CTICasesPulmonary Langerhans Cell Histiocytosis
Imaging findings
Chest CT demonstrates numerous irregular, thick-walled, and bizarre-shaped cystic spaces primarily concentrated in the upper and mid lung zones, with relative sparing of the lung bases. In addition to the cystic lesions, there are small, centrilobular nodular opacities, some of which demonstrate central cavitation or thick inflammatory cuffs representing peribronchiolar infiltration.
Key takeaways
Pulmonary Langerhans cell histiocytosis (PLCH) is a smoking-related lung disease characterized by a combination of centrilobular nodules, cavitary nodules, and irregular, thick- or thin-walled cysts. The classic distribution is upper and mid-lung predominant, with sparing of the costophrenic angles. The progression from nodules to cavitating nodules and ultimately to permanent bizarre-shaped cysts is highly characteristic and helps differentiate PLCH from lymphangioleiomyomatosis (LAM), which features diffuse, uniform, round, thin-walled cysts without a nodular phase.
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