ABSTRACT
Pulmonary Langerhans' cell histiocytosis (PLCH) remains an important diagnostic consideration
in the differential diagnosis of diffuse infiltrative lung disease, particular among
smokers. This review highlights recent progress in our understanding of the etiology,
clinical presentation, and diagnostic and therapeutic approaches to this unusual lung
disorder. Evolving studies strongly link the basic pathogenesis of PLCH as an uncommon
reaction to tobacco smoke. Recent progress in the clinical approach to these patients
has emphasized important roles for high-resolution computed tomographic (CT) scanning
and immune reactive tissue markers including cluster differentiation (CD) 1a antigen
in the diagnosis of this disorder. A rationale diagnostic algorithm and current management
strategies are summarized.
KEYWORD
Pulmonary Langerhans' cell histiocytosis - pulmonary eosinophilic granuloma - Langerhans'
cells