• 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.

• 대한세포병리학회:학술대회논문집
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• 대한세포병리학회 1991년도 제5차 춘계학술대회 초록집
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• pp.13.1-13.1
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• 1991

• 대한세포병리학회:학술대회논문집
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• 대한세포병리학회 1993년도 제7차 춘계학술대회 초록집
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• pp.25-26
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• 1993

• Journal of Photoscience
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• 제9권2호
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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.

• 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.

• Journal of Korean Dental Science
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• 제9권1호
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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.

• 대한두개안면성형외과학회지
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• 제14권1호
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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.

• Journal of Korean Neurosurgical Society
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• 제43권6호
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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.

• 대한영상의학회지
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• 제82권4호
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• pp.936-942
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• 2021

랑게르한스 세포 조직구증은 희귀한 질환으로 일반적으로 근골격계를 침범하며, 대부분 소아에게서 발생하고 성인의 쇄골에서 발병하는 경우는 극히 드물다. 이 증례 보고는 50세 남자에게서 영상학적으로 발견되어 병리학적으로 확진된 쇄골의 랑게르한스 세포 조직구증의 사례이다. 또한 저자들은 문헌고찰과 함께 단순촬영, 컴퓨터단층촬영, 자기공명영상법, 그리고 양전자방출단층촬영-컴퓨터단층촬영 영상 등의 다양한 영상검사 소견을 보고하고자 한다.

• 대한영상의학회지
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• 제84권2호
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• pp.472-476
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• 2023

코뼈에 발생하는 랑게르한스 세포 조직구증 증례는 거의 보고된 적이 없다. 저자들은 왼쪽 코에 만져지는 종괴를 주소로 내원한 13세 환자를 검사하였다. 초음파 검사상 코 좌측에 저음영 에코를 보이는 종괴가 관찰되었다. 자기공명영상 검사에서 경계가 분명하면서 비교적 균질한 조영증강을 보이는 종괴가 관찰되었다. 조직병리학적 검사에서 검체는 랑게르한스 세포 조직구증으로 확인되었다.