Diffuse pulmonary lymphangiomatosis in a young adult

We report a new case of diffuse pulmonary, lymphangiomatosis in a 22 year-o ld man with a dysmorphic syndrome. The disease started with dyspnea which b ecame rapidly disabling. The diagnosis was established from a pulmonary, bi opsy. Our patient developed severe chronic respiratory failure. Diffuse pul monary lymphangiomatosis is a very uncommon disease. It is seen predominant ly in children, exceptionally in adults, and affects both sexes equally. Sy mptoms like dyspnea and cough, pulmonary function with restrictive pattern, and interstitial syndrome, are not specific. Only pathology is evocative, characterized primarily, by multifocal proliferation of pulmonary lymphatic vessels and increased number of complex anastomosing channels. These chann els tend to dilate with time. The prognosis is poor and the treatment essen tially palliative.