• 제목/요약/키워드: Langerhans cell

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LANGERHANS CELL HISTIOCYTOSIS IN THE JUVENILE MANDIBLE (소아의 하악골을 침범한 랑거한스세포 조직구 증식증의 임상적 고찰)

  • Kang, Yeon-Hee;Park, Se-Hyun;Seo, Dong-Jun;Cha, In-Ho;Yi, Choong-Kook;Kim, Hyun-Sil;Kim, Jin;Kim, Hyung-Jun
    • 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.

Topical Irradiation of UVA to The Eye Induces Immunosuppression in The Mice via Nitric-Oxise Dependent Neuronal Pathways

  • Hiramoto, Keiichi;Yanagihara, Nobuyo;Sato, Eisuke F.;Inoue, Masayasu
    • Journal of Photoscience
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    • v.9 no.2
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    • pp.470-471
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    • 2002
  • It has been well documented that dermal irradiation by ultraviolet A (UVA) locally decreases the number of Langerhans cells and suppresses contact hypersensitivity of the skin. We found that topical irradiation of UVA to the eye systemically decreased the number of Langerhans cells (LC) in the dorsalskin and lymph nodes and elicited lymphocyte apoptosis in the latter tissues but not in the thymus. Optic nerve resection, but not ciliary ganglionectomy, eliminated the UVA-induced decrease in dermal Langerhans cells by a mechanism that was partially inhibited by hypophysectomy. The immunosuppressive effect of UVA was not observed in knockout mice lacking inducible-type of nitric oxide synthase (iNOS). These results suggested that topical irradiation of UVA to the eye induced immunosuppression via NO-dependet neuronal pathways.

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Langerhans cell histiocytosis

  • Oh, Su-Jin;Kim, Cheoul-Hun;Choi, Soel-Hi;Hoe, Jun;Park, Sung-Hwan;Jang, Chang-Dug;Shin, Sang-Hun;Hwang, Hee-Sung
    • 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.

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Treatment and Rehabilitation of Repetitively Recurrent Langerhans Cell Histiocytosis: A Case Report

  • Yoo, Hee Young;Park, Kyung Soo;Lee, Baek Soo;Kwon, Yong Dae;Choi, Byung Joon;Ohe, Joo Young;Lee, Jung Woo
    • Journal of Korean Dental Science
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    • v.9 no.1
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    • pp.35-41
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    • 2016
  • Langerhans cell histiocytosis (LCH) is characterized by proliferation of histiocyte-like cells (Langerhans cell histiocytes) with characteristic Birbeck granules, accompanied by other inflammatory cells. Treatments of LCH include surgery, chemotherapy, and radiotherapy. One of the representative forms of chemotherapy is intralesional injection of steroids. Surgical treatment in the form of simple excision, curettage, or even ostectomy can be performed depending on the extent of involvement. Radiotherapy is suggested in case of local recurrence, or a widespread lesion. This article shows the case of repetitively recurrent LCH of a 56-year-old man who had been through surgical excision and had to have marginal mandibulectomy and radiotherapy when the disease recurred. After the first recurrence occurred, lesions involved the extensive part of the mandible causing pathologic fracture, so partial mandibular bone resection was performed from the right molar area to the left molar area followed by the excision of the surrounding infected soft tissues. The resected mandibular bone was reconstructed with a segment of fibula osteomyocutaneous free flap and overdenture prosthesis supported by osseointegrated implants.

Unifocal Langerhans Cell Histiocytosis of Frontal Bone in a Child (소아 전두골에 발생한 랑게르한스세포 조직구증)

