Pulmonary capillary hemangiomatosis as a rare cause of pulmonary hypertension.
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.
Masur
Y
Y
Institute of Pathology, University of the Saarland, Homburg/Saar, F.R.G.
Remberger
K
K
Hoefer
M
M
D000328:Adult; D017809:Fatal Outcome; D018324:Hemangioma, Capillary / Q000150:complications* / Q000473:pathology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D008175:Lung Neoplasms / Q000150:complications* / Q000473:pathology*; D008297:Male