• 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.19 no.1
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• pp.55-60
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• 1997

Langerhans Cell Histiocytosis(Idiopathic Histiocytosis, Histiocytosis-X) is most often found in children and young adults, and cell proliferation with specific phenotype shows ultrastructural and immunohistochemical similarities with Langerhans Cells that normally exist in epithelium and mucosa. This disease occurs as single or multiple lesions in skull, ribs, vertebrae, mandible and long bones, and when it involves mandible, clinical sign and symptoms such as bone swelling and pain are noticed. When it involves alveolar bone, severe tooth mobility as well as gigival inflammation, proliferation, and ulceration are commonly found, and so it is not easy to differentiate it from general inflammatory diseases. Any local lesion at the tooth apex on the x-ray view needs to be differentiated from inflammatory disease, and multiple lesions from multiple ostoeoma and chronic multifocal osteomyelitis. This case is LCH in 51-year-old male patient ; this is a rare case, for the patient belongs to an age group with very low incidence rate of the disease. although three-timed biopsy tests and longterm observation at two university hospitals, it was misdiagnosed as multifocal osteomyelitis.

• 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.

• 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.

• 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.

• Korean Journal of Head & Neck Oncology
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• v.33 no.2
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• pp.81-84
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• 2017

Langerhans cell histiocytosis (LCH) is a heterogeneous disease, characterized by accumulation of dendritic cells with features similar to epidermal Langerhans cells. It is a rare entity that may involve various organ levels such as the skeletal, pulmonary, hematopoietic and lympho-vascular systems. The patient was a 1-year-old female presented with fever associated with otorrhea and palpable cervical lymph node for 4 days. Neck ultrasonography and Computed tomography imaging revealed multiple enlarged lymph nodes suggesting suspicious malignant morphology. Lymph node biopsy was performed under general anesthesia. Histological and immunophenotypic examination showed the lymph node to be consistent with LCH. The patient was given chemotherapy.

• 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.

• 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.

• 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.

• The Journal of the Korean bone and joint tumor society
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• v.19 no.1
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• pp.28-32
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• 2013

Erdheim Chester disease (ECD) is very rare non-Langerhans cell histiocytosis (LCH) which occurs in the skeletal system and multiple organs. As it is progressive, sometimes it causes fatal results. However, it is often misdiagnosed as LCH or multiple bone metastasis and, thus, is very difficult to diagnose. In Korea, only 10 cases were first reported in 1999. In particular, there have been a few orthopedic approaches or reports in English-speaking literatures, and no report has been issued in Korea. The authors performed bone biopsy in patients with knee and lower extremity pain who were referred for the integrated treatment. We attempts to report this diagnosis experience with literature review.