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Lupus and Skin: Manifestations, Photosensitivity & Systemic Involvement

Cutaneous Lupus Erythematosus (CLE) Subtypes

1. Acute Cutaneous Lupus (ACLE)

  • Malar ("butterfly") rash: Erythema over cheeks/nose, sparing nasolabial folds. Photosensitive.
  • Generalized maculopapular rash: Trunk, arms, photosensitive.
  • Often coincides with systemic flares.

2. Subacute Cutaneous Lupus (SCLE)

  • Annular or psoriasiform plaques on sun-exposed areas (neck, arms, trunk).
  • Strong anti-Ro/SSA association.
  • Less scarring; can be drug-induced.

3. Chronic Cutaneous Lupus (CCLE) / Discoid Lupus (DLE)

  • Well-defined erythematous plaques with scaling, follicular plugging.
  • Scarring, atrophy, dyspigmentation, permanent hair loss (scarring alopecia).
  • Face, scalp, ears, neck. Can occur without systemic lupus (5–10% progress to SLE).

4. Other Skin Manifestations

  • Vasculitis: Palpable purpura, ulcers, livedo reticularis.
  • Photosensitivity: 60–100% of SLE; UV triggers skin + systemic flares.
  • Alopecia: Non-scarring (active SLE) vs scarring (DLE).
  • Mucosal ulcers: Oral, nasal, vaginal (painless).
  • Chilblains (lupus pernio): Cold-induced acral lesions.

Systemic Involvement Beyond Skin

SystemKey Manifestations
JointsNon-erosive arthritis (hands, wrists, knees); Jaccoud's arthropathy.
RenalLupus nephritis (proteinuria, hematuria, casts); biopsy class guides Rx.
NeuropsychiatricHeadaches, seizures, psychosis, cognitive dysfunction, stroke, PNS.
HematologicAutoimmune cytopenias (AIHA, ITP, leukopenia); antiphospholipid syndrome.
CardiopulmonaryPleuritis, pericarditis, Libman-Sacks endocarditis, PAH, ILD.
GIVasculitis, pancreatitis, protein-losing enteropathy.

Management Principles

  • Sun protection: Broad-spectrum SPF 50+, UPF clothing, avoidance—non-negotiable.
  • Topical: Corticosteroids, calcineurin inhibitors (tacrolimus/pimecrolimus) for face/folds.
  • Systemic: Hydroxychloroquine (foundation for all SLE), methotrexate, mycophenolate, belimumab, anifrolumab, voclosporin (nephritis).
  • Intralesional steroids for discoid plaques.
  • Multidisciplinary: Rheumatology + dermatology + nephrology + neurology as needed.

Skin is often the window to systemic activity—treat aggressively to prevent damage.

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