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Search Results: 1 - 10 of 173 matches for " Histiocytosis "
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Langerhans cell histiocytosis of skin: A clinicopathologic analysis of five cases
Punia Rajpal,Bagai Maneesh,Mohan Harsh,Thami G
Indian Journal of Dermatology, Venereology and Leprology , 2006,
Abstract: Background and Aims: Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of histiocytes characterized by a proliferation of abnormal and clonal Langerhans cells. We retrospectively studied clinicopathologic features of this disorder in five cases. Methods: Clinical and histopathological findings of five cases of cutaneous LCH were reviewed based on the hospital records. Results: The age of patients ranged from 28 days to 5 years and M: F ratio was 1:1.5. Clinically, the diagnoses suggested were histiocytosis, varicella, transient neonatal pustular melanosis, keloid, sarcoidosis, seborrheic keratosis and LCH. The most common type of skin lesion was a generalized papular lesion. Histologically, all cases showed aggregates of large mononuclear histiocytes (Langerhans cells) with reniform, irregular, cleaved nuclei and abundant eosinophilic cytoplasm. There was multi-systemic involvement in two patients and single-system involvement in three patients. Conclusion: Cutaneous lesions may be the sole presenting feature of LCH. Diagnosis is based on demonstration of S-100 positive histiocytes.
Thymic langerhans cell histiocytosis in children: A case report  [PDF]
Marie Poncelet, Camille Chabert, Jean Pierre Pracros, Perrine Marec Berard
Open Journal of Pediatrics (OJPed) , 2012, DOI: 10.4236/ojped.2012.24052
Abstract: Langerhans cell histiocytosis has long been described as a rare systemic disorder involving the proliferation of Langerhans cells with formation of granuloma. The disease may be localised or diffuse. Typical forms of the disease involving the bone, the skin or the pituitary gland have been well described whereas others, such as thymic histiocytosis, are still poorly understood. Here, we report a case of isolated Langerhans cell histiocytosis of the thymus in an infant with non-specific symptoms. We provide a description of the disease and discuss patient management.
Langerhans cell histiocytosis: A case report of a three year old boy with primary oral manifestations
Seraj B,Ramyar A,Ahmadi R,Ghadimi S
Journal of Dental Medicine , 2006,
Abstract: Langerhans cell histiocytosis (LCH) is a rare disease with formation of granulomatous infiltrations consisting of Langerhans cells, histiocytes, lymphocytes and eosinophilic granulocytes. The ethiopathogenesis of the disease has not been fully clarified yet. It can occur as focal or disseminated form - acute or chronic. Oral manifestations may be the first signs. This article reports a case of a 3 year old boy with LCH suffering from severe dental mobility and foul breathe. Radiographically, the lesions appeared as well-defined radiolucent defects in maxilla and mandible. The microscopic findings consisted of sheet like arrangements of histiocytes with a mixture of eosinophils and other inflammatory cells. Biochemical tests were within normal limits.
Fever of unknown origin as the first manifestation of Erdheim-Chester disease  [PDF]
Ning Li, Mingquan Chen, Huaping Sun, Yun Bao, Jiming Zhang
Case Reports in Clinical Medicine (CRCM) , 2013, DOI: 10.4236/crcm.2013.26095
Abstract: Erdheim-Chester disease (ECD) is an uncommon non-Langerhans cell histiocytosis that affects multiple body systems and can present clinically in a myriad of ways. We report the case of a 29-year-old man presented with fever and diffuse bone pain. Physical examination showed bilateral and symmetrical long bone pain, especially the knees. Radiographs showed multiple mixed bone lesions with sclerotic and lytic areas of the femora, tibiae, and fibula. Biopsy of the tibial area displayed foamy lipid-laden histiocytes, confirming the diagnosis of ECD.
Adult Langerhans Cell Histiocytosis: A Rare Etiology of Spinal Cord Compression  [PDF]
Fadila Kouhen, Naoual Benhmiddou, Mohammed Afif, Fadoua Rais, Youssef Mahdi, Basma Khannoussi, Khadija Bellahamou, Ibrahim Ghissassi, Khouloud Boussouni, Hanan Elkacemi, Sanaa Elmajjaoui, Tayeb Kebdani, Noureddine Benjaafar
Open Journal of Internal Medicine (OJIM) , 2015, DOI: 10.4236/ojim.2015.53005
Abstract: Langerhans cell histiocytosis is a rare disease involving clonal proliferation of langerhans cells seen in children and young adults. Clinical presentation is variable, ranging from a single location in the bone to severe multivisceral involvement. Moreover, spinal involvement causing myelopathy is even rare and unusual. We report a rare case of adult Langerhans cell histiocytosis in the dorsal spine causing a spinal cord compression associated with a pulmonary process treated by surgery, radiotherapy and systemic therapy with good evolution.
Doen?a de Rosai-Dorfman cutanea: relato de caso
Eiras, Josie da Costa;Schettini, Ant?nio Pedro Mendes;Lima, Livia Lima de;Tubilla, Liana Hortência Miranda;Oliveira, Rosa Maria Libório de;
Anais Brasileiros de Dermatologia , 2010, DOI: 10.1590/S0365-05962010000500014
Abstract: rosai-dorfman disease, otherwise known as sinus histiocytosis with massive lymphadenopathy, is a non-langerhans cell histiocytosis with a benign course and unknown etiology. it was described in 1969 as a painless cervical lymph node enlargement in association with fever, weight loss and sweating. extranodal disease has been reported in 43% of cases, with involvement of multiple organs. purely extranodal rosai-dorfman disease has been already reported, including forms restricted to the skin. this paper reports a case of purely cutaneous rosai-dorfman disease, which is of interest in view of the rarity of this condition.
