lymphangioleiomyomatosis LAM is a rare female only disease with cystic lung lesions

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Lymphangioleiomyomatosis (LAM) is a rare lung disease characterized by diffuse cystic lesions. Characteristic population includes young female (30s) with tuberous sclerosis (skin lesions), progressive dyspnea, recurrent pneumothorax, chylous pleural effusions, and characteristic CT findings of cysts.

Lymphangioleiomyomatosis is associated with renal angiomyolipoma and tuberous sclerosis.

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• lymphangioleiomyomatosis LAM
  • lymphangioleiomyomatosis LAM is a rare female only disease with cystic lung lesions

Chylous complications happens from obstruction of lymph system. Chylous fluid can be seen in multiple locations including pleural effusion, ascites, pericardium, urine. History of white material in urine, stool, bronchi, vaginal discharge can be clue to diagnosis. ¹

• lymphangioleiomyomatosis has cystic lesions with chylous effusions on chest imaging

Footnotes

1. Murray and Nadel Ch 97 Lymphangioleiomyomatosis ↩