  • Hong, Seong Jae;Cho, Sang Hun;Eo, Su Rak
    • Archives of Craniofacial Surgery
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    • v.14 no.1
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    • pp.69-72
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    • 2013
  • Lateral eyebrow mass with primary skull lesion are rare in pediatric population. Although epidermoid cyst and dermoid cyst are the most commonly encountered skull lesions in pediatric population, Langerhans cell histiocytosis (LCH) is rarely reported. We report a case of LCH arising from the lateral eyebrow with osteolytic lesion involving the frontal bone. A 5-year-old boy was presented with a hard, fixed mass in his lateral eyebrow. Contrast magnetic resonance imaging revealed inhomogeneous enhancement of the mass with direct invasion of the frontal bone and adjacent dura mater. Under general anesthesia, linear incision at the lateral eyebrow region was made. Intraoperative evaluation revealed hard, fixed and well-defined soft tissue mass. The final extirpated mass was $2.5{\times}2.4cm$ in size, and was accompanied by a $1{\times}1cm$ sized defect on the frontal bone with intact dura mater. The surgical wound was closed primarily by a layer-by-layer fashion. Histologic examination was later performed for definite diagnosis. The histologic examination revealed abnormal proliferation of Langerhans cell with granuloma formation. Radionuclide bone scan and positron emission tomography was taken and revealed free of multi-organ involvement. At 3 months after surgery, natural looking contour at the lateral eyebrow region was observed with no tumor recurrence. Differential diagnosis of the hard and fixed mass at the lateral eyebrow region affecting the primary skull lesion from pediatric population includes epidermoid cyst, dermoid cyst and LCH. Generally, brief physical examination with plain X-ray view can be performed for clinical evaluation, but for a definite diagnosis, contrast MRI may be helpful.

Eosinophilic Granuloma Presenting as an Epidural Hematoma and Cyst

  • Lee, Young-Suk;Kwon, Jeong-Taik;Park, Yong-Sook
    • Journal of Korean Neurosurgical Society
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    • v.43 no.6
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    • pp.304-306
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    • 2008
  • Langerhans' cell histiocytosis (LCH) is a rare immunologic disorder characterized by histiocyte proliferation in multiple organ systems. Eosinophilic granuloma, a benign bone lesion, represents a focal form of LCH. We experienced a case of Langerhans' cell histiocytosis in a patient who presented with intracranial epidural hematoma and cyst on the midline of the frontal skull. A 10-year-old boy presented with a rapidly growing large scalp mass on the midline frontal area after mild head trauma. The scalp mass was painless and immobile. Plain skull x-ray showed a punched-out bone lesion. Computed tomography and magnetic resonance imaging showed a non-enhancing osteolytic lesion presenting with an epidural hematoma and cyst on the midline of the frontal skull. The lesion of the skull was completely resected and the patient's recovery was uneventful. The acute presentation of a solitary eosinophilic granuloma of skull with an epidural hematoma has been described in only five cases in the literature and we report the first case of LCH presenting as an intracranial epidural hematoma on frontal area.

Langerhans Cell Histiocytosis of the Clavicle in a 50-Year-Old Male: A Case Report (50세 남자에게서 발견된 쇄골의 랑게르한스 세포 조직구증: 증례 보고)

  • Changhyun Park;Yong Hoon Kim;Soon Joo Cha;Ji-Ye Kim
    • Journal of the Korean Society of Radiology
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    • v.82 no.4
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    • pp.936-942
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    • 2021
  • Langerhans cell histiocytosis (LCH) is a rare condition that usually occurs in children and commonly affects the skeletal system. It is extremely rare in adults, especially in the clavicles. In this report, we describe a pathologically confirmed case of LCH in the clavicle of a 50-year-old male. We report various radiological findings, such as plain radiography, CT, MR, and PET-CT, along with a review of the literature.

Langerhans Cell Histiocytosis in the Nasal Bone: A Rare Case (코뼈에 발생한 랑게르한스 세포 조직구증: 증례 보고)

  • Won Ik Ahn;Jong Chang Jang;Dong Jin Yang;Tae Eun Kim;Hyou Chun Park;Dong Kyu Lee;Jae Min Seong
    • Journal of the Korean Society of Radiology
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    • v.84 no.2
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    • pp.472-476
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    • 2023
  • Nasal bone involvement of Langerhans cell histiocytosis is rarely reported. Here we present a case of a 13-year-old boy with a palpable nasal mass. Ultrasonography revealed a hypoechoic mass on the left side of the nose. Both CT scanning and MRI showed an osteolytic mass. The lesion seen on MRI was well-defined mass with homogeneous enhancement. Histopathological examination of the resected specimen confirmed the diagnosis of LCH.