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Rovner

COLLAGEN VASCULAR DISORDERS

  1. Lupus erythematosis

    1. Discoid LE (DLE)
      1. pink, violaceous well defined plaques in sun exposed distribution
      2. scarring, including alopecia
      3. follicular plugging, atrophy
      4. ANA-negative
      5. no systemic manifestations
      6. Treatment:
        1. steroids (systemic, topical)
        2. antimalarials
        3. sunscreens
    2. Systemic LE (SLE)
      1. autoimmune antibody/antigen complex deposition with Type III inflammation
      2. drug induced
        1. hydralazine
        2. procainamide
        3. isoniazid INH
      3. 4 or more ARA criteria for diagnosis:
        1. malar ("butterfly") rash with sun exposure
        2. discoid lesions
        3. photosensitivity
        4. oral ulcers
        5. arthritis
        6. serositis (pleuritis, pericarditis)
        7. renal disease
        8. neurologic sx's (seizures, neuropathy, psychosis)
        9. hematologic (anemia, pancytopenia)
        10. immunologic (anti-DNA ab's; LE prep; false + RPR) ANA
      4. Treatment:
        1. steroids
        2. immunosuppressants
        3. antimalarials
        4. sunscreens
    3. Subacute LE (SCLE)
      1. ANA negative lupus
      2. +SSA, SSB antibodies (anti Ro, anti La)
      3. less cardiac, renal involvement compared to SLE
      4. small, annular red plaques in sun exposed areas
      5. Neonatal lupus:
        1. (infants born to mothers with SCLE)
        2. 30 heart block
        3. discoid lesions
        4. anemia, "HELP" syndrome (Hepatic Enzymes Low Platelets)
        5. usually resolves within 6 months, but heart block permanent
  2. Dermatomyositis
    1. Inflammatory disorder of proximal muscles, skin
    2. Associated with internal malignancy 25 to 50% in patients over 55
    3. Skin:
      1. "heliotrope" rash
      2. Gottron's papules
      3. periungual telangiectases
    4. Muscle: limb/girdle weakness with abnormal EMG findings
    5. Laboratory:
      1. elevated CPK, Aldolase
      2. + ANA
      3. ESR elevation
    6. Treatment
      1. treat underlying malignancy
      2. steroids
  3. Scleroderma
    1. Morphea (localized)
      1. atrophic plaques without systemic involvement
    2. CREST Syndrome
      1. Calcinosis cutis
      2. Raynaud's disease
      3. Esophageal strictures
      4. Sclerodactyly
      5. Telangiectases
    3. PSS (Progressive Systemic Sclerosis)
      1. proximal sclerosis
      2. pulmonary fibrosis
      3. renal disease
      4. cardiac (restrictive cardiomyopathy)
    4. Treatment:
      1. steroids
      2. d-penicillamine