1298
Refers to a small-medium blood vessel disease caused by arterial embolization of debris from a disrupted atherosclerotic plaque.
Usually seen in patients who undergo arterial catheterization, prolonged anticoagulation,or acute thrombolytic therapy, although spontaneous embolization can occur.
Typically presents in elderly man, with more than 50% of patients older than seventy year, with cardiovascular disease.
Skin is the most commonly involved organ ranging in frequency from 35 to 96% of clinically apparent cases.
Presentation is usually subacute with the time from an inciting event to the presentation ranging from days to months.
Presentation maybe nonspecific or complex involving multiple organ systems.
Embolization in the descending aorta can cause ischemia in skin and muscle tissue of the trunk and lower limb, kidneys, and gastrointestinal tract.
If embolization occurs in or proximal to the ascending aortic arch confusion, visual change, and skin changes in the upper limbs may be seen.
Livedo reticularis is the most frequent skin change, although cyanosis, gangrene, ulceration, nodules, purpura and petechiae may be seen.
Purpuric toe lesions are a cutaneous manifestations, but can be seen with vasculitides
Patients may experience nonspecific symptoms including: fever, anorexia, weight loss, fatigue, and myalgias.
Patients may present with acute elevations of blood pressure due to the occlusion of cholesterol crystals of the vasculature and release of renin secondary to renal ischemia.
Laboratory abnormalities include: eosinophilia, anemia, low complement levels, elevated ESR elevated C reactive protein.
Kidney involvement may be diagnosed by urinalysis and microscopy showing proteinuria, pyuria, eosinophilia and hematuria.
A diagnostic triad includes: history of a precipitating factor, acute renal failure, and cutaneous a retinal manifestations.
Biopsy is needed to confirm the diagnosis.
Biopsy of peripheral tissues from skin or muscles is recommended to avoid more invasive procedures.
If retinal cholesteryl crystals or Hollenhorst plaques are present, biopsy is not necessary as that is strong evidence of the disease.
Treatment includes supportive care and aggressive control of risk factors, with the use of statin therapy, antiplatelet therapy and avoidance of further vascular instrumentation.
One year survival rates for 69-87%.
Approximately one in three patients develop clinically significant kidney injury.
The most common cause of death is cardiovascular, being attributed to the high incidence of pre-existing cardiovascular disease.