Search results for "Langerhans cell histiocytosi"
showing 6 items of 16 documents
Successful treatment of adult multisystemic Langerhans cell histiocytosis with psoralen-UV-A, prednisolone, mercaptopurine, and vinblastine.
2008
Background Langerhans cell histiocytosis (LCH) is a rare disease with a peak incidence in childhood. There is limited experience with treatment options for adult patients having multisystemic LCH involvement. We report successful treatment of a 70-year-old woman with adult onset of LCH and multisystem disease (diabetes insipidus centralis, bone marrow infiltration, and lung and skin involvement). Observations A 70-year-old woman with erythematous plaques and papules of the submammary and inguinal skin attended our outpatient clinic and was diagnosed as having LCH. Organ involvement was found in the infundibulum of the pituitary gland, associated with diabetes insipidus centralis, bone marro…
An unusual Erdheim-Chester disease with orbital involvement: a case report
2017
Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis with multiorgan involvement and a specific tropism for perivascular and fatty connective tissue, of unclear origin, with poor response to therapy. Its identification is difficult because of the variable clinical presentation and its lack of knowledge. We report the case of a 63-years-old woman, with a history of bilateral orbital pseudotumor, who comes to our attention because of progressively worsening asthenia, vomiting and systemic inflammation. Total body computerized tomography scan showed a volumetric increase of choroid plexus of the temporal horn of the left lateral ventricle, presence of solid retrobulbar tissue at…
Inflammatory pituitary lesions
2021
Abstract Inflammatory pituitary lesions are rare but important differential diagnoses of nonfunctioning pituitary masses. Primary hypophysitis as an autoimmune disorder has received increasing attention over the past decades. In the last decade, immune checkpoint inhibitor–induced hypophysitis has emerged as a new type of secondary hypophysitis. Various pituitary tumors may also cause secondary hypophysitis. Systemic, noninfectious granulomatous diseases that can involve the pituitary region include sarcoidosis, granulomatosis with polyangiitis, Langerhans cell histiocytosis, Erdheim-Chester disease, and Rosai-Dorfman disease. Furthermore, infectious diseases can affect the pituitary gland.…
Bifocal manifestation of eosinophilic granuloma in a paediatric patient-A case report
2009
Background: Eosinophilic granuloma (EG) is a clinical variant of the Langerhans cell histiocytosis (LCH) characterized by unifocal or multifocal bone lesions which predominantly affects children, adolescents, and young adults. Case Report: A case is reported of a 13-year-old Caucasian boy who presented unifocal EG in the mandible as the first clinic manifestation. Radiographic examination and skeletal scintigraphy revealed a further localization with an osteolytic lesion in the right femur. The therapeutic protocol used for the mandibular lesion included causal periodontal therapy, extraction of the compromised teeth, alveolar curettage, and intralesional injections of corticosteroids, in c…
A rare case of oral multisystem Langerhans cell histiocytosis
2017
Langerhans cell histiocytosis (LCH) is a rare disorder characterized by high proliferation of Langerhans dendritic cells. LCH is a solitary or multifocal disease that primarily involves bone tissue and often affects children and young men. A 29 years-old Caucasian man was referred to the Oral Surgery Unit of George Eastman Hospital - Umberto I teaching hospital, with third degree mobility of teeth belonging to second, third and fourth quadrant. Panoramic radiograph showed multiple radiolucent areas with well demarcated borders on the right and left site of the mandible and on the left site of the maxilla. Extractions of compromised teeth and biopsy of the osteolytic tissue were performed. T…
Langerhans’s cell histiocytosis in old subjects: two rare case reports and review of the literature
2012
doi: 10.1111/j.1741-2358.2012.00629.x Langerhans’s cell histiocytosis in old subjects: two rare case reports and review of the literature Background: Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells that generally affects children; LCH onset is rare in adults; immunohistochemistry is essential to obtain the correct diagnosis, and treatment protocols are controversial. Objective: To describe two new cases of adult onset oral LCH. Case reports: Case 1: a 71-year-old woman, complaining of diffuse oral pain, presented with erythematous mucosal lesions; the panoramic radiograph and CT scan showed multiple mandible radiolucent areas. Immunohistochemical a…