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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Histiocytosis X encompasses three syndromes characterized by the idiopathic proliferation of histiocytes: eosinophilic granuloma, Hand-Schuller-Christian syndrome, and Letterer-Siwe disease. At the Mayo Clinic between 1926 and 1978, 22 patients with histiocytosis X had involvement of the ear or temporal bone. These patients comprised 15% of all patients with histiocytosis X seen during that period. The ages at onset of the disease ranged from 2 months to 49 years. The most frequent otologic symptom was aural discharge (15 patients), followed in frequency by swelling in the temporal region (11 patients), vertigo (6 patients), and deafness (5 patients). Clinical findings included otitis media (13 patients), otitis externa with or without granulation tissue (10 patients), and osteolytic lesions of the temporal bone (9 patients). A high index of suspicion is required to recognize the otologic manifestations of histiocytosis X for two reasons: the systemic manifestations of the disease are often so dramatic that the ear findings are overlooked, and the otologic findings of histiocytosis X can mimic more common diseases, including simple otitis externa, aural polyps, acute mastoiditis, chronic otitis media, and metastatic lesions.
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PMID:Histiocytosis X of the ear and temporal bone: review of 22 cases. 31 4

The preoperative diagnosis of temporal bone histiocytosis X has been based traditionally on clinical examination, plain radiography, and pluridirectional tomography. Clinical misdiagnosis is common because otologic findings can mimic those of acute and chronic infectious ear disease. Similarly, plain radiographic and tomographic findings may be confused with those of mastoiditis, cholesteatoma, and temporal bone metastasis. The three cases of histiocytosis X presented here illustrate the advantages of CT compared with traditional radiographic methods in the diagnosis and staging of this disease. Computed tomography clearly delineates osseous involvement, including erosion of the bony labyrinth. Computed tomography also better defines the soft tissue margins of the granulomatous mass in relationship to the central nervous system and extratemporal tissues.
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PMID:Histiocytosis X of the temporal bone: CT findings. 325 22

Langerhan's cell histiocytosis is an uncommon granulomatous disease, characterized by the idiopathic proliferation of Langerhan's cells or their marrow precursors. It encompasses the diseases previously associated with histiocytosis X-eosinophilic granuloma, Hand-Schuller-Christian syndrome and Letterer-Siwe syndrome. A series of 54 patients were diagnosed with this condition in Dublin over a 33-year-period (1959-1992). Twenty-seven patients had aural symptoms, of whom 15 had no other lesions at the time of presentation. Otorrhoea was the most frequent otological symptom, followed by lesions in the temporal bone. LCH may mimic common aural conditions such as otitis externa, otitis media or acute mastoiditis and a high index of suspicion is required to recognize it on clinical presentation. The clinical presentation of multi-focal disease may be so dramatic that the otologic findings may be initially overlooked. The mortality rate was 14.8%. Therapeutic regimes included no treatment, curettage, chemotherapy, radiotherapy or multi-modality treatment.
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PMID:Aural symptoms as primary presentation of Langerhan's cell histiocytosis. 887 94

Langerhans cell histiocytosis implies the proliferation and accumulation of anomalous, cytologically benign tissue macrophages in a given site. In the Hospital General y Universitario of Alicante, Spain, from 1975 to 1996 four patients presented with granulomatosis and otorhinolaryngological signs and symptoms: a 7-year-old girl with a right mastoid eosinophil granuloma, a 2-month-old boy with Letterer-Siwe syndrome whose illness started as acute mastoiditis, a 23-month-old boy who developed eosinophil granulomas of both mastoids and one ischium, and an 8-month-old girl with a right zygomatico-temporal eosinophil granuloma. The treatment and clinical course of each case is described and compared with the results of other authors.
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PMID:[Langerhans cell histiocytosis. Report of four cases]. 964 66

Langerhans cell histiocytosis (LCH) is a rare disorder of unknown cause, characterized by the proliferation of histiocytic cells in various tissues and organs. The role of the otolaryngologist is important in the early and accurate evaluation, staging and diagnosis of LCH, because it may mimic more common diseases such as otitis externa and acute mastoiditis. We discuss a case report of bilateral mastoid involvement in a child with a history of otalgia unresponsive to medical therapy.
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PMID:Langerhans' cells histiocytosis. 1036 75

