• J Swol, D Würflein, J Böhm, and J H Ficker.
• Department of Respiratory Medicine, Allergology and Sleep Medicine, Paracelsus Medical University Nuremberg, Nuremberg, Germany.
• Hippokratia. 2021 Apr 1; 25 (2): 83-86.

BackgroundDiffuse cystic lung diseases are a group of heterogeneous pathophysiological processes and include neoplastic, inflammatory, and infectious etiologies. This manuscript focuses on manifestations of pulmonary Langerhans cell histiocytosis (PLCH) and lymphangioleiomyomatosis (LAM). Description of the cases: Three female patients with LAM and one with PLCH are described. Stress dyspnea was a key symptom. There were similar cyst patterns in more than one lung lobe with a slow, progressive course. Histopathology confirmed the LAM diagnosis resulting from the nodular proliferate and the cyst wall that strongly expressed Human Melanoma Black-45 (HMB-45). A typical constellation for PLCH was demonstrated in high-resolution computed tomography (HRCT). It was found to be disseminated and relatively thick-walled cysts, mainly in the upper and middle parts. An individualized therapy was applied. Three patients with mild symptoms were followed up, including HRCT evaluations. Sirolimus was administered to one patient with a severe manifestation of LAM.ConclusionLAM and PLCH are rare. High-resolution computed tomography is an essential diagnostic tool. Lung emphysema as misdiagnosis should be avoided. The characteristics of pulmonary cysts, the cyst's wall regularity, and identification of associated pulmonary lesions, should be evaluated. A promising new therapy concept are mTOR inhibitors are, especially in LAM. The most important recommendation in PLCH is the cessation of cigarette smoking. HIPPOKRATIA 2021, 25 (2):83-86.Copyright 2021, Hippokratio General Hospital of Thessaloniki.

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