Pulmonary capillary haemangiomatosis

Abstract

A Cypriot male of 22 years died after suffering recurrent daily haemoptyses of varying severity and slowly increasing effort intolerance for a period of four and a half years. Examination of a lung biopsy specimen revealed dilated vascular channels suggestive of a congenital anomaly of the pulmonary vasculature. Histological examination of the lung following necropsy revealed the diagnosis of pulmonary capillary haemangiomatosis. In this condition sheets of thin-walled blood vessels infiltrate the lung parenchyma, the walls of pulmonary arteries and veins, the bronchi and the pleura. The infiltration of pulmonary veins and venules induces secondary pulmonary veno-occlusive disease. This appears to be the second example of this disease which has been reported. It is open to clinical and histological mis-diagnosis and clinicians and pathologists should be aware of the existence of this entity.