Massaccesi C, Colella S, Fioretti F, D'Emilio V, Panella G, Primomo G, Barbisan F, Pela R, Poletti V
Pulmonary Unit, "C. e G. Mazzoni" Hospital, Ascoli Piceno, Italy.
Department of Pathology, "Ospedali Riuniti", Ancona, Italy.
Respir Med Case Rep. 2017 Jan 16;20:116-119. doi: 10.1016/j.rmcr.2017.01.006. eCollection 2017.
Erdheim- Chester disease is a rare non- Langerhans cell histiocytosis that usually involves the bones, heart, central nervous system, retroperitoneum, eyes, kidneys, skin and adrenals. Lungs are affected in up to one-half cases; at CT scan various patterns are described: interstitial disease, consolidations, micronodules and microcysts, with or without pleural involvement. We presented a case of a 59 year-old man with unusual intrathoracic manifestation of Erdheim- Chester disease. Singularities of our report are the lonely thoracic involvement at the onset of the disease and a histiocytic lesion in the posterior mediastinum.
Erdheim-Chester病是一种罕见的非朗格汉斯细胞组织细胞增多症,通常累及骨骼、心脏、中枢神经系统、腹膜后、眼睛、肾脏、皮肤和肾上腺。肺部受累高达一半的病例;CT扫描描述了多种模式:间质性疾病、实变、微结节和微囊肿,伴或不伴胸膜受累。我们报告了一例59岁男性,患有Erdheim-Chester病不寻常的胸内表现。我们报告的独特之处在于疾病初发时仅有胸部受累以及后纵隔的组织细胞病变。