Histiocitose de células de Langerhans em margem anal: relato de caso e revis?o da literatura
Magno, Jaime Coelho Carlos;D'Almeida, Dianice Gallo;Magalh?es, José Pinheiro;Pires, Vilmar José;Araújo, M?nica Leite de;Miranda, Cláudia Barreto de;Nagel, Janaina;
Revista Brasileira de Coloproctologia , 2007, DOI: 10.1590/S0101-98802007000100012
Abstract: a rare case of langerhans cell histiocytosis with perianal involvement in a 34 year old white man is presented. during nineteen months this patient had a complaint of anal pain with bleeding, due to a large perianal ulcer. the initial diagnosis was crohn's disease, anal tuberculosis, syphilis, pyoderma gangrenosum or donovanosis. after the third biopsy, the surgical specimens showed microscopic changes suggestives of langerhans cell histiocytosis. the imunohistochemical study was positive to s100 protein and cd1a. the patient was treated with six doses of intralesional triancinolona and oral thalidomide for three months. treatment was well tolerated and complete resolutions of peri-anal ulcer occurred.
Histiocitose de células de Langerhans com acometimento vulvar e com resposta terapêutica à talidomida: relato de caso
Fernandes, Lana Bezerra;Guerra, Jackeline Gomes;Costa, Maurício Barcelos;Paiva, Isadora Guimar?es;Duran, Fernanda Paglia;Jacó, Diogo Neves;
Anais Brasileiros de Dermatologia , 2011, DOI: 10.1590/S0365-05962011000700020
Abstract: langerhans cell histiocytosis is a member of a group of rare histiocytic syndromes and is characterized for the proliferation of histiocytes called langerhans'cells. its manifestations vary from a solitary injury to systemic involvement, and vulvar lesions are uncommon. we describe a refractory case of cutaneous limited disease in a 57-year-old woman. she presented with a 6-year history of an erythematous papular eruption of the scalp, face, vulva, trunk and axillae. the diagnosis is difficult and in this case it was confirmed through immunohistochemical study and clinical improvement was achieved with thalidomide
Histiocitosis de células de Langerhans con afectación tímica como manifestación única de la enfermedad
Hernández Pérez,J. M.; Franquet Casas,T.; Rodríguez,S.; Giménez,A.;
Anales de Medicina Interna , 2007, DOI: 10.4321/S0212-71992007001000008
Abstract: the langerhans′cell histiocytosis (lch), also known as histiocitosis x it is an illness not very frequent granulomatosus etiology not clarified yet, that it can have different manifestations and localizations, however the thymic localization as initial and exclusive place gives presentation hcl it is quite unusual. the present case is presented a patient that debuted with a clinical unspecific, where the tests give image they put she gives apparent a mass in previous mediastinum and that after the pathologic and immunohistochemical analysis they evidenced a proliferation langerhans's cells and eosinophils it being positive for cd1a and s-100 confirming the diagnosis of the lch.
COMPROMISO OSEO EN HISTIOCITOSIS DE CELULAS DE LANGERHANS EN EL NI?O: ESTUDIO RADIOLOGICO SIMPLE. PRESENTACION CLINICA Y DIAGNOSTICO RADIOLOGICO
Rojas C,Raúl; García B,Cristián; Parra R,Dimitri; Solar G,Antonieta; Oyanedel Q,Roberto; Díaz B,Fernán; Fortune H,Juan; Etchart K,Martín;
Revista chilena de radiología , 2005, DOI: 10.4067/S0717-93082005000300005
Abstract: introduction. langerhans' cell histiocytosis is an uncommon disease, with various radiological presentations. its cause remains unknown, with the pathological hallmark being proliferation of langerhans' cells. the spectrum of disease ranges from solitary bone lesions to multisystemic affection. usually, the first diagnostic test performed is the plain radiograph, and the radiological findings may be hard to analyze. differential diagnosis should include osteomyelitis, ewing's sarcoma and lymphoma. objectives: to show the plain radiographic findings of lch, that allow the radiologist to suspect it. methods and materials: the clinical and radiological presentations of the biopsy proven lch cases of the national bone tumor file (renato) between the years 1961 and 1999 were analyzed retrospectively. results: from a total of 59 patients, 58% were boys and 42% were girls. age ranged from 0.8 to 17 years, with an average of 6.1 years. medical history was obtained in 56 cases (95%). the mean duration of symptoms at diagnosis was 5,5 months, with pain at the site of affection being the most frequent 50/59 of them. forty-eigth patients (81%) presented with a solitary bone lesion and 11 (19%) presented with two or more lesions; 35 patients had afection of long bones and 26 of axial skeleton. the most frequently radiological pattern was a purely osteolytic lesion (n=45). 13 patients presented with a permeative lesion and a vertebra plana was found in 3. periostal reaction and soft tissue mass were found in 30 and 29 cases respectively. conclusions: despite the fact that lch has several radiological manifestations, its diagnosis should be suspected by the radiologist. this study with biopsy proven cases of langerhans′ cell histiocytosis allows us to characterize the manifestations of this disease in the skeleton and is useful for the differential diagnosis of a bone lesion in the pediatric patient
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