• Journal of Chest Surgery
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• 제36권7호
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• pp.539-543
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• 2003

Langerhans 세포 조직구증은 과거에 조직구증 X로 불려진 질환으로서, 국소적 혹은 전신증상을 나타내며 비특이적인 조직구 증식을 특징으로 하는 질환군을 일컫는 말이다. 이 질환의 원인과 병리 기전은 아직 밝혀지지 않았으며, 호산구성 육아종, Hand-Shuller-Christian병, Letterer-Siwe병으로 대별되는 세 가지 질환 군에서 기본적인 병리학적 소견은 동일하다. 주로 어린 나이에 발생하나 어른에서도 발생한다. 저자들은 성인에서 늑골에 발생한 호산구성 육아종을 2예 치험하였다. 한 예는 요붕증을 동반하였고 다른 한 예는 늑골에만 발생한 경우였다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제18권4호
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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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• 제8권1호
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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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• 제34권6호
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• pp.506-510
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• 2001

Langerhans 세포 조직구증(LCH, Langerhans\` Cell Histiocytosis)은 Langerhans\` cell histiocyte의 이상 증식을 특징으로 하는 원인 불명의 질환이다. 이 질환은 eosinophilic granuloma, Hand-Sch ller-Christian씨 병, Letterer-Siwe병을 포함하는 것으로 과거에는 histiocytosis X로 불리던 질환이다. 피부, 림프절, 골, 골수 및 체내 모든 조직과 기관을 침범할 수 있으나 국내에서 흉벽에서 발생된 예는 보고된 증례가 많지않다. 18개월 된 남자 환아에서 흉벽의 늑골에서 기원하여 골용해 소견을 동반한 종괴가 있어 수술적 절제한 후 LCH로 확진된 증례가 있어 문헌고찰과 함께 보고하는 바이다

• 대한두개안면성형외과학회지
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• 제10권1호
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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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• 제18권4호
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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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• 제41권2호
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• pp.152-157
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• 1994

본 저자들은 양측성으로 반복되는 자연기흉 소견을 보인 21세 남자 환자에서BAL은 시행하여 조직구내 Birbeck granule을 찾음으로써 확진할 수 있었던 골 침범을 동반한 폐 조직구증 1예를 문헌 고찰과 함께 보고하는 바이다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제30권6호
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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.