• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.45 no.3
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• pp.167-172
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• 2019

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of dendritic cells resulting in local or systemic symptoms. The clinical symptoms of patients with Langerhans cell histiocytosis depend on the site and the degree of involvement. This article describes two case histories of unifocal bony Langerhans cell histiocytosis with mandibular involvement and further discusses the appropriate management of such via a review of the literature.

• 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.

• Journal of Chest Surgery
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• v.41 no.1
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• pp.137-140
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• 2008

Puimonary Langerhans cell histiocytosis is very rare, especially when accompanied by active pulmonary. tuberculosis. A patient was hospitalized due to excessive dyspnea and she was diagnosed with active pulmonary tuberculosis by a sputum AFB smear. The HRCT taken after hospitalization showed multiple micronodules and tiny cysts. An open lung biopsy confirmed the coexistence of pulmonary Langerhans cell histiocytosis.

• 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.

• Journal of Chest Surgery
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• v.43 no.4
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• pp.447-449
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• 2010

Langerhans cell Histiocytosis can present as a single or multiple lesion and can affect one or several organ systems. A 41-year-old woman with a history of multisystemic Langerhans cell Histiocytosis invading lung and thyroid was admitted with left-sided spontaneous pneumothorax. Here we report a case of uncommon pulmonary Langerhans-cell Histiocytosis presenting with spontaneous pneumothorax as a multisystemic Langerhans cell Histiocytosis.

• 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.1
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• pp.120-124
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• 2001

Sclerosing cholangitis is a chronic cholestatic disease characterized by inflammation and obliterative fibrosis of the bile ducts, leading to biliary cirrhosis and ultimately to liver failure. In children, sclerosing cholangitis is known to be associated with Langerhans cell histiocytosis, as well as with congenital immunodeficiencies and cystic fibrosis. Secondary sclerosing cholangitis is suspected in Langerhans cell histiocytosis with chronic cholestasis, liver dysfunction and portal hypertension. Unlike primary sclerosing cholangitis, the cholangitis associated Langerhans cell histiocytosis is destructive in nature and progresses more rapidly to biliary cirrhosis, therefore uniformly the prognosis is poor. In this setting, liver transplantation should be considered early in children with sclerosing cholangitis complicating Langerhans cell histiocytosis before end-stage liver failure and variceal bleeding. We experienced a case of secondary sclerosing cholangitis in Langerhans cell histiocytosis in a 2-year-old boy. We report this case with brief review of the related literatures.

• 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.

• Pediatric Infection and Vaccine
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• v.16 no.2
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• pp.220-223
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• 2009

Langerhans cell histiocytosis is a rare disease in children. However, Langerhans cell histiocytosis encompasses a wide spectrum of clinical presentations and mimics other conditions. A 1-year-old boy presented with signs of periorbital cellulitis that initially responded to antibiotics, but remained as a same-sized mass with serial orbital computed tomography. The lesion was partially excised. Histopathology and immunohistochemical staining confirmed the diagnosis of Langerhans cell histiocytosis. This case demonstrates that in patients with periorbital cellulitis which has relapsed or responded inadequately to antibiotics, further investigation should initiated to rule out other inflammatory causes.