• 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.

• 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.

• 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.

• Journal of Chest Surgery
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• v.34 no.6
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• pp.506-510
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• 2001

Langerhans\` Cell Histiocytosis(LCH) is a disease of unknown origin, characterized by abnormal proliferation of Langerhans\` cells. Previously, it has been called histiocytosis X, which included eosinophilic granuloma, Hand-Sch ller-Christian\`s disease, and Letterer-Siwe disease. Any organs or tissues such as skin, lymph nodes, bone and bone marrow can be involved. However, LCH of chest wall is rarely reported in our country. We experienced a 18 month old male child, who had osteolytic lesion involving the rib with axillary lymph node metastasis. The tumor was confirmed as LCH after surgery.

• Archives of Craniofacial Surgery
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• v.10 no.1
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• pp.37-39
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• 2009

Purpose: Langerhans cell histiocytosis is a heterogenous group of Langerhans cell proliferative disorders and includes eosinophilic granuloma, Letterer-Siwe diseases, and Hand-Schuller Christian disease. We report a case of eosinophilic granuloma on frontal area. Methods: A 17-year-old male presented with swelling and tenderness on Lt. frontal and periorbital area. CT and MRI showed a $33{\times}25mm$ sized mass that involved Lt. frontal calvarium, frontotemporal meninges, and orbital roof. Results: Total excision of the mass and adjacent soft tissue, calvarium, and orbital roof was performed. Orbital roof defect was reconstructed with absorbable plate and calvarial defect was done with outer cortex of temporal bone flap. The histology revealed proliferation of histiocytes and eosinophils. Immunologically, these histiocytic cells expressed S-100 protein and CD1a. The patient is currently taking conservative treatment. Conclusion: The severity of these disease and their prognosis and treatments are various. For unifocal cranial Langerhans cell histiocytosis, complete excision is the treatment of choice. We report this case with review of literature.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.18 no.4
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• pp.647-651
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• 1996

Langerhans cell histiocytosis(LCH) appears to arise from Langerhans cell and comprises a spectrum of clinical disease previously described in the literature by a variety of eponyms including histiocytosis X, eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe syndrome. This rare disorder occurs in all groups, predominently affecting children & young adults. LCH has a wide spectrum of clinical features. The differentiation of several forms of this disease is primarily a clinical and not a histologic one. The radiographic characteristics include the appearance of solitary "intraosseous" lesions, the multiplicity of "alveolar bone" lesions, the bone lesions, periosteal new bone formation, and slight root resorption. Prognosis of a single bone lesion, is known to be excellent. In contrast, disseminated disease has seen associated with a chronic course, a high rate of morbidity and late consequences, and possible mortality. Treatment of LCH remains problematic. Treatment of multisystem disease, where organ function is being compromised has generally been with high-dose systemic corticosteroids or multiple chemotherapy.

• 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.

• 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.