Clinical manifestation

Skin findings: symmetrical, small, yellow papules, coalescing into plaques in a linear pattern, giving affected skin "plucked chicken" appearance; first noted on the lateral neck and later involving antecubital fossae, axillae, popliteal fossae, inguinal and periumbilical areas, oral, vaginal and rectal mucosa; with disease progression, skin sometimes becomes soft, lax, wrinkled, and hangs in folds; elastosis per-forans serpiginosa may coexist Ocular findings: bilaterally symmetrical angioid streaks of retina, noted several years after onset of cutaneous lesions; may have to retinal hemorrhages; progressive loss of central vision

Cardiovascular findings: usually the last lesions to be recognized; peripheral pulses often severely diminished; hypertension; coronary artery disease causes angina pec-toris and subsequent myocardial infarction; mitral valve prolapse; gastrointestinal hemorrhage, usually gastric in origin; less commonly, hemorrhaging occurs in urinary tract or cerebrovascular system

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