Lupus Erythematosus: Systemic with Skin Manifestations

Lupus Erythematosus: Systemic with Skin Manifestations

Lupus Erythematosus: Systemic with Skin Manifestations

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SLE Basics - Lupus Unmasked

  • Definition: Systemic Lupus Erythematosus (SLE) is a chronic, relapsing-remitting, multisystem autoimmune disease characterized by diverse mucocutaneous and systemic manifestations.
  • Key Pathogenesis:
    • Autoantibody formation: ANA (most sensitive), anti-dsDNA (correlates with disease activity, nephritis), anti-Sm (highly specific).
    • Immune complex deposition: Type III Hypersensitivity leading to vasculitis and tissue damage.
    • Complement activation: ↓C3, ↓C4.
    • Genetic predisposition: Associated with HLA-DR2, HLA-DR3.
    • Environmental triggers: UV light exposure, certain drugs (e.g., hydralazine, procainamide, isoniazid), infections.
  • Epidemiology: Affects predominantly women of childbearing age (Female:Male ratio ≈ 9:1).

⭐ Anti-Sm (Smith antigen) antibody is highly specific for SLE, though less sensitive than ANA.

Skin Deep Lupus - Rash Decisions

  • Gilliam Classification of Cutaneous LE (CLE):
    • Acute Cutaneous LE (ACLE):
      • Localized: Malar "butterfly" rash (spares nasolabial folds).
      • Generalized: Widespread maculopapular rash on sun-exposed areas.
      • Strongly associated with systemic disease activity.
    • Subacute Cutaneous LE (SCLE):
      • Highly photosensitive, non-scarring, non-indurated lesions.
      • Forms: Papulosquamous (psoriasiform) or annular/polycyclic.
      • Often Anti-Ro/SSA positive; can be drug-induced (e.g., HCTZ, terbinafine).
    • Chronic Cutaneous LE (CCLE):
      • Discoid LE (DLE) - most common CCLE:
        • Erythematous indurated plaques, adherent scale, follicular plugging.
        • Leads to atrophy, scarring, dyspigmentation.
        • Sites: Face, scalp (scarring alopecia), ears.
        • 📌 DLE: A SCAR (Atrophy, Scale, Central hypopigmentation/depigmentation, Active erythematous border, Rim of hyperpigmentation, Follicular plugging).
      • Other forms: LE profundus (panniculitis), Chilblain lupus, Tumid lupus.
  • Other Mucocutaneous Manifestations:
    • Painless oral/nasal ulcers.
    • Non-scarring diffuse alopecia ("lupus hair").
    • Raynaud's phenomenon, Livedo reticularis, cutaneous vasculitis.

Collage of lupus skin manifestations

⭐ The malar rash in ACLE characteristically spares the nasolabial folds, a key diagnostic clue.

Spotting SLE - Lupus Detectives

  • EULAR/ACR Criteria (2019):
    • Entry: ANA titer ≥1:80.
    • Score ≥10 for classification.
    • Mucocutaneous: Non-scarring alopecia (2), Oral ulcers (2), SCLE/DLE (4), Acute cutaneous lupus (6).
  • Investigations:
    • Skin Biopsy (lesional): Interface dermatitis, liquefaction degeneration, epidermal atrophy, thickened BM (PAS+).

    • DIF/LBT: Granular IgG, IgM, C3 at DEJ. Lesional (>90%), sun-exposed non-lesional (SLE ~50-70%).

    • Serology: ANA (>95%), Anti-dsDNA (activity, nephritis), Anti-Sm (specific). 📌 'S'm 'S'pecific, d'S'dna 'S'evere. Low C3/C4.

      • Others: Anti-Ro/SSA (SCLE, neonatal lupus), Anti-La/SSB, Anti-U1 RNP, Anti-histone (drug-induced).
  • Diagnostic Flow (Simplified):

⭐ A positive Lupus Band Test (IgG at DEJ) in sun-exposed, non-lesional skin is highly suggestive of systemic lupus erythematosus.

Managing the Wolf - Skin Shield

  • General: Photoprotection (broad-spectrum sunscreen SPF $ \ge 30-50 $, protective clothing, sun avoidance), Smoking cessation, Vitamin D.
  • Topical (CLE):
    • Corticosteroids: Potent (clobetasol - DLE); mid-potency. Risk: atrophy.
    • Calcineurin inhibitors: Tacrolimus, pimecrolimus (face, steroid-sparing).
  • Systemic (Severe CLE/SLE):
    • Antimalarials: HCQ ($ \le 5 \text{ mg/kg/day} $ actual body weight) or Chloroquine. 1st line CLE & mild SLE. Ophthalmology screen (retinal toxicity).
    • Systemic Corticosteroids: Prednisolone for flares/severe disease, tapered.
    • Immunosuppressants: MTX, AZA, MMF (refractory CLE, steroid-sparing).
    • Biologics: Belimumab, Rituximab, Anifrolumab (refractory/severe SLE).
    • Other: Dapsone, Thalidomide (refractory DLE, teratogenicity/neuropathy risk).

Annular lesions of subacute cutaneous lupus erythematosus

⭐ Hydroxychloroquine is a cornerstone in managing cutaneous lupus; requires baseline and annual ophthalmological screening after 5 years (or sooner with risk factors).

High‑Yield Points - ⚡ Biggest Takeaways

  • Malar rash: Classic photosensitive "butterfly" erythema, spares nasolabial folds.
  • Discoid Lupus (DLE): Erythematous plaques, scale, follicular plugging; causes scarring alopecia, dyspigmentation.
  • Subacute Cutaneous LE (SCLE): Highly photosensitive, non-scarring; strong anti-Ro/SSA association.
  • Lupus profundus: Deep, firm nodules (panniculitis); may result in lipoatrophy.
  • Oral ulcers: Typically painless, common on palate/buccal mucosa.
  • Key Serology: ANA >95%; anti-dsDNA & anti-Sm highly specific.
  • Drug-induced SLE: Anti-histone Ab positive; skin signs less common.

Practice Questions: Lupus Erythematosus: Systemic with Skin Manifestations

Test your understanding with these related questions

Which of the following auto antibodies is most likely to be present in a patient with systemic lupus erythematosus?

1 of 5

Flashcards: Lupus Erythematosus: Systemic with Skin Manifestations

1/10

_____ Lupus Erythematosus presents most commonly as a non scarring papulosquamous eruptions in 2/3rd cases

TAP TO REVEAL ANSWER

_____ Lupus Erythematosus presents most commonly as a non scarring papulosquamous eruptions in 2/3rd cases

Subacute Cutaneous

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