• Tuberculosis and Respiratory Diseases
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• v.40 no.4
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• pp.424-430
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• 1993

Pulmonary eosinophilic granuloma or histiocytosis X is a chronic interstitial lung disease characterized by proliferations of Langerhans cells and, therefore, not truly histiocytosis. Both histiocytes and Langerhans cells are believed to be related to the mononuclear phagocyte system. In Eosinophilic granuloma, extra-pulmonary such as mediastinal or hilar lymph nodes involvement is very rare in adult. We report a case of young man with eosinophilic granuloma involving lung and anterior mediastinal lymph node simultaneously which is confirmed by open thoracotomy.

• Tuberculosis and Respiratory Diseases
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• v.45 no.2
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• pp.421-428
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• 1998

Pulmonary eosinophilic granuloma, a rare form of Langerhans cell histiocytosis confined to lung, has no known etiology and variable natural history. It is characterized by discrete proliferation of Langerhans cells. It is known that over 90% of the patients are cigarette smokers, and it is rarely reported in non- or passive-smoking patients, especially female. Here we describe a case of pulmonary eosinophilic granuloma in a passive-smoking, reproductive female patient presented with spontaneous pneumothorax. We identified S-100 and CDla positive histiocytes on immunohistochemical stain of the lung tissue obtained by open lung biopsy.

• Tuberculosis and Respiratory Diseases
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• v.39 no.2
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• pp.186-193
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• 1992

Eosinophilic granuloma (histiocytosis $\times$) is an uncommon granulomatous disorder affecting the mononuclear phagocytic cell system caused by unknown etiology. When confined to the lungs, it is also known as pulmonary eosinophilic granuloma or pulmonary histiocytosis $\times$. After the first case was described in 1951 by Farinacci et ai, more 250 cases were reported in the English literature. However only 4 cases has been introduced in our country. We describe two additional cases. In both cases, open lung biopsies are performed for definite diagnosis and examined by immunohistochemical stain for S-100 protein and electron microscopy for ultrastructural identification of Birbeck granule.

• Tuberculosis and Respiratory Diseases
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• v.41 no.2
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• pp.152-157
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• 1994

Histiocytosis-X is a tenn used to define three diseases with similar morphologic characteristics : Letterer-Siwe diseae, Hand-Schuller-Christian disease and Eosinophilic granuloma. In general, they differ in terms of their age of onset, severity of clinical course and site of involvement. Eosinophilic granuloma typically is seen in young adults. Eosinophilic granuloma is diagnosed in the presense of diffuse pulmonary infiltrate, bony involvement. However, the pulmonary radiologic findings of eosinophilic granuloma are variable accordinary to stage of disease. therefore pathologic diagnosis of involving site is essential for confirmative diagnosis of eosinophilic grananuloma. Pathologically. the three disease are characterized by granulomatous infiltration of alveolar septa and bronchial walls and often involvement of bone. The hallmark of this disease is proliferation of the Langerhans' cell. The identifying feature is the X-body or Birbeck granule that is present in Langerhans' cells and histiocytic cells found in the lung of EG patient. We report a case of bilatera1, recurrent and spontaneous pneumothoraces in a 21 year old man with pulmonary histiocytosis-X, which is confirmed by eosinophilc granuloma in bone marrow biopsy and ultrastructural examination in cells obtained from BAL.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.31 no.3
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• pp.279-282
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• 2005

Hitiocytosis X, recently termed Langergans cell histiocytosis, is a rare disease histologically characterized by an abnormal proliferation of histiocytes, and of which the pathophysiology is still ambiguous. The clinical presentation is highly varied. Typically, there is a solitary or multiple bony involvements, and less frequently, lesions are found in other organs such as the lungs, liver, lymph nodes, skin, and mucosae. Interestingly, so far, there has been no report which shows multiple developments in both soft and hard tissues. In this report, we present a case of hitiocytosis X in both mandible and lung. This may provide some information for understanding the pathophysilogy of the histiocytosis X.

