Differentiating Pulmonary Lymphangioleiomyomatosis From Pulmonary Langerhans Cell Histiocytosis And Birt-Hogg-Dube Syndrome

    January 2013 in “ Lung India ”
    Himanshu Bhardwaj, Bhaskar Bhardwaj
    TLDR Radiological findings help distinguish LAM, PLCH, and BHD to avoid invasive tests.
    The document discussed the differentiation of pulmonary lymphangioleiomyomatosis (LAM) from pulmonary Langerhans cell histiocytosis (PLCH) and Birt-Hogg-Dube (BHD) syndrome, which are all cystic lung diseases. PLCH is characterized by irregularly shaped lung cysts and a strong association with smoking, while BHD is a genetic disorder with features like skin lesions and renal cancers. LAM, often associated with tuberous sclerosis complex, predominantly affects women and features uniform, round lung cysts. The document emphasized the importance of radiological findings in distinguishing these conditions to avoid invasive testing.
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