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Nized by characteristic skin lesions, cafe-au-lait spots, and cutaneous neurofibromas.1 The disease is occasionally complicated with systemic vasculopathy, such as renovascular hypertension, myocardial infarction, cerebral infarction, ischemic bowel disease, or rupture of an arterial aneurysm.2, 3 Only one case of pulmonary artery involvement has been reported up to now.4 We report here two cases of von Recklinghausen disease complicated with prominent pulmonary hypertension. Vasculopathy of von Recklinghausen disease might underlie pulmonary hypertension in the two patients.
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