Bronchoalveolar Lavage as a Diagnostic Tool in an Atypical Pulmonary Langerhans Cell Histiocytosis

Bronchoalveolar Lavage as a Diagnostic Tool in an Atypical Pulmonary Langerhans Cell Histiocytosis

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Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse cystic lung disease that occurs almost exclusively in young adult smokers.High-resolution arcade smokey the bear belt computed tomography of the chest allows a confident diagnosis of PLCH in typical presentation, when nodules, cavitating nodules, and cysts coexist and show a predominance for the upper and middle lung.Atypical presentations require histology for diagnosis.Histologic diagnosis rests on the demonstration of increased numbers of Langerhans cells and/or specific histological changes.PLCH is one of the few diseases in which bronchoalveolar lavage (BAL) has a high diagnostic value and can in some circumstances replace lung biopsy.

We present a case of PLCH in an elderly non-smoker.Chest imaging revealed the presence of advanced interstitial lung disease with a fibrocystic revlon colorstay lip liner plum pattern.BAL cellular analyses disclosed a macrophage pattern with CD1a phenotype that strongly supports the PLCH diagnosis, even in the setting of atypical clinical presentation and a lack of smoking exposure.PLCH is extremely rare in non-smokers and could represent a distinct phenotype.