Multisystemic Langerhans Cell Histiocytosis with advanced lung involvement

摘要

Langerhans cell histiocytosis is a rare disease of unknown cause, characterized by the proliferation of histiocytic cells (Langerhans cells), that can sometimes be especially difficult to diagnose due to its wide clinical spectrum, ranging from a single lesion to a multisystemic disorder. Appropriate disease staging is fundamental, since treatment depends upon the severity of the disease, and imaging methods play a fundamental role not only in diagnosing and assessing the extent of Langerhans cell histiocytosis, as well as guiding the appropriate treatment for the patient and their monitoring. Keywords: Langerhans cell histiocytosis, computed tomography, lung, skin diseasesCASE REPORTA 54-year old farmer presented with papular lesions on the scalp, ears, underarms, back, abdomen and oral cavity for four years, and an ulcerated lesion in the oral cavity (figure 1). He had a 26 pack year history of smoking, stopped smoking 10 years ago and was otherwise relatively well and asymptomatic and there was no significant past medical history. Physical examination revealed no other abnormality and no lymphadenopathy or organomegaly was detected. Hematologic investigations and biochemical blood tests were unremarkable. Biopsy of the ulcerated lesion of the oral cavity (figure 2) showed Langerhans cell histiocytosis (LCH) and immunohistochemistry was positive for CD1a and S100 protein. Treatment with thalidomide was started with improvement of skin lesions. Open in a separate windowFigure 1 A 54-year old man with multisystemic Langerhans cell histiocytosis. Multiple papular lesions on the scalp (A), underarms (B) and inguinal region (C), and ulcerated lesion in the oral cavity (D).