Pulmonary capillary hemangiomatosis as a rare cause of pulmonary hypertension

Abstract

Pulmonary capillary hemangiomatosis (PCH), first described by Wagenvoort et al. in 1978, is a rare cause of pulmonary hypertension which occurs predominantly in young adults. In the literature only 18 cases have been reported. In 1988 Langleben et al. proposed a hereditary form of PCH with probable autosomal-recessive transmission. Histologic findings include irregular small nodular foci of thin-walled capillary-sized vessels which diffusely invade the lung parenchyma, the bronchial/bronchiolar walls and the adventitia of large vessels. We report on a case of PCH in a 24 year old man who presented the clinical signs of interstitial lung disease.