Lupus Erythematosus: Cutaneous Forms

Lupus Erythematosus: Cutaneous Forms

Lupus Erythematosus: Cutaneous Forms

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CLE Overview - Lupus's Skin Game

Cutaneous Lupus Erythematosus (CLE) presents with diverse skin manifestations, often independent of systemic lupus erythematosus (SLE).

  • Classification:
    • Acute Cutaneous LE (ACLE)
    • Subacute Cutaneous LE (SCLE)
    • Chronic Cutaneous LE (CCLE)
      • Discoid LE (DLE) is the most common CCLE.

Cutaneous Lupus Erythematosus Classification

⭐ SCLE is strongly associated with anti-Ro/SSA antibodies (found in ~70-90% of cases).

ACLE - Butterfly's Kiss

  • Acute Cutaneous Lupus Erythematosus (ACLE).
  • Classic Sign: Malar "butterfly" rash - erythematous, edematous eruption on cheeks & nasal bridge.
  • Distribution: Strongly photosensitive; occurs in sun-exposed areas.
  • Systemic Link: High association with active Systemic Lupus Erythematosus (SLE).
  • Outcome: Usually heals without scarring; post-inflammatory pigment changes possible. Acute Cutaneous Lupus Erythematosus (ACLE) Malar Rash

⭐ Often the first manifestation of SLE or signals a disease flare, especially with systemic symptoms like fever or arthralgia.

SCLE - Sun's Scaly Souvenir

  • Photosensitive, typically non-scarring eruption on sun-exposed sites (V-neck, arms, back). 📌 Sun's Scaly Souvenir!
  • Two main types:
    • Annular/polycyclic (raised red borders, central clearing)
    • Papulosquamous (scaly plaques, resembles psoriasis/eczema)
  • Antibodies: Anti-Ro/SSA ( >70-90% ), Anti-La/SSB ( ~30-50% ).
  • ~50% fulfill criteria for mild SLE.
  • Drug-induced SCLE is common (e.g., HCTZ, PPIs, terbinafine). Subacute Cutaneous Lupus Erythematosus on face and neck

⭐ Strong Anti-Ro/SSA positivity ( >70% ); risk of neonatal lupus if mother is positive.

DLE/CCLE - Discoid Damage Drama

  • Most common Chronic Cutaneous LE (CCLE).
  • Lesions: Well-demarcated erythematous plaques; adherent scale, follicular plugging, central atrophy, scarring alopecia.
    • Sun-exposed areas: face, scalp, ears.
  • 📌 Mnemonic "SCAR": Scarring alopecia, Central atrophy, Adherent scale, Rim of erythema.
  • Low risk of systemic LE progression (~5-10%).
  • Chronic lesions: risk of Squamous Cell Carcinoma (SCC). Discoid lupus erythematosus with follicular plugging

⭐ "Carpet tack" sign: horny plugs on undersurface of lifted scale, classic for DLE/CCLE an exam favourite finding indicating follicular plugging when the scale is removed from a lesion of discoid lupus erythematosus (DLE).

Rarer CLE Forms - Lupus's Odd Bunch

  • Tumid Lupus Erythematosus (TLE)
    • Urticarial plaques, minimal epidermal change, dermal mucin.
    • Excellent prognosis, responds well to antimalarials.
    • Photosensitive.
  • Lupus Erythematosus Panniculitis (LEP)/Lupus Profundus
    • Deep, firm nodules; may ulcerate, cause lipoatrophy ("doughnut-like" depressions).
    • Often on proximal limbs, face. Lupus panniculitis with lipoatrophy and histology
  • Chilblain Lupus Erythematosus (CHLE)
    • Purple, painful plaques on acral sites (fingers, toes, nose, ears) in cold, damp weather.
    • May coexist with other LE forms.

⭐ Lupus Panniculitis can precede systemic lupus erythematosus (SLE) diagnosis by years or occur with active SLE or DLE (Discoid Lupus Erythematosus).

CLE Diagnostics - Unmasking Lupus

  • Clinical: Photosensitive rash, discoid lesions, alopecia.
  • Histopathology (HPE): Interface dermatitis, follicular plugging, liquefaction degeneration.
  • Direct Immunofluorescence (DIF): Lupus Band Test (LBT) - IgG, IgM, C3 at dermoepidermal junction (DEJ).
    • LBT: +ve in lesional skin (90%), sun-exposed non-lesional (70-80%), non-sun-exposed non-lesional (50%).
  • Serology: ANA (sensitive), Anti-dsDNA, Anti-Sm (specific for SLE), Anti-Ro (SSA), Anti-La (SSB).

⭐ Lupus Band Test: Granular deposition of IgG and C3 at the DEJ is characteristic. Positive in >90% of lesional skin in DLE.

CLE Management - Skin Shield Strategy

  • Core: Sun protection (SPF >30 UVA/UVB), smoking cessation.
  • Topical:
    • Corticosteroids (potent/mid-potency).
    • Calcineurin inhibitors (face, flexures).
  • Systemic (widespread/refractory):
    • Antimalarials (Hydroxychloroquine - HCQ).
    • Immunosuppressants (MTX, AZA, MMF).
    • Retinoids, Dapsone.

⭐ Hydroxychloroquine (HCQ) mandates baseline and annual eye exams due to risk of retinal toxicity.

High‑Yield Points - ⚡ Biggest Takeaways

  • Discoid Lupus Erythematosus (DLE) is the most common chronic form, presenting with well-demarcated plaques, adherent scale, follicular plugging, and atrophic scarring.
  • Subacute Cutaneous Lupus Erythematosus (SCLE) features photosensitive, non-scarring, annular or papulosquamous lesions, often anti-Ro/SSA positive.
  • Acute Cutaneous Lupus Erythematosus (ACLE) typically manifests as a malar rash ("butterfly rash") and strongly correlates with active Systemic Lupus Erythematosus (SLE).
  • Lupus profundus involves deep, tender nodules leading to lipoatrophy.
  • Direct Immunofluorescence (DIF) of lesional skin showing a "lupus band" (IgG, IgM, C3 at dermoepidermal junction) is diagnostic.
  • Key management includes strict sun protection, topical/intralesional corticosteroids, and hydroxychloroquine.
  • Drug-induced SCLE is commonly associated with drugs like terbinafine, hydrochlorothiazide, and PPIs.
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Practice Questions: Lupus Erythematosus: Cutaneous Forms

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Flashcards: Lupus Erythematosus: Cutaneous Forms

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

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

Subacute Cutaneous

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Lupus Erythematosus: Cutaneous Forms | Autoimmune Skin Diseases - OnCourse NEET-PG