking regression of inflammatory features and scarring 5, 2

Disease Activity Indices

• Use validated activity indices (SLEDAI, BILAG, or ECLAM) to monitor lupus activity and detect flares 4

Critical Pitfalls to Avoid

• Never delay treatment: Permanent scarring alopecia develops in 34% of patients, particularly with prolonged disease and early scalp involvement 1
• Do not discontinue hydroxychloroquine unless specific contraindication exists 4
• Avoid prolonged high-dose glucocorticoids: Risks substantially increase above 7.5 mg/day continuous dosing 4
• Do not overlook systemic progression: 67.9% have localized disease, but regular monitoring for systemic involvement is essential 3

Special Considerations for Post-Menopausal Women

• Assess for osteoporosis: Screen according to guidelines for postmenopausal women, particularly those on steroids 6
• Ensure adequate calcium and vitamin D intake, regular exercise, and smoking cessation 6
• Cardiovascular risk assessment: Monitor blood pressure, lipids, glucose, and body mass index at least annually 6
• HRT considerations: May be used cautiously for severe vasomotor symptoms in stable/inactive disease with negative antiphospholipid antibodies 6