Xanthoma disseminatum with respiratory tract involvement and fatal outcome

Xanthoma disseminatum (XD) is a rare mucocutaneous xanthomatosis classified as a benign form of non-langerhans' cell histiocytosis. The case history i s presented of a 61 year old woman with XD who developed dyspnoea and spiro metric features of airflow obstruction. Bronchoscopy and computed tomograph y confirmed involvement of the large and medium sized bronchi and she subse quently died from acute respiratory failure. The post-mortem findings and t he importance of respiratory tract disease in this unusual condition are di scussed.