• 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.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.31 no.3
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• pp.249-253
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• 2009

Langerhans cell histiocytosis(LCH), previously known as histiocytosis X, is rare, proliferative disorder in which the accumulation of pathologic Langerhans cell leads to tissue infiltration and destruction. The jaw is involved 10~20% of all LCH and most common oral symptoms are jaw swelling or a palpation mass. Most of subjects are males and disease appears to start before the age of 10 years. An 11 years old girl was referred from pediatric department. Histological examination confirmed the diagnosis LCH. She was treated intra-lesional injection of corticosteroid. The overall outcome was excellent. After a follow-up period of 20 month, the patient present with no evidence of residual disease on Panoramic view and whole body bone scan. We report this early recognized LCH case treated by corticoid injection in good progress with literature review.

• Archives of Craniofacial Surgery
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• v.10 no.1
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• pp.37-39
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• 2009

Purpose: Langerhans cell histiocytosis is a heterogenous group of Langerhans cell proliferative disorders and includes eosinophilic granuloma, Letterer-Siwe diseases, and Hand-Schuller Christian disease. We report a case of eosinophilic granuloma on frontal area. Methods: A 17-year-old male presented with swelling and tenderness on Lt. frontal and periorbital area. CT and MRI showed a $33{\times}25mm$ sized mass that involved Lt. frontal calvarium, frontotemporal meninges, and orbital roof. Results: Total excision of the mass and adjacent soft tissue, calvarium, and orbital roof was performed. Orbital roof defect was reconstructed with absorbable plate and calvarial defect was done with outer cortex of temporal bone flap. The histology revealed proliferation of histiocytes and eosinophils. Immunologically, these histiocytic cells expressed S-100 protein and CD1a. The patient is currently taking conservative treatment. Conclusion: The severity of these disease and their prognosis and treatments are various. For unifocal cranial Langerhans cell histiocytosis, complete excision is the treatment of choice. We report this case with review of literature.

• Journal of Chest Surgery
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• v.34 no.6
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• pp.506-510
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• 2001

Langerhans\` Cell Histiocytosis(LCH) is a disease of unknown origin, characterized by abnormal proliferation of Langerhans\` cells. Previously, it has been called histiocytosis X, which included eosinophilic granuloma, Hand-Sch ller-Christian\`s disease, and Letterer-Siwe disease. Any organs or tissues such as skin, lymph nodes, bone and bone marrow can be involved. However, LCH of chest wall is rarely reported in our country. We experienced a 18 month old male child, who had osteolytic lesion involving the rib with axillary lymph node metastasis. The tumor was confirmed as LCH after surgery.

• 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.

• The Korean Journal of Cytopathology
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• v.2 no.2
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• pp.153-159
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• 1991

Langerhans' cell histiocytosis (LCH), known for histiocytosis X, is a clinicopathologic entity characterized by proliferation of Langerhans' cells (LCs) throughout the body including the reticuloendothelial system, bone, and skin. LCs is currently considered as a distinct type of histlocytic cells, not primarily phagocytic in nature. Recently, we could make the diagnosis on cytologic specimen in a 3 month-old-boy and a 3 year-old-boy. The cases were diagnosed on scraping smear from the skin and fine needle aspiration cytology from the lymph node, respectively. The characteristic cytologic features of Langerhans' cells were noted in the nuclei, namely eccentric, indented, elongated, and grooved nuclei. The cells also had abundant and acidophilic cytoplasm. The cytologic diagnoses were confirmed on the biopsies from the skin and lymph node, respectively.

• The Journal of the Korean bone and joint tumor society
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• v.19 no.2
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• pp.78-82
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• 2013

Langerhans cell histiocytosis is known as one of the diseases related to excessive proliferation of normal monocytes and has the variety of clinical courses and treatment. Especially, in cases with the spine, it shows a feature of single or multiple osteolysis. According to the location, disease progression and concomitant symptom, variety of treatments (observation, radiotherapy, chemotherapy, surgery, etc.) have been attempted, however, appropriate treatment has not been established yet. The authors introduce the case of single system Langerhans cell histiocytosis which involves cervical and lumbar vertebrae simultaneously with bone marrow destruction and pathologic fracture.

• Journal of Yeungnam Medical Science
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• v.22 no.2
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• pp.259-265
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• 2005

Central diabetes insipidus (DI) is a syndrome characterized by thirst, polydipsia and polyuria. Langerhans cell histiocytosis is one of the etiologies of DI. Recently we experienced a central DI associated with Langerhans cell histiocytosis. The 44 years old female patient complained right hip pain, polydipsia and polyuria. We carried out water deprivation test. After vasopressin injection, urine osmotic pressure was increased from 109 mOsmol/kg to 327 mOsmol/kg (300%). Brain MRI showed a thickened pituitary stalk and air bubble like lesions sized with 5cm, 7cm was shown on fifth L-spine and right hip bone at hip bone CT. CT guided biopsy revealed abnormal histiocytes proliferation and abundant lymphocytes. The final diagnosis was central DI associated with systemic Langerhans cell histiocytosis invading hip bone, L-spine and pituitary stalk. Desmopressin and etoposide chemotherapy were performed to the patient.

• Investigative Magnetic Resonance Imaging
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• v.24 no.1
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• pp.61-65
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• 2020

Langerhans cell histiocytosis (LCH) is generally considered a childhood disease that exhibits various nonspecific clinical and radiological manifestations that mimic infection or malignancy. Here, we present a case of LCH involving the rib in an adult patient. CT and MRI revealed an expansile lytic lesion with periosteal reaction on the left 8th rib, suggesting a malignant bone tumor. Surgical resection was performed and histopathological examination was consistent with LCH. Owing to its rare occurrence in adults and nonspecific aggressive features, LCH should be included in the differential diagnosis of an aggressive-appearing rib lesion in both adults and children.

• Journal of Korean Neurosurgical Society
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• v.47 no.6
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• pp.458-460
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• 2010

Langerhans cell histiocytosis (LCH), a disorder of the phagocytic system, is a rare condition. Moreover, spinal involvement causing myelopathy is even rare and unusual. Here, we report a case of atypical LCH causing myelopathy, which was subsequently treated by corpectemy and fusion. A 5-year-old boy presented with 3 weeks of severe neck pain and limited neck movement accompanying right arm motor weakness. CT scans revealed destruction of C7 body and magnetic resonance imaging showed a tumoral process at C7 with cord compression. Interbody fusion using cervical mesh packed by autologus iliac bone was performed. Pathological examination confirmed the diagnosis of LCH. After the surgery, the boy recovered from radiating pain and motor weakness of right arm. Despite the rarity of the LCH in the cervical spine, it is necessary to maintain our awareness of this condition. When neurologic deficits are present, operative treatment should be considered.