A Case of Primary Pulmonary Histiocytosis-X associated With Central diabetes insipidus.

Kim, Young Min; Park, Yung In; Choi, Young Kuen; Lee, Jae Seung; Lee, Woo Chul; Hong, Jin Hee; Lee, Soo Bong; Reu, Ki Chan; Lee, Min Ki; Lee, Chang Hun; Lee, Hyoung Ryel; Park, Soon Kew

doi:1999.46.1.110

Tuberc Respir Dis > Volume 46(1); 1999 > Article

Case Report

Tuberculosis and Respiratory Diseases 1999;46(1):110-115.
DOI: https://doi.org/10.4046/trd.1999.46.1.110 Published online January 1, 1999.

A Case of Primary Pulmonary Histiocytosis-X associated With Central diabetes insipidus.

Young Min Kim, Yung In Park, Young Kuen Choi, Jae Seung Lee, Woo Chul Lee, Jin Hee Hong, Soo Bong Lee, Ki Chan Reu, Min Ki Lee, Chang Hun Lee, Hyoung Ryel Lee, Soon Kew Park

¹Department of Internal Medicine, Pusan National University, College of Medicine, Pusan, Korea.
²Department of Pathology, Pusan National University, College of Medicine, Pusan, Korea.
³Department of Thoracic surgery, Pusan National University, College of Medicine, Pusan, Korea.

Abstract

Pulmonary histiocytosis X is a granulomatous disorder of the lung of unknown cause. Patients with this disease often complain of cough, dyspnea on exertion and , occaionally, chest pain from pneumothorax or bone involvement. However, DI is uncommon in these patients. We report a case of primary pulmonary histiocytosis X with central diabetes insipidus. A 23-year-old man presented with dyspnea suffered from dry cough, exertional dyspnea, polydipsia and polyuria for 4 months. He was a heavy smoker. He was found to have reticulonodular interstitial opacities on chest X-ray film. High-resolution computed tomography revealed thin-walled cysts of various sizes in both lungs. Open lung biopsy was done. On light microscopic examination revealed proliferation and infiltration of Langerhans cells. Immunohistochemically, Langerhans cells showed strong cytoplasmic staining with S-100 protein and electronmicroscopic examination showed Birbeck granules in Langerhans cells. Water deprivation test showed central-type diabetes insipidus and brain MRI showed no abnormal lesion on suprasellar region. Smoking cessation was recommended. He was treated with oral desmopressin.

Key Words: Pulmonary histiocytosis X, Birbeck granules, central diabetes insipidus