Histiocitosis de células de Langerhans, una afección pulmonar infradiagnosticada en fumadores: reporte de caso y revisión de la literatura

Langerhans cell histiocytosis (LCH) is a disease that can affect patients of any age, but in adults it is a rare disorder of unknown etiology that occurs predominantly in young smokers, without differences in gender. Although certain peculiarities of the disease can be the same than in the pediatric population, the proportion of cases with pulmonary involvement is much higher in adults. It often evolves through successive flare-ups and its severity ranges from benign tolife-threatening. Some patients develop significant functional impairment with psychosocial repercussions, that impact the quality of life and are associated with prolonged disability. The diagnostic key will be determined by the history of smoking, and the presence of nodules, cavitated nodules, and thick and thin-walled cysts on high-resolution chest computed tomography (HRCT). However, the definitive diagnosis requires the identification of Langerhans cell granulomas, which is generally achieved by performing a lung biopsy and its histopathological and immunohistochemicalstudy. Today, we could consider this a rare entity, of which there is no clear pathogenic mechanism, and therefore, does not have yet specific therapeuticstrategies. The purpose of this review is centered on the diagnostic and therapeutic approach of Langerhans cell histiocytosis in adults, which allows its recognition in early stages and improve the prognosis in people who suffer from it.