livedo reticularis; livedoid vasculopathy; mottled skin; cutis marmorata

Classification

• cutis marmorata
  • mottling appears on exposure to cold
  • mottling disappears on warming
• livedo reticularis idiopathica
  • persists on warming
  • not associated with known diseases
  • occurs in persons with unstable nervous systems
  • ulceration is a complication
• livedo reticularis symptomatica
  • persists on warming
  • frequently accompanies polyarteritis nodosa
  • ulceration is a complication

Etiology

• neurohumoral disease
  • pheochromcytoma
  • carcinoid syndrome
• hematologic disease
  • polycythemia vera
  • leukemia
  • thrombocytosis
  • cold agglutinin syndrome
• hypercoagulable state (thrombophilia)
  • anti-phospholipid antibody syndrome
• paraproteinemia
  • multiple myeloma & associated cryoglobulinemia type-1
  • Waldenstrom's macroglobulinemia & other hyperviscosity syndromes
• autoimmune disease (connective tissue disease)
  • systemic lupus erythematosus (SLE)
  • dermatomyositis
  • rheumatoid arthritis
    • Still disease
    • Felty syndrome
  • scleroderma
  • Sjogren's syndrome
• infections
  • parvovirus B19
  • hepatitis C
  • syphilis
  • meningococcemia
  • pneumococcal sepsis
  • tuberculosis
• drug reactions
  • amantadine
  • quinidine
  • warfarin
  • minocycline
• vasculitides (vasculitis)
  • polyarteritis nodosa
  • Wegener's granulomatosis
  • microscopic polyarteritis
  • Churg-Strauss syndrome
  • Takayasu arteritis
  • temporal arteritis
• calciphylaxis
• cholesterol embolization
  • livedo reticularis over the legs & thighs
  • embolization generally involves digits
    • ischemia, painful
  • prior vascular catheterization
• endocarditis
• microvascular occlusion syndromes
  • hemolytic uremic syndrome
  • thrombotic thrombocytopenic purpura
  • disseminated intravascular coagulation
  • heparin necrosis
  • paroxysmal nocturnal hemoglobinuria
• cryoglobulinemia

Epidemiology

• mean age 45 years
• 71% women

Pathology

• stagnation of blood in dilated superficial capillaries & venules
• pathologic changes in larger vessels deeper in the skin
• hyalinizing vascular disease
• thrombosis & ulceration of the lower extremities

Clinical manifestations

• mottling of the skin of the arms &/or legs with round patches of normal skin in a background of cyanotic-appearing skin
• macular, lesions not elevated
• more commonly affects lower extremities (bilateral in 80%)
• lesions of livedo reticularis blanch on diascopy
• may be painful
• ulceration (69%)
• may occur during pregnancy

* images

Laboratory

• factor V Leiden mutation (22%)
• prothrombin G20210A gene mutation (8%)
• lupus anticoagulant (18%)
• anticardiolipin Ab (29%)
• decreased protein C activity (13%)
• serum homocysteine (homocysteinemia in 14%)
• vascular biopsy show intraluminal thrombosis in 98%

More general terms

• sign/symptom
• vascular disease (vasculopathy)

Additional terms

• livedoid vasculitis