• 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.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.18 no.4
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• pp.718-725
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• 1996

Histiocytosis X is the abnormal proliferation of histiocytes and usually a massive aggregation of eosinophilic leukocytes. The clinical features and prognosises of subclasses of histiocytosis X depend on the severity and extent of the organ involvement. The subclasses include Letterer-Siwe disease(Acute disseminated histiocytosis X), eosinophilic granuloma, Hand-Schuller-Christian disease. Letterer-Siwe disease(acute, subacute form) refers to the form of disease that is most often fatal because of the widespread skeletal, extraskeletal lesion ; this usually affects infants and children less than 3 years of age. Eosinophilic granuloma(histiocytosis X, localized) refers to a chronic form of disease with skeletal lesion ; It usually affects children and young adult. Hand-Schuller-Christian disease(chronic form) refers to a chronic form of disease with skeletal, extraskeletal lesions. It usually affects children over the age of 3 years. In our hospital, 8 year old female patient visited complaining of hard swelling of mandibule body, left side and 6 year old female patient visited complaining of painful swelling of mandibular angle, right side. We diagnosed eosinophilic granuloma and curetted the lesiones and refered to Dept. pediatrics for chemotherapy. We present 2 cases to you with literatures.

• Tuberculosis and Respiratory Diseases
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• v.42 no.3
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• pp.419-423
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• 1995

Pulmonary histiocytosis X is an idiopathic benign disease characterized by proliferation and infiltration of lung tissue by characteristic Langerhans cells and eosinophils. Pulmonary histiocytosis X is common in young male adults, and shows variable clinical characteristics. We experienced a case of pathologically proven pulmonary histiocytosis X in a 30-year-old man who visit to our hospital due to chest discomfort and cough. The chest radiograph of our patient shows right pneumothorax and characteristic multiple thin-walled cysts on the both upper lung fields. The HRCT shows multiple thin-walled cysts, a few scattered nodules in both upper and right middle lung, and right pneumothorax.

• The Korean Journal of Cytopathology
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• v.4 no.2
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• pp.140-145
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• 1993

Langerhans cell histiocytosis or histiocytosis X is a disease of unknown etiology characterized by proliferation of mature histiocytes. While a few descriptions of the cytologic features of eosinophilic granuloma ocurring in the bone have been published, reports of cytologic findings of lymph node-based Langerhans cell histiocytosis are very rare. We report the cytologic findings of a case of Langerhans cell histiocytosis diagnosed by fine needle aspiration cytology from the left supraclavicular and right inguinal lymph nodes in a 65-year-old male. Cytologic smears showed characteristic reticuloendothelial cells which have elongated, folded, grooved nuclei and abundant pale cytoplasms. Particularly, nuclei were highly irregular and multilobated. A few mitotic figures were present. The cytologic diagnosis was confirmed by tissue biopsies from the left supraclavicular and right inguinal lymph nodes. Proliferation of histiocytes were also present in the skin. Immunohistochemistry for S-100 protein, vimentin, $\alpha1-antichymotrypsin$ and lysozyme showed positive staining. Electron microscopy disclosed Birbeck granules.

• Journal of Chest Surgery
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• v.36 no.7
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• pp.539-543
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• 2003

Langerhans cell histiocytosis (LCH), previously called histiocytosis X, refers to a spectrum of disease characterized by idiopathic proliferation of histiocytes producing focal or systemic manifestations. Causes and pathogenesis remain unclear The basic histopatholgic fndings are identical in the three well-established clinical syndromes (eosinophilic granuloma, Hand-Shuller-Christian disease, Letterer-Siwe disease). The disease has a predilection for children, although it may occur in adults. We experienced two cases of adult onset Langerhans cell histiocytosis in the ribs. One case was associated with diabetes insipidus and the other case had a solitary lesion.

• 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.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.19 no.1
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• pp.55-60
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• 1997

Langerhans Cell Histiocytosis(Idiopathic Histiocytosis, Histiocytosis-X) is most often found in children and young adults, and cell proliferation with specific phenotype shows ultrastructural and immunohistochemical similarities with Langerhans Cells that normally exist in epithelium and mucosa. This disease occurs as single or multiple lesions in skull, ribs, vertebrae, mandible and long bones, and when it involves mandible, clinical sign and symptoms such as bone swelling and pain are noticed. When it involves alveolar bone, severe tooth mobility as well as gigival inflammation, proliferation, and ulceration are commonly found, and so it is not easy to differentiate it from general inflammatory diseases. Any local lesion at the tooth apex on the x-ray view needs to be differentiated from inflammatory disease, and multiple lesions from multiple ostoeoma and chronic multifocal osteomyelitis. This case is LCH in 51-year-old male patient ; this is a rare case, for the patient belongs to an age group with very low incidence rate of the disease. although three-timed biopsy tests and longterm observation at two university hospitals, it was misdiagnosed as multifocal osteomyelitis.

• Journal of Korean Neurosurgical Society
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• v.30 no.sup1
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• pp.124-127
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• 2001

Langerhans' cell histiocytosis is a rare disease showing proliferation of histiocytes in multiple organ system. Bone lesions are the most common radiolologic manifestation of Langerhans' cell hysticytosis. Extraskeletal sites of Langerhans' cell hystiocytosis involvement include the skin, lymph nodes, thymus, lungs, central nervous system, liver, pancrease, spleen, and bowel. The authors have experienced a case of Langerhans' cell histiocytosis which involved the skull. A 3-year-old female presented with soft tissue mass on right periorbital area. Plain skull X-ray showed punched out bone lesion. Computed tomography showed non-enhancing osteolytic lesion on right frontal skull. Histologic findings of an excised mass revealed pathologic features of Langerhans' cell histiocytosis.

• Applied Microscopy
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• v.8 no.1
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• pp.17-22
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• 1978

Two cases of Histiocytosis X, a Hand-Schuller-Christian disease and a Letterer-Siwe disease, are,examined light and electron microscopically. Many of the histiocytes without appreciable lipid contained numerous rod-shaped structure identical to Langerhan's granules in the cytoplasm. The significance of this structure was discussed together with review of literature.

• 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.