We report the case of a child presented by her parents to the ENT outpatient service for swelling of the right temporal bone. The child had a history of recurrent bilateral inflammation of the middle ear. Tympanometry revealed a reduced compliance. Due to conductive hearing loss it was impossible to measure otoacustic emissions. Otherwise a normal ENT status was found. Imaging (MRI/CT) demonstrated bitemporal soft-tissue changes with extensive osseous destruction, but no typical imaging signs of an inflammatory, dysplastic or expansive process. The tentative diagnosis of Langerhans' cell histiocytosis (LCH) made on the basis of the clinical and imaging findings was confirmed by biopsy. After exclusion of disseminated LCH, chemotherapy was initiated, and the child underwent follow-up imaging after 3 months. CT showed clear signs of bitemporal reossification. The case reported here illustrates the problems encountered in diagnosing LCH which may present with unspecific clinical symptoms despite advanced osseous destruction. ENT specialists should be familiar with this very heterogeneous entity and think of LCH especially in children presenting with therapy-refractory otitis media, otitis externa, or mastoiditis in order to ensure a timely diagnosis and to thus improve the chances of successful therapy. Imaging modalities (CT, MRI) have a role in the early diagnosis and follow-up of this disorder.
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PMID:[Langerhans cell histiocytosis: petrosal remodelling after chemotherapy--case report and review of the literature]. 1267 14

Langerhans' cell histiocytosis (LCH) is a rare paediatric disease of unknown etiology affecting 1 to 5 children per 1 million each year. It is characterized by the idiopathic proliferation of Langerhans' cells. The clinical spectrum of disease is quite varied, ranging from a solitary eosinophilic granuloma to diffuse multisystem involvement. The head and neck is the most common site of involvement, occurring in approximately 60% of LCH patients. Head and neck manifestations are diverse and include skull and temporal bone lesions, cervical lymphadenopathy, and skin rash. Diagnosis can be difficult as these lesions mimic other common conditions seen by the otolaryngologist, including otitis externa, acute mastoiditis, and gingivitis. A retrospective study was carried out to study our centre's experience with LCH over the last 10 years. Twenty-one patients were diagnosed between January 1990 and December 1999. Patient's age at time of diagnosis ranged from 6 days to 14 years. Fifty-seven percent of patients had localized bony lesions; the remaining 43% had diffuse multisystem disease. The head and neck was also the most commonly involved site in our study, affecting 67% of our patients. Presentation and diagnosis of these lesions are discussed in detail. Treatment, complications, and patient outcomes will also be discussed.
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PMID:Langerhans' cell histiocytosis in the paediatric population: presentation and treatment of head and neck manifestations. 1277 59

Multifocal Langerhans' cell histocytosis is a rare condition in adults. We present the case of a 31-year-old female who initially presented with a clinical appearance of acute mastoiditis. The patient ultimately had involvement of bilateral temporal bones, lungs, and hypothalamus. Treatment with methotrexate, steroids, and desmopressin acetate (DDAVP) resulted in initial clinical improvement though not resolution. The patient also underwent radiation therapy to the hypothalamic lesion. The literature is reviewed, focusing on the diagnostic challenge of this disease process in adults. The additional morbidity of surgery, specifically mastoidectomy, in the setting of Langerhan's cell histiocytosis is discussed.
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PMID:Multifocal langerhans' cell histiocytosis involving bilateral temporal bones, lungs, and hypothalamus in an adult. 1717 Oct 82

Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder with an unpredictable clinical course and highly varied clinical presentation ranging from single system to multisystem involvement. Although head and neck involvement is common in LCH, isolated bilateral temporal bone involvement is exceedingly rare. Furthermore, LCH is commonly misinterpreted as mastoiditis, otitis media and otitis externa, delaying diagnosis and appropriate therapeutic management. To improve detection and time to treatment, it is imperative to have LCH in the differential diagnosis for unusual presentations of the aforementioned infectious head and neck etiologies. Any lytic lesion of the temporal bone identified by radiology should raise suspicion for LCH. We hereby describe the radiologic findings of a case of bilateral temporal bone LCH, originally misdiagnosed as mastoiditis.
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PMID:Bilateral temporal bone langerhans cell histiocytosis: radiologic pearls. 2447 12

Langerhans cell histiocytosis (LCH) is a rare disease ranging from a benign to a rapidly fatal condition affecting young children predominantly, and is characterized by an abnormal clonal proliferation of Langerhans cells. We report a case of a 3-year-old child presenting with a 1-year history of otorrhea and otorrhagia followed by a 6-month history of postauricular swelling in the right ear. Imaging demonstrated a large mass of organized tissue. A biopsy was conducted, and the diagnosis of LCH was confirmed by histopathological and immunohistochemical examination. The child was treated with a 12-month course of vinblastine chemotherapy with prednisolone. No clinical evidence of recurrence was noticed after 3 years of follow-up. This rare case highlights the importance for otolaryngologists to keep LCH in mind for differential diagnosis in very young patients with symptoms and signs suggestive of acute mastoiditis or chronic otitis media.
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PMID:A rare disorder mimics otitis media: Langerhans cell histiocytosis of the temporal bone in a child with interstitial pulmonary fibrosis. 2513 21


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