• Tuberculosis and Respiratory Diseases
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• v.43 no.2
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• pp.164-172
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• 1996

Background : Transforming growth factor-$\beta$(TGF-$\beta$) may play a role in a variety of fibroproliferative disorders including pulmonary fibrosis via the induction of extracellular matrix accumulation. TGF-$\beta$ not only stimulates extracellular matrix production, but also decreases matrix degradation. Interstial lung diseases have demonstrated marked expression of TGF-$\beta$. Methods : To evaluate the possible role of TGF-$\beta$ in human pulmonary fibrosis, by using neutralizing antibody of TGF-$\beta$ we investigated immunohistochemically the expression of TGF-$\beta$ in the formalin-fixed, paraffin-embedded tissue sections of the 5 normal cases for the control, and a couple of pieces of tissues taken out of 3 cases with idiopathic pulmonary fibrosis, 3 cases with ILD from bleomycin toxicity, 3 cases with ILD from sarcoidosis, and 3 cases with ILD from eosinophilic granuloma. Results : In the 5 normal cases for the control, the TGF-$\beta$ was expressed in bronchial and alveolar epithelial cells. Up-regulation of the TGF-$\beta$ expression was showed in the interstitial fibroblast cells of alveolar septa in 5 pieces and proliferated alveolar pneumocytes in 1 piece among 6 pieces tissues taken out of 3 cases with idiopathic pulmonary fibrosis. Also up-regulation of the TGF-$\beta$ expression was showed in alveolar lining pneumocytes, intra-alveolar mononuclear cells, and epithelioid cells in most of cases of ILD from bleomycin toxicity, sarcoidosis and eosinophilic granuloma. Conclusion : These findings suggest that up-regulation of the TGF-$\beta$are involved in pathogenesis of interstitial lung fibrosis from variety of causes.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.30 no.6
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• pp.577-583
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• 2008

Langerhans cell histiocytosis (LCH) is characterized by proliferation of pathological Langerhans cells within different organs. It mainly affects children, but adult cases also occur, with an incidence rate of one to two per million. LCH results from the clonal proliferation of Langerhans cells. And its etiopathogenesis is still unknown. The hypothesis that it is a neoplastic or inflammatory disease, as well as the existence or not of immunological, viral or genetic predisposing factors, has been widely discussed in the literature, but no conclusive proof has ever been provided. Although lesions may appear in tissues of various origins such as skin, hypothalamus, liver, lung, or lymphoid tissue, bone is the most common site of the disease. The head and neck are affected in almost 90% of cases. The maxillary and mandibular bones are affected in 5 to 10% of cases. In our report, we present four cases of LCH in patients aged 3, 4, 7 and 9 years respectively, with primary manifestation in maxillofacial area.

• Tuberculosis and Respiratory Diseases
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• v.48 no.5
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• pp.802-807
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• 2000

Diffuse pulmonary nodular lesions have many causes. When they are caused by infection, the likely organisms are M. tuberculosis and various fungi. Silicosis, eosinophilic granuloma and pulmonary metastasis should be considered for differential diagnosis. Differential diagnosis needs detailed clinical history, physical examination and various laboratory tests. A case of persistent diffuse pulmonary nodular lesions which had persisted 5 years is reported. The patient was a 25 years old man with minimal pulmonary symptoms. Detailed past history and physical examination suggested thyroid tumor. Chest radiography showed numerous evenly sized well-defined nodules scattered in entire lung fields. Previous chest X-rays showed similar nodular lesions, which had lasted for 5 years. The number of nodules was slightly increased. Neck CT showed heterogenous mass in left lobe of thyroid gland and multiple lymphadenopathies along both internal jugular chains. Total thyroidectomy was performed. A case of lung metastasis which progressed slowly in papillary thyroid cancer is reported.

• Tuberculosis and Respiratory Diseases
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• v.51 no.4
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• pp.346-353
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• 2001

Background : Pulmonary Langerhans cell histiocytosis forms part of a spectrum of diseases that are characterized by a monoclonal proliferation and infiltration of organs by Langerhans cells. Several organ systems may be involved in Langerhans cell histiocytosis, including the lungs, bone, skin, pituitary gland, liver, lymph nodes and thyroid. Pulmonary histiocytosis X represents 2.8% of interstitial lung disease. Here we present the clinical, radiological, therapeutic aspects of pulmonary histiocytosis X. Method : Fourteen cases of biopsy-proven pulmonary histiocytosis X patients who were diagnosed in Seoul National University Hospital during the period from January 1990 to December 1998 were analyzed retrospectively. Result : There were 12 men and 2 women in this study. The initial presenting symptoms were dyspnea, cough, chest pain, which was associated with the pneumothorax, and chest radiography abnormalities. Only 8 patients (57%) were smokers. There were 5 patients with extra-pulmonary histiocytosis (pituitary, bone, skin). Eight patients had received the chemotherapy. There were no mortalities and only one patient experienced an aggravation of symptom during the follow-up period. Conclusion : In contrast to previous reports from other countries, the patients with pulmonary histiocytosis X in this study presented with several different clinical characteristics, such as a male predominance, relatively low smoker's rate, and a better prognosis.