Clinical Features

 

Systemic lupus erythematosus (SLE)

Fever, weight loss, fatigue, myalgia, and lymphadenopathy

As proposed by ACR, SLICC and EULAR/ACR classification systems:

  • American College of Rheumatology (ACR) criteria: ≥4 for SLE diagnosis
    1. Malar rash
    2. Discoid lupus erythematosus (DLE)
    3. Photosensitivity
    4. Ulcers – painless oral or nasopharyngeal
    5. Arthritis in ≥2 joints
    6. Serositis – pleuritis or pericarditis
    7. Renal disease – proteinuria > 0.5g/day or cellular casts
    8. Neurologic disorder (seizures or psychosis)
    9. Hematologic – hemolytic anemia, leukopenia, lymphopenia or thrombocytopenia
    10. Immunologic disorder – anti-dsDNA, Anti-sm or antiphospholipid antibodies.
    11. Anti-nuclear antibodies (ANA) antibodies

The Systemic Lupus International Collaborating Clinics (SLICC) classification system criteria: ≥4 criteria, ≥ 1 clinical and ≥ 1 immunologic criterion OR 2 Lupus nephritis with positive ANA or anti-dsDNA antibodies

  1. Acute cutaneous lupus erythematosus (CLE)
  2. Chronic CLE
  3. Oral ulcers
  4. Nonscarring alopecia
  5. Synovitis involving ≥ 2 joints, OR tenderness in ≥2 joints and ≥30 minutes of morning stiffness
  6. Serositis
  7. Renal involvement
  8. Neurologic involvement
  9. Hemolytic anemia
  10. Leukopenia (< 4000/mm3 at least once)
  11. Thrombocytopenia (<100,000/mm3) at least once

  1. ANA
  2. Anti-dsDNA
  3. Anti-sm
  4. Antiphospholipid antibodies
  5. Low complement
  6. Direct Coombs test in the absence of hemolytic anemia

 

Cutaneous Lupus Erythematosus (CLE)

Classified into specific (histopathology = interface dermatitis) and non-specific skin lesions

See “Specific to skin (CLE)” tab for details

  • Acute CLE, (ACLE)
  • Subacute CLE (SCLE)
  • Chronic CLE (CCLE)

  1. Diffuse non-scarring alopecia
  2. Raynaud phenomenon
  3. Nail fold telangiectasias and erythema (dermoscopy: mildly dilated and elongated capillary loops)
  4. Palmar erythema
  5. Papular AND nodular mucinosis (75% will have SLE)
  6. Sweet syndrome-like neutrophilic dermatosis
  7. Vasculitis: urticarial vasculitis, small vessel vasculitis, polyarteritis nodosa-like lesions, ulcerations
  8. Cutaneous signs of antiphospholipid syndrome: livedo reticularis, ulcerations, acrocyanosis, atrophie blanche-like lesions, livedoid vasculopathy, degos-like lesions

Livedo reticularis and ischemic central nervous system disease

Antiphospholipid syndrome