Tuberc Respir Dis.  2015 Oct;78(4):463-468. 10.4046/trd.2015.78.4.463.

Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report

• Choi YS
• Lim JS
• Kwon W
• Jung SH
• Park IH
• Lee MK
• Lee WY
• Yong SJ
• Lee SJ
• Jung YR
• Choi J
• Choi JS
• Jeong JT
• Yoo JS
• Kim SH

• KMID: 2320729
• DOI: http://doi.org/10.4046/trd.2015.78.4.463

Abstract

Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus.

Keyword

Histiocytosis, Langerhans-Cell; Cystic Disease of Lung; Diabetes Insipidus; Diabetes Mellitus; Smoking Cessation

MeSH Terms

Adult*
Biopsy
Cough
Diabetes Insipidus
Diabetes Insipidus, Neurogenic*
Diabetes Mellitus*
Diabetes Mellitus, Type 2
Dyspnea
Follow-Up Studies
Histiocytosis, Langerhans-Cell*
Humans
Lung
Lung Diseases
Magnetic Resonance Imaging
Male*
Polydipsia
Smoke
Smoking
Smoking Cessation
Thoracic Surgery, Video-Assisted
Water Deprivation
Smoke

Figure

• 

  Figure 1 Magnetic resonance imaging of the hypothalamic-pituitary system. The hyperintense signal of the posterior pituitary on the T1-weighted image could not be detected. Lack of this bright spot (arrow) is typical of central diabetes insipidus.

• 

  Figure 2 Computed tomographic scans of the chest. (A) Multiple irregular cysts and centrilobular nodules can be seen in both lung fields, with sparing of the costophrenic angles. (B) Follow-up scan at 1-year after the patient stopped smoking. The size and thickness of the irregular cysts are reduced, and both lungs show a decrease in the number of centrilobular nodules.

• 

  Figure 3 Langerhans cell histiocytosis. (A) The cut surface of wedge-resected lung shows numerous cystic spaces with whitish gray stellate fibrous scars. (B) Multiple cystic spaces are evident, with diffuse thickening, cellular infiltration, and fibrous tissue (H&E stain, ×12.5). (C) The cytoplasm of the infiltrated cells is pale and eosinophilic, and the nuclei are grooved or infolded (H&E stain, ×400). (D) Immunohistochemical staining of the proliferating cells is diffuse and strongly positive for CD1a (×400).

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