Treatment of localized langerhans' cell histiocytosis of the mandible with intralesional steroid injection: report of a case (original) (raw)
A pathologic mandibular fracture revealing a bifocal location of Langerhans cell histiocytosis
Annals of Medicine and Surgery, 2020
Introduction: Langerhans cell histiocytosis is a rare disease. When it occurs in the cranium/facial bones, the mandibular location is the most frequent. Presentation of case: A 31 years-old man was referred to our department for a mandibular chronic discomfort during an acute exacerbation, spontaneous teeth mobility and an alteration of the dental occlusion revealing a pathologic mandibular fracture. The diagnosis was confirmed by a subsequent CT scan. The surgical procedure was performed under general anesthesia by a maxillo-facial senior surgeon. The therapeutic plan combined teeth extractions, enucleation of both the left maxillary and right mandibular cystic lesions and osteosynthesis of the pathologic mandibular fracture with a miniplate. Histological and immunohistochemistry analysis of the maxillary and mandibular cystic lesions pointed the diagnosis of a bifocal Langerhans cell histiocytosis of the oral cavity. Several investigations were done in order to find another location, showing no abnormalities. Discussion: This is a case of rare single system LCH at two distinct locations: one at the mandibular bone and the other at the upper left maxilla. Both were uncovered by an acute exacerbation of a chronic discomfort secondary to a mandibular pathologic fracture. This should draw attention to the issues of the diagnosis. Conclusion: An early LCH diagnosis and a multidisciplinary treatment plan allow the improvement of the patient 's prognosis and quality of life.
Collegium antropologicum, 2013
Our aim is to provide review of available studies on Langerhans cell histiocytosis (LCH) and discuss treatment for polyostotic monosystem form of disease based on our clinical experience. LCH is an enigmatic disease with insufficiently understood etiology, pathophysiology, and variety of clinical presentations ranging from solitary eosinophilic granuloma to severe multisystem disease. It is marked by formation of granuloma in practically any organ. We present rare case of multifocal bone disease in 36-year old patient without visceral involvement. Treatment protocols for adult LCH patients, especially for uncommon form in our case have not yet been defined. Our therapeutical trial with corticosteroids showed limited success with numerous side-effects. We conclude that LCH treatment can commence only after diagnosis and staging of the disease. Other factors like patient's age, comorbidity, general condition, severity of symptoms and contraindications for therapy modalities should...
Langerhans Cell Histiocytosis of Maxilla and Mandible in 6 Years Old Child: A Case Report
International Journal of Clinical Pediatric Dentistry, 2009
Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized bone marrow-derived Langerhans cells (LCs) and mature eosinophils. Its etiology is unknown but it could be due to antigenic stimulus of an infectious, genetic abnormality, deregulated immune response, or even clonal origin. Clinical presentation may be localized and systemic, invading skin, lungs and bone in adult, and bone marrow and lymph node in children. Obtaining a biopsy that yields cells that are morphologically and immunohistochemically compatible with Langerhans cells, can make a definitive diagnosis of LCH. Poor prognosis factor include advanced age, disease extent and systemic organ abnormality. Conventional treatment of LCH is with surgery, radiotherapy, chemotherapy and steroid injections, alone or in combination. Spontaneous regression of localized disease has also been reported.
Langerhans cell histiocytosis: recurrent lesions affecting mandible in a 10-year-old patient
Journal of Clinical Pediatric Dentistry, 2001
Hand-Schuller-Christian disease is a multifocal variant of eosinophilic granuloma, characterised by the classical triad of bony lesions, exophtamos and diabetes insipidus. This case relates recurrent Langerhan's cell histiocytisis lesions presented as destruction of periodontal support associated with diabetis in a 10-year–old patient. Medical history suggests that the case represents a case of Hand- Schuller Christian disease.
Annals of Medicine and Surgery, 2019
Background: Langerhans cell histiocytosis (LCH) is a rare group of disorders without a well understood etiology. Known formerly as histiocytosis X, the disease has a wide spectrum of clinical presentations, including eosinophilic granuloma (solitary bone lesion), diabetes insipidus, and exophthalmos. Many of these patients initially present to orthopaedic surgeons, and misdiagnosis is frequent. Methods: We deliver a case of a 10-month-old boy who consulted to our department. Previously misdiagnosed as a Kawasaki syndrome, TORCH, and osteomyelitis. He had undergone several examinations and had been discussed in clinocipathological conference (CPC) to narrow down the diagnosis. Result: After serial examinations, the diagnosis of Langerhans Cell Histiocytosis was confirmed and chemotherapy was initiated. And after 6 cycles of chemotherapy, with 1-week interval of each therapy, the clinical appearance of this patient significantly improved. Conclusion: Despite major advances in our understanding and management of LCH, it remains one of the most challenging diagnoses for the orthopedic surgeon. By doing a comprehensive examination, it is possible to narrowing down the diagnosis and planning the accurate treatment.
