Treatment of localized langerhans' cell histiocytosis of the mandible with intralesional steroid injection: report of a case (original) (raw)
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Langerhans cell histiocytosis of the mandible in a six-year-old child
Indian Journal of Dermatology, Venereology and Leprology, 2007
Histiocytosis is a term applied to a group of rare disorders of the reticuloendothelial system. Eosinophilic granuloma, the most benign and localized of the three Langerhans cell histiocytosis entities, may be solitary or multiple. Eosinophilic granuloma can affect almost any bone, but commonly involves the mandible when the jaws are affected. 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. We report a six and a half-year-old patient with Langerhans cell histiocytosis-solitary eosinophilic granuloma of the mandible that initially regressed but rapidly recurred even after radical treatment and had a fatal outcome.
Eosinophilic granuloma is the localized and most benign form of Langerhans' cell histiocytosis. The disease shows a particular predilection for the head and neck region and usually involves the skull bones, where it manifests as well-defined lytic lesions on standard radiographs. The case of an extensive lesion involving the body of the mandible in a 52-year-old man is reported. Operative procedures consisted of enucleation of the lytic lesion and follow-up with clinical examinations and computerized tomographic studies of the mandible at 2, 12, and 18 months postoperatively. Reconstructive surgery without radiotherapy was performed with an autologous bone graft from the iliac crest and implant placement to provide support for a dental restoration. INT J ORAL MAXILLOFAC IMPLANTS 2006;21: 124–130
Otorhinolaryngology clinics : an international journal, 2013
Introduction: Langerhans cell histiocytosis (LCH) is a relatively rare and unique benign disease characterized by an abnormal proliferation of immature dendritic cells which usually affects children and young adults. Jaws are involved in less than 10% of children with the disease. Objective: To add on to the literature one more case of this rare and unique disease which accounts for less than 1% of all bone tumors and also to discuss its oral manifestation and management.
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.