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Livedo Reticularis

Clinical presentation
  • Transient or persistent red-blue-purple cyanotic discoloration with net-like pattern due to disturbances in cutaneous blood flow
  • When underlying cause is vasospasm or viscosity issues → LR network is typically fine and complete → Livedo Reticularis
  • When underlying cause is due to vessel wall pathology or intraluminal obstruction → LR pattern is more likely to be patchy, ramificado → Livedo Racemosa (de ramificado)
 
Pathogenesis
Physiologic
Pathologic
Disappears with warming
May vary with warming but typically persists
No underlying abnormality
Underlying abnormality
  • The cutaneous system consists of 1-3 cm cones with the base near the skin surgace and the apex deeper in the epidermis
  • Apex of the cone → ascending arteriole
  • Base → lower density and smaller arterial vessels
  • Margins of the base → superficial venous plexus is more prominent
  • If lower blood flow to the skin → increased deoxygenated blood predominantly in the venous area at the margins of the cones
 
Etiology
  • Congenital
    • Cutis marmorata telangiectatica congenita
  • Acquired
    • Vasospasm
      • Physiologic livedo reticularis/cutis marmorata
      • Idiopathic livedo reticularis
      • Connective tissue disease
      • Raynaud’s disease
      • Raynaud’s phenomenon
    • Vessel wall abnormalities
      • Calciphylaxis
      • Sneddon syndrome
      • Vasculitis (PAN, cryoglobulinemic, CTD-associated)
      • Livedoid vasculopathy
    • Intraluminal abnormalities
      • Increased red cells or platelets (Polycythemia vera, thrombocythemia)
      • Protein abnormalities (cryoglobulins, cryofibrinogen, cold agglutinins, paraproteins)
      • Hypecoagulability (antiphospholipid antibodies, protein C&S deficiency, antithrombin III deficiency, factor V Leiden mutation, homocystinuria, hyperhomocysteinemia, DIC)
        • Livedo racemosa → in antiphospholipid syndrome
      • TTP
      • Embolic (cholesterl, septic, atrial myxoma, decompression sickness (nitrogen), carbon dioxide arteriography)
    • Other
      • Medications (IFN, norepinephrine, amantadine)
      • Infections (syphilis, hepatitis, mycoplasma)
      • Neoplasms (pheochromocytoma)
      • Neurologic (reflex sympathetic dystrophy, paralysis)
      • Moyamoya disease
 
Livedo Racemosa
  • typically related to Sneddon syndrome
  • most common dermatologic presentation of antiphospholipid syndrome
  • large branching pattern that favors trunk and proximal limbs