• 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.

• 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.

• Clinical and Experimental Pediatrics
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• v.50 no.6
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• pp.524-530
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• 2007

The recent advances in the basic hematology and immunology have significantly enhanced the understanding of histiocytic disorders. The Histiocyte Society which was established in 1985 enabled the randomized trials for these diseases, and important knowledge regarding pathogenesis, clinical presentation, diagnosis, therapy and late consequences has been obtained. The treatment of Langerhans cell histiocytosis (LCH) has varied greatly over last decades, and is still controversial. Therapy can be reduced for low risk patients, and it is possible to discriminate early the non-responding patients with risk disease who might require more intensified treatment. Current therapy of LCH recommended by the Histiocyte Society (LCH-III protocol) is activated in 2001. Hemophaocytic histiocytosis (HLH) is fatal if diagnosis is delayed and appropriate therapy is not instituted rapidly. The diagnostic criteria for HLH is revised by the Histiocyte Society for the current treatment protocol (HLH-2004) which consists of dexamethasone, etoposide, and cyclosporin in combination with intathecal methotrexate. Hematopoietic stem cell transplantation is usually necessary for the primary HLH and recurrent secondary HLH.

• Journal of Photoscience
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• v.9 no.2
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• pp.194-196
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• 2002

While ultraviolet radiation alters various cutaneous cell functions, little is known about photo-immunological and photobiological effects of infrared radiation (IR) on the skin except its local thermal effects. The fIrst part of this study demonstrated that single exposure of mouse skin to near IR (0.7 - 1.3 $\mu$m) reversibly suppressed the proliferating activity of the epidermis, the density of Langerhans cells, and the ability of skin to induce contact hypersensitivity reaction. The second part demonstrated that the rate of wound closure was significantly accelerated by repeated exposures in animal models. The production of transforming growth factor-$\beta$l and matrix metalloproteinase-2, which are responsible for the wound healing processes, was significantly upregulated by irradiation, as shown by enzyme immunoassay, zymography, and reverse transcription polymerase chain reaction. Thermal controls were negative. The results suggest that near-IR irradiation can modulate the epidermal proliferation and part of the skin immune system, and stimulate the wound healing processes, presumably by non-thermal effects.

• Journal of Chest Surgery
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• v.51 no.3
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• pp.223-226
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• 2018

Erdheim-Chester disease (ECD) is a form of non-Langerhans cell histiocytosis that most commonly involves the skeletal system. We report an unusual case of ECD presenting as an anterior mediastinal tumor without skeletal involvement. A 60-year-old man with no remarkable medical history was referred for evaluation of a mediastinal mass. The patient underwent surgical excision of the tumor via video-assisted thoracoscopic surgery. Histologic examination revealed marked proliferation of atypical histiocytes with sclerosis, and the results of immunohistochemical staining were suggestive of ECD.

• 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.

• Imaging Science in Dentistry
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• v.43 no.2
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• pp.117-122
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• 2013

Eosinophilic granuloma is a common expression of Langerhans cell histiocytosis and corresponds with typical bone lesions. The radiographic appearance of eosinophilic granuloma in the jaw is variable and not specific. It may resemble periodontitis, radicular cyst, or malignancies. The purpose of this report is to describe the characteristic radiographic features of eosinophilic granuloma of a 39-year-old male. The lesion in the anterior mandible was first diagnosed as radicular cyst because the radiographic findings were ovoid radiolucent lesion with well-defined border. However, careful interpretation revealed a non-corticated border and floating tooth appearance that were the characteristic radiographic features for the differential diagnosis. Early clinical signs of eosinophilic granuloma can occur in the jaw and a bony destructive lesion might be mistaken for periodontitis or an odontogenic cystic lesion; therefore, careful interpretation of radiographs should be emphasized.

• Tuberculosis and Respiratory Diseases
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• v.45 no.2
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• pp.421-428
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• 1998

Pulmonary eosinophilic granuloma, a rare form of Langerhans cell histiocytosis confined to lung, has no known etiology and variable natural history. It is characterized by discrete proliferation of Langerhans cells. It is known that over 90% of the patients are cigarette smokers, and it is rarely reported in non- or passive-smoking patients, especially female. Here we describe a case of pulmonary eosinophilic granuloma in a passive-smoking, reproductive female patient presented with spontaneous pneumothorax. We identified S-100 and CDla positive histiocytes on immunohistochemical stain of the lung tissue obtained by open lung biopsy.

• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.26 no.1
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• pp.58-62
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• 2015

Xanthoma disseminatum (XD) is a benign, non-Langerhans cell histiocytosis characterized by disseminated xanthomatous lesions with face, flexures, and mucosa. Most of XD develops in mucocutaneous lesions including skin, oral cavity and pharynx, however laryngeal involvement is uncommon. While the natural course of XD is usually benign and often self-limiting, but XD develop in critical anatomical locations may result in morbidity and mortality. Localized mucous lesions in oropharynx and larynx lead to dysphagia, dyspnea and air way obstruction. The diagnosis of XD was based on clinical, histological and immunohistochemical findings. The treatment is complex and non-consensual. Local treatment with cryotherapy, radiotherapy, surgery, and carbon dioxide lasers have been attempted with various results. Systemic medication with peroxisome proliferator-activated gamma receptors, statins, fenofibrate, chlorodeoxyadenosine, cyclophosphamide, doxycycline, and cyclosporine have also been reported, but none have proven particularly successful. A 59-year-old man presented with respiratory symptoms because of laryngeal involvement of XD. We had to remove the obstructive lesion for relieving the symptoms. We experienced XD in Larynx that was rare in otorhinolaryngology. So we report this case with review of literatures.

• Molecules and Cells
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• v.44 no.5
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• pp.310-317
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• 2021

Cluster of differentiation 1 (CD1) is a family of cell-surface glycoproteins that present lipid antigens to T cells. Humans have five CD1 isoforms. CD1a is distinguished by the small volume of its antigen-binding groove and its stunted A' pocket, its high and exclusive expression on Langerhans cells, and its localization in the early endosomal and recycling intracellular trafficking compartments. Its ligands originate from self or foreign sources. There are three modes by which the T-cell receptors of CD1a-restricted T cells interact with the CD1a:lipid complex: they bind to both the CD1a surface and the antigen or to only CD1a itself, which activates the T cell, or they are unable to bind because of bulky motifs protruding from the antigen-binding groove, which might inhibit autoreactive T-cell activation. Recently, several studies have shown that by producing T_H2 or T_H17 cytokines, CD1a-restricted T cells contribute to inflammatory skin disorders, including atopic dermatitis, psoriasis, allergic contact dermatitis, and wasp/bee venom allergy. They may also participate in other diseases, including pulmonary disorders and cancer, because CD1a-expressing dendritic cells are also located in non-skin tissues. In this mini-review, we discuss the current knowledge regarding the biology of CD1a-reactive T cells and their potential roles in disease.