Langerhans Cell Histiocytosis with Temporal Bone Involvement- A Case Report
INTERNATIONAL JOURNAL OF ANATOMY RADIOLOGY AND SURGERY, 2021
Langerhans Cell Histiocytosis (LCH) is a rare disorder of the reticuloendothelial system associated with proliferation of Langerhans cells and mature eosinophils. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhan’s cell. Any organ or system can be affected. Here, a case of multisystem LCH with skeletal, lung and hepatobiliary involvement in a two-year child who presented with painless forehead swelling following trauma. The patient was systematically worked up with blood investigations, imaging and histopathological analysis which ultimately revealed the diagnosis of LCH. This case report is unique in that it presented with involvement of temporal bone wherein it can be confused with inflammatory pathologies like Cholesteatoma and tumours like Rhabdomyosarcoma. The child was started on vinblastine based chemotherapy and showed good response to therapy. This case report discusses the imaging differential diagnosis in temporal bone LCH a...
2020
Most patients are children or adolescents but the disease can affect any age group, including the elderly [4]. It is classified into three clinical forms depending on the age and presentation: 1) Chronic focal Langerhans Cell Histiocytosis or Eosinophilic Granuloma this is the most frequent and benign of the three clinical forms which appears as a unifocal or multifocal lesion in single or multiple bones with or without soft tissue involvement presenting at any age. 2) Chronic diffuse LCH (Hand Schuller Christian Disease) which usually appears in children or young adults with the characteristic triad of Exophthalmos, Osteolytic lesions of cranium and Diabetes insipidus. Abstract Eosinophilic Granuloma represents one of a triad, of lesions encompassing a disease under the generic name Histiocytosis X or Langerhans Cell Histiocytosis. It is a benign inflammatory reaction to an unknown etiological agent and corresponds with bony lesions. The most commonly involved bones are the skull, ...
Langerhans cell histiocytosis (histiocytosis x) – in the mandible
Brazilian dental science, 2013
This article reports a case of 65 year-old man consulted in a private radiology dental clinic for a panoramic radiography, where was indicated a radiolucent area, extending into the periapical region of the teeth 35 to 43 to the base of the mandible. Radiography diagnosis was difficult. The immunohistochemical examination showed immunoreactivity for the proteins CD 68, S-100 and CD 1a. Histopathological diagnosis was obtained for Langerhans cell granuloma (histiocytosis X). Langerhans cell histiocytosis is caused by the abnormal proliferation of histiocytes, previously called Histiocytosis X. It is a rare disease that preferentially affects children and young adults with a predilection for males and can affect different parts of the body.
Orphanet Journal of Rare Diseases, 2021
Background: Langerhans cell histiocytosis (LCH) is a rare disease that originates from the uncontrolled proliferation and accumulation of bone marrow-derived immature myeloid dendritic cells. Dendritic cells are a type of histiocyte that play an important role in the human immune system and are found in the bone, skin, stomach, eyes, intestines, and lungs. Objective: This systematic review aimed to collect and report published case reports of rare bone disease caused by LCH to avoid misdiagnoses or delays in diagnosis. Methods: We systematically searched Scopus, PubMed, Embase, and Web of Sciences from August 1, 2000 to December 31, 2019. Studies reporting cases of LCH with rare bone involvement were included. Results: We identified 60 articles including 64 cases. Of the identified cases, 31 (48.4%) involved children, and 33 (51.6%) involved adults. Additionally, 46.9% (30 individuals) were from Asian countries. The mean age of the children was 7.6 ± 4.3 years and that of the adults was 36 ± 12 years. The findings indicated that unifocal bone involvements were the most prevalent form of the disease (68.7%), and, overall, the skull and chest wall were the most commonly affected bones in both adults and children. The spine and long bones were the second most commonly affected bones in children, and the spine and jaw were the second most commonly affected bones in adults. Pain and swelling were the most frequent presenting signs among the investigated cases, and loss of consciousness, myelopathy, nerve palsy, visual loss, torticollis and clicking sounds were rare signs. Osteolytic lesions were the most frequent radiologic feature (62.5%), and intracranial hemorrhage, fluid-fluid level, dura and intracranial extension and pathologic fractures were rare radiological features. Total excision, curettage and observation in the unifocal group of patients and systemic chemotherapy in the other groups (i.e., multifocal and multisystem) were the most frequent management approaches. The recovery rates of the unifocal and multifocal groups were 77.3% and 81.8%, respectively, while that of the multisystem group was 55.5%. The rates of recurrence and mortality in the multisystem group were 11% and were higher than those in the other groups. Conclusions: LCH is a rare disease that can affect any organ in the human body. However, bone is the most commonly involved organ, and rare bone involvements may be the first or only symptom of the disease due to the rarity of such lesions; a lack of familiarity with them may result in misdiagnosis or delayed diagnosis.