Multifocal distribution of pulmonary capillary haemangiomatosis

Aims: We investigated a case of pulmonary capillary haemangiomatosis, a rare condition, to determine the extent of the pathological changes within the lungs. Systematic histological sampling has not previously been performed in this condition. Methods and results: A 52-year-old woman with a history of ischaemic cardiomyopathy suffered from repeated respiratory infections, which were attributed to chronic pulmonary congestion. She died suddenly of fulminant pulmonary thrombembolism. An autopsy was performed and lung tissue was sampled at multiple sites. Beside passive congestion, the lungs showed well-circumscribed areas containing proliferations of small capillaries infiltrating the pulmonary septa and the walls of otherwise normal blood vessels and bronchi. The most severely affected areas were found to be in the periphery of both lower lobes. A diagnosis of pulmonary capillary haemangiomatosis was made. Conclusions: This is the first case of pulmonary capillary haemangiomatosis in which systematic histological sampling has been performed. Mapping of lesions disclosed the multifocal distribution of pulmonary capillary haemangiomatosis in this patient.