Pseudoxanthoma Elasticum is an autoimmune/collagen disease
Pathophysiology
1)Â pseudoxanthoma elasticum is an inherited disorder of connective tissue matrix 2) cause is unknown
Signs and Symptoms of Pseudoxanthoma Elasticum
1) skin has raised, yellowish papules on areas of flexion (neck, antecubital fossa, popliteal fossa, and inguinal area) 2) angoid streaks on funduscopic exam 3) gastrointestinal hemorrhage 4) severe, early coronary artery disease 5) occlusion of radial and ulnar arteries with positive Allen’s test 6) claudication 7) angina 8) easy bruising 9) hypertension 10) hanging and redundant folds of skin
Histology/Gross Pathology
1) fragmentation and calcification of elastic fibers 2) endocardial fibroelastosis 3) abnormal deposition of calcium in blood vessels, skin, and eyes
Associated Conditions
long-term administration of D-penicillamine causes a pseudoxanthoma elasticum-like condition
Treatment
1) avoidance of dietary calcium 2) aggressive treatment of hypertension 3) aggressive elimination of risk factors of atherosclerosis
Tips for USMLE
if question mentions skin like a “plucked chicken” think pseudoxanthoma elasticum. If question mentions skin disorder of the lateral or posterior neck think pseudoxanthoma elasticum
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