1/65. Xanthoma disseminatum: a case with hepatic involvement, diabetes insipidus and type IIb hyperlipidaemia.Xanthoma disseminatum (XD) is a rare benign non-X-histiocytic disorder of unknown aetiology. We report a 37-year-old man who presented with XD preceded by a decade of cranial diabetes insipidus, with associated type IIb hyperlipidaemia and computed tomographic evidence of hepatic involvement. A review of the literature is also included.- - - - - - - - - - ranking = 1keywords = disseminatum (Clic here for more details about this article) |
2/65. Langerhans cell histiocytosis of the vulva: an ultrastructural study.langerhans cells histiocytosis (LCH) is a proliferative disorder of langerhans cells. The lesions are normally characterized by infiltration of eosinophils, neutrophils, lymphocytes, plasma cells, and langerhans cells. The specific cells of LCH contain Birbeck granules, express the phenotype of langerhans cells but with markers fixed at an early stage of activation, and are functionally defective in antigen-presenting ability. The disease most often affects children; when it occurs in older patients, anal and groin involvement is quite common and vulvar lesions can be found in older females. The authors report a case of a 64-year-old woman with LCH of the vulva and diabetes insipidus. An immunohistochemical and ultrastructural study of the vulvar lesions showed an infiltrate in which antigenically and morphologically mature langerhans cells, monocytoid cells, and cells with an intermediate phenotype between monocytes and langerhans cells were concurrently observed. Although the clinical and histological aspects of LCH are well established, the pathogenetic mechanism of lesions is not yet known. The finding of an infiltrate composed by langerhans cells and many putative precursors of these cells suggests the hypothesis of an in situ differentiation of langerhans cells from immature monocytoid precursors.- - - - - - - - - - ranking = 0.063385136579229keywords = histiocytosis (Clic here for more details about this article) |
3/65. diabetes insipidus in Langerhans' cell histiocytosis: report of a case.The incidence of diabetes insipidus secondary to Langerhans' cell histiocytosis (LCH) varies among different reports, ranging from 9.5 to 50%, but it has never been reported in literature in taiwan. Therefore, we presented a case suffering from polyuria, polydipsia, body weight loss for more than one year and seborreic dermatitis-like skin lesions over the scalp and trunk for more than two years. Her body weight and body length were both less than 3 percentile. Fluid restriction and vasopressin test were performed to differentiate nephrogenic from neurogenic diabetes insipidus. skin biopsy revealed picture of LCH and LCH with complete central diabetes insipidus was diagnosed. brain MRI and other laboratory examinations were all within normal limits. She received nasal DDAVP treatment and chemotherapy with TPOG-H 94 protocol. After 3 months treatment, her skin lesions disappeared and daily urine amount returned to normal range.- - - - - - - - - - ranking = 0.063385136579229keywords = histiocytosis (Clic here for more details about this article) |
4/65. A patient with diabetes insipidus and periorbital swellings; erdheim-chester disease.erdheim-chester disease is a rare multisystem disease in which a progressive xanthogranulomatous infiltration of several tissues can be seen. We describe a woman, known to have diabetes insipidus for ten years, with periorbital, retroperitoneal, mediastinal, axillar and inguinal involvement. On histological examination a granulomatous infiltration of fatty tissue and striated muscle was seen, consisting of Touton giant cells, histiocytes with foamy cytoplasm and lymphocytes. Immunohistochemical staining with CD-1a and S-100 was negative and on electron microscopy no Langerhans granules were seen. These findings led to the diagnosis of erdheim-chester disease. She had a good response on steroids. Because of some similar clinical features of Langerhans cell histiocytosis and erdheim-chester disease, a histiocyte disorder seems the most probable cause.- - - - - - - - - - ranking = 0.012677027315846keywords = histiocytosis (Clic here for more details about this article) |
5/65. Langerhans' cell histiocytosis: report of an atypical case.A patient is described with Langerhans' cell histiocytosis and polyneuropathy diagnosed 12 years after the development of diabetes insipidus after head trauma.- - - - - - - - - - ranking = 0.063385136579229keywords = histiocytosis (Clic here for more details about this article) |
6/65. Pulmonary involvement by histiocytosis X in the pediatric age group.The lungs may be involved by histiocytosis X either as part of a generalized disease or as a separate entity, primary pulmonary histiocytosis X. The former is much more frequent in the pediatric age group, and although it occurs primarily in those who are quite sick with widely disseminated disease, the lung involvement rarely caused symptoms and almost invariably clears. By contrast, primary pulmonary histiocytosis X is mainly seen in young adult males and frequently leads to severe disability. Twelve patients less than 20 years old with lung involvement by histiocytosis X are presented.- - - - - - - - - - ranking = 0.10141621852677keywords = histiocytosis (Clic here for more details about this article) |
7/65. histiocytosis X and compulsive water drinking: report of a case.We describe a patient with histiocytosis X and compulsive water drinking. The association of diabetes insipidus with histiocytosis X is well recognized, and this patient was initially considered to have diabetes insipidus. It was only after further testing that the proper diagnosis was made.- - - - - - - - - - ranking = 0.025354054631692keywords = histiocytosis (Clic here for more details about this article) |
8/65. Benign cephalic histiocytosis with diabetes insipidus.Benign cephalic histiocytosis is a rare skin condition consisting of small tan papules on the face and upper trunk that is believed not to be associated with internal organ involvement. The infiltrating histiocytes are not Langerhans' cells (LCs). We report a 5-year-old girl who presented with diabetes insipidus 1 year after developing multiple small brown asymptomatic skin papules. Histologic examination revealed a non-LC histiocytic proliferation in the dermis without epidermal invasion. She had infiltration of the pituitary stalk on brain imaging. diabetes insipidus has heretofore been associated with LC histiocytosis and xanthoma disseminatum but not benign cephalic histiocytosis.- - - - - - - - - - ranking = 1.1231067285132keywords = xanthoma disseminatum, disseminatum, histiocytosis, xanthoma (Clic here for more details about this article) |
9/65. Langerhans cell histiocytosis involving the thyroid and parathyroid glands.Langerhans cell histiocytosis (LCH) is a rare illness, and the disease afflicting the thyroid gland is very uncommon, even in the presence of multisystem involvement. In this report, we document histologically, for the first time, concurrent involvement of the thyroid and parathyroid glands by LCH. A young Chinese woman with a history of diabetes insipidus and hypogonadism underwent a total thyroidectomy for enlarged thyroid gland secondary to LCH causing airway obstruction. Microscopic examination of the excised specimen disclosed CD1a- and S-100-positive LCH cells involving the thyroid and parathyroid glands. In a patient with LCH affecting the thyroid gland, parathyroid gland disease should be suspected when the serum calcium levels are depressed in association with an inappropriate serum parathyroid hormone level, such as a normal parathyroid hormone level in this case.- - - - - - - - - - ranking = 0.063385136579229keywords = histiocytosis (Clic here for more details about this article) |
10/65. Central diabetes insipidus as the first manifestation of neurosarcoidosis in a 10-year-old girl.Central diabetes insipidus with thickening of the pituitary stalk has been demonstrated in adults with Langerhans cell histiocytosis (LCH), tuberculosis or sarcoidosis, and in children with LCH. We present a 10.5-year-old girl with central diabetes insipidus. magnetic resonance imaging (MRI) revealed thickening of the central part of the pituitary stalk and multiple hyperintense lesions in the frontal white matter on T(2)-weighted images. Laboratory findings were normal except for an elevated serum angiotensin-converting enzyme (ACE) level. The MRI findings together with the elevated serum ACE level highly suggest the diagnosis of sarcoidosis. We conclude that central diabetes insipidus can be the first clinical manifestation of sarcoidosis in children.- - - - - - - - - - ranking = 0.012677027315846keywords = histiocytosis (Clic here for more details about this article) |
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