1/402. Activation of monocytes in vivo causes intracellular accumulation of lipoprotein-derived lipids and marked hypocholesterolemia--a possible pathogenesis of necrobiotic xanthogranuloma.necrobiotic xanthogranuloma (NXG) is a rare histiocytic disease with generalized xanthomatosis. However, most cases with NXG are normolipidemic or hypolipidemic. The mechanism for the formation of xanthoma in NXG has not yet been clarified. We observed a case of NXG with severe hypocholesterolemia (total cholesterol: 1.69 mmol/l) and analyzed the function of monocytes in this case. Histological examinations by light microscopy revealed a large amount of lipid deposition in the patient's freshly isolated monocytes. The patient's monocytes showed a 3-fold increase in cholesteryl ester content and a 3-fold enhancement of acetyl low density lipoprotein (LDL) uptake compared with the control monocytes. However, no significant difference was noted in the expression of CD36 protein and the mRNA levels of scavenger receptor-class A (SR-A) between the monocytes of the patient and the control. The phagocytotic ability of the patient's monocytes was enhanced 1.5-fold compared with that of the control monocytes. These findings suggest that the activated monocytes may have degraded the modified LDL via a pathway other than CD36 or SR-A, and accumulated a great amount of lipids in vivo. In conclusion, the present study has demonstrated a possible pathogenesis of NXG that the activation of monocytes in vivo may contribute to the intracellular accumulation of lipoprotein-derived lipids leading to non-inherited xanthomatosis and the marked hypocholesterolemia.- - - - - - - - - - ranking = 1keywords = xanthoma (Clic here for more details about this article) |
2/402. Juvenile chronic myelogenous leukemia, neurofibromatosis 1, and xanthoma.The triple association of leukemia, xanthomatous skin lesions, and neurofibromatosis 1 (NF) was first described by Royer et al. in 1958. Most of the leukemias were of the juvenile chronic myelogenous type (JCML). We describe a 7-year-old male child with xanthoma, neurofibromatosis 1, and juvenile chronic myelogenous leukemia. His mother also had NF1. We suggest that the presence of xanthomas and NF1 in a young child should raise awareness of the possible development of JCML, especially in patients with a family history of NF1.- - - - - - - - - - ranking = 2.3333333333333keywords = xanthoma (Clic here for more details about this article) |
3/402. Verruciform xanthoma and concomitant lichen planus of the oral mucosa. A report of three cases.Verruciform xanthomas are benign muco-cutaneous lesions of unknown aetiology. They have a papillated surface and histologically they are characterised by the presence of foam cells in connective tissue papillae between elongated parakeratinised epithelial rete ridges. Three cases are reported in which oral mucosal verruciform xanthoma and oral mucosal lichen planus occurred concomitantly.- - - - - - - - - - ranking = 2keywords = xanthoma (Clic here for more details about this article) |
4/402. Verruciform xanthoma of the nose.We report a case of verruciform xanthoma of the nasal skin. The case is unique because the lesion both bled and has shown evidence of multicentricity.- - - - - - - - - - ranking = 1.6666666666667keywords = xanthoma (Clic here for more details about this article) |
5/402. Surgical excision of the tendon xanthoma in familial hypercholesterolemia--a case report.Familial hypercholesterolemia is an autosomal dominant disorder characterized by increased low-density lipoprotein cholesterol, premature atherosclerosis and tendon xanthomas. Genetic studies reveal familial hypercholesterolemia to be a dysfunction of LDL receptor gene on cell surface. Recently various mutations in the LDL receptor gene have been reported. When dna method is not available, the occurrence of tendon xanthomas, an isolated elevation of plasma cholesterol, with a normal concentration of plasma triglycerides virtually establishes the diagnosis of familial hypercholesterolemia. In this report, a 42-year-old male had tendon xanthoma at extensor surface of metacarpophalangeal joint of his right hand, olecranon of the left elbow and both knees, and Achilles tendons. The tendon xanthoma was excised for cosmetic reasons, and the wound healing was slower than average in this case. We suggest that before suture removal, wound healing must be complete. It is important that the hand surgeon recognize that tendon xanthoma is a physical sign of a potentially life-threatening disorder to the patient as well as his family, and that this disorder may respond favorably to early examination and management.- - - - - - - - - - ranking = 3keywords = xanthoma (Clic here for more details about this article) |
6/402. 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 = 0.00027785660149724keywords = histiocytosis (Clic here for more details about this article) |
7/402. poems syndrome with xanthomatous cells. Polyneuropathy Organomegaly Endocrinopathy M-protein skin changes.A diffuse xanthomatous infiltration was detected on a biopsy of the hyperpigmented patches in a 40-year-old man with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, skin changes) syndrome. Multiple biopsies of the hyperpigmented patches showed diffuse or perivascular foamy histiocytes in the upper and mid dermis, a sparse infiltrate of lymphocytes, fibrosis, hyperpigmentation of the basal layer, and capillary proliferation. The foamy cells were positive for periodic acid-Schiff (PAS) with diastase, oil red-O, and CD68. To our knowledge, this is the first case with foamy histiocytes without xanthomatous-appearing skin lesions in poems syndrome. It would be worth searching for xanthomatous histiocytes in the hyperpigmented skin of patients with poems syndrome.- - - - - - - - - - ranking = 2.3333333333333keywords = xanthoma (Clic here for more details about this article) |
8/402. Management of cutaneous verruciform xanthoma.Verruciform xanthoma is an uncommon mucocutaneous condition of uncertain cause that only occasionally affects the skin. The histopathology is distinctive for the presence of foamy histiocytes present within elongated dermal papillae. Although simple excision of intraoral lesions is reportedly curative, treatment of cutaneous lesions has not been previously reported. We describe a 62-year-old man with a large lesion of verruciform xanthoma affecting both inguinal folds. Immunohistochemical staining, reverse transcriptase polymerase chain reaction for human papilloma virus, and ultrastructural analysis were performed to investigate the pathogenesis of this lesion. The results of these studies support the theory that the source of lipid in dermal histiocytes is degenerating keratinocytes. Initial treatment with wire loop electrosection, pulsed dye (585 nm) laser, and x-ray therapy of this patient proved unsuccessful. Preliminary success has been achieved using wide surgical excision with primary closure.- - - - - - - - - - ranking = 2keywords = xanthoma (Clic here for more details about this article) |
9/402. Extensive intracranial xanthoma associated with type II hyperlipidemia.Xanthomas are associated with a spectrum of medical conditions, most commonly disorders of lipid storage and lipid metabolism. They occur primarily in the subcutaneous tissues, especially along the achilles tendon and the extensor tendons of the hands. Intracranial xanthomas are extremely rare. We present a case of an extensive xanthoma of the temporal bone in a patient with hyperlipidemia.- - - - - - - - - - ranking = 2keywords = xanthoma (Clic here for more details about this article) |
10/402. Xanthogranulomatous appendicitis--an incidental finding of localized pathology.The clinical, histopathological, and electron microscopic features of an unusual case of xanthogranulomatous appendicitis are reported. The patient, a 37-year-old female, presented with typical signs of acute appendicitis and the appendix appeared slightly dilated at laparatomy. The histopathological sections showed numerous xanthoma cells mixed with inspissated fecaliths. Electron microscopy disclosed the presence of xanthoma cells filled with electron-lucent lipid droplets of variable size. The ultrastructural characteristics of these cells enabled the distinction of two types of lipid-laden histiocytes, in relationship to the size of the lipid droplets. Since the lipid droplets were seen also in cells other than histiocytes, it appears that these changes are secondary to a common mechanism, comprising factors such as obstruction, hemorrhage, inflammation, and local hypoxia.- - - - - - - - - