1/33. 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 = 1keywords = histiocytosis (Clic here for more details about this article) |
2/33. Intraosseous xanthoma without lipid disorders. Case-report and literature review.A case of intraosseous xanthoma in a patient with a normal lipid profile is reported. Hyperlipidemia is present in most patients with xanthomas. Intraosseous xanthomas are rare, particularly in normolipidemic patients, in whom the presenting symptom is pain without skin lesions. A lytic lesion with a rim of sclerosis is seen on radiographs. histology shows foam cells, giant cells, and fibrosis. Intraosseous xanthoma is a benign tumor, and other diagnoses must be ruled out (histiocytosis X, Erdheim Chester disease, clear cell carcinoma metastasis). Surgical excision of the lesion is the elective treatment.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
3/33. Erdheim-Chester syndrome, presenting as hypogonadotropic hypogonadism and diabetes insipidus.Erdheim-Chester syndrome is a rare multisystem disease in which progressive xanthogranulomatous infiltration of several tissues are seen. knee and leg pain are the most common symptoms and bilateral symmetric sclerosis of metaphyseal region of long bones of the lower extremity is typical. Histologically, it resembles Langerhans cell histiocytosis (LCH). However, it is still a matter of discussion whether Erdheim-Chester syndrome is a distinct entity or a type of LCH. The present case is a 46-yr-old man, that presented with signs and symptoms of diabetes insipidus and hypogonadotropic hypogonadism simultaneously. x-rays and bone scintigraphy showed typical and pathogonomic findings of Erdheim-Chester syndrome. Bone biopsy and immunohistochemical staining strongly support the diagnosis of non-Langerhans cell histiocytosis.- - - - - - - - - - ranking = 2keywords = histiocytosis (Clic here for more details about this article) |
4/33. Papular xanthoma associated with angiokeratoma of Fordyce: considerations on the nosography of this rare non-Langerhans cell histiocytoxanthomatosis.BACKGROUND: Papular xanthoma (PX) is a rare normolipidemic non-Langerhans cell histiocytoxanthomatosis affecting both children and adults. OBJECTIVE: We describe an adult case of PX associated with angiokeratoma of Fordyce and review the literature in order to compare and discuss previous reports. methods: We studied the clinical, histopathological, immunocytochemical and ultrastructural findings. RESULTS: We report the findings of our case and compare our case with those described in the literature. CONCLUSIONS: Three clinical patterns of PX appeared to emerge in the review of the literature: a self-healing form, a persistent form and a progressive form. The progressive form of PX can be considered the same clinical entity that is also described as progressive nodular histiocytosis.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
5/33. Generalized papular xanthomatosis in mycosis fungoides.Xanthomas can occur in association with underlying lymphoproliferative disease, or they can result from lipid deposition in damaged or altered skin. We report a case of generalized papular xanthomas that developed in a patient with sezary syndrome. The xanthomas were composed of foamy histiocytes that were shown by immunoperoxidase staining to be of the monocyte/macrophage lineage. Electron microscopic studies revealed lipid vacuoles, lysosomes, and myelin figures but no Birbeck granules, features that are consistent with a non-X histiocytosis. Generalized papular xanthomatosis has not been previously described in a patient with cutaneous T-cell lymphoma.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
6/33. Treatment of xanthoma disseminatum with cyclophosphamide.Xanthoma disseminatum is a rare non-Langerhans cell (class II) histiocytosis, which is often resistant to treatment. We describe an illustrative case with extensive mucocutaneous, ocular, laryngeal, pituitary and central nervous system involvement, which responded to treatment with cyclophosphamide. The presentation, course and treatment of the condition are reviewed. Many of the non-Langerhans cell histiocytoses represent a spectrum of diseases of dermal dendrocytes ranging from self-limiting and benign conditions to multisystem progressive diseases that respond poorly to treatment and severely impair quality of life. We suggest that chemotherapy should be considered at an early stage in the more aggressive subtypes of non-Langerhans cell histiocytoses.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
7/33. Xanthoma disseminatum with large plaques confined to the back, pulmonary involvement and multiple intestinal xanthomas.Xanthoma disseminatum (XD) is a rare benign mucocutaneous xanthomatosis that is classified as a benign non-Langerhans cell histiocytosis. We report a 68-year-old man who presented with peculiar, large plaques confined to the back 7 years after the onset of cranial diabetes insipidus. Histopathological features of the cutaneous lesions were typical of XD. The patient had lower respiratory tract involvement with histiocytic infiltrates, which was unresponsive to various treatments and resulted in a fatal outcome. Gastrointestinal endoscopies revealed multiple xanthomas in the sigmoid colon and the rectum. To our knowledge, this is the first reported case of intestinal xanthomas associated with XD.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
8/33. erdheim-chester disease: MR imaging, anatomic, and histopathologic correlation of orbital involvement.erdheim-chester disease (ECD) is a rare form of histiocytosis of unknown origin characterized by tissue infiltration by lipid-laden histiocytes. Typically, the diaphyseal and metaphyseal portions of the tubular bones are affected, leading to a characteristic radiographic pattern of bone sclerosis. Orbital involvement is not infrequent and is manifested by exophthalmos and periorbital xanthomatous lesions, with associated visual problems. This case report documents imaging and pathologic findings in a patient with ECD with extensive orbital involvement.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
9/33. necrobiotic xanthogranuloma associated with lymphoplasmacytic lymphoma. Palliative treatment with carbon dioxide laser.necrobiotic xanthogranuloma (NXG) with paraproteinemia is a rare non-x histiocytosis with conspicuous lesions mainly located on the periorbital skin. A 68-year-old woman, with a previous history of lymphoplasmacytic lymphoma associated with IgG monoclonal gammopathy over a period of almost 4 years, presented typical lesions of NXG on the periorbital regions and left buttock. Treatment with dioxide carbon laser resulted in great improvement of cutaneous lesions, and no evidence of relapse after a 12-month follow-up. The association of NXG with lymphoplasmacytic lymphoma has not been previously described to our knowledge. The treatment of these lesions represents a true challenge for the clinician and palliative treatment of cutaneous lesions of NXG with CO(2) laser may constitute an alternative treatment in selected cases.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
10/33. A case report of langerhans histiocytosis presenting sequentially over a 21-year period with Letterer-Siwe disease, hand-Schuller-Christian disease and eosinophillic granuloma of bone.Langerhans cell histiocytosis (LCH) is a very rare disorder, and usually considered a disease of childhood. The adult form of LCH is even rare, and in some case may be considered to represent delayed presentation of a pathologic process beginning in childhood. This case report highlights the range of presentations of LCH possible even in a single patient, and supports the contention that some adult LCH cases may be due to delayed presentation of childhood LCH.- - - - - - - - - - ranking = 5keywords = histiocytosis (Clic here for more details about this article) |
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