Tuberc Respir Dis.  1999 Jan;46(1):110-115. 10.4046/trd.1999.46.1.110.

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

• Kim YM
• Park YI
• Choi YK
• Lee JS
• Lee WC
• Hong JH
• Lee SB
• Reu KC
• Lee MK
• Lee CH
• Lee HR
• Park SK

• KMID: 1842433
• DOI: http://doi.org/10.4046/trd.1999.46.1.110

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.

Keyword

Pulmonary histiocytosis X; Birbeck granules; central diabetes insipidus

MeSH Terms

Biopsy
Brain
Chest Pain
Cough
Cytoplasm
Deamino Arginine Vasopressin
Diabetes Insipidus
Diabetes Insipidus, Neurogenic*
Dyspnea
Histiocytosis, Langerhans-Cell*
Humans
Langerhans Cells
Lung
Magnetic Resonance Imaging
Pneumothorax
Polydipsia
Polyuria
S100 Proteins
Smoking Cessation
Thorax
Water Deprivation
X-Ray Film
Young Adult
Deamino Arginine Vasopressin
S100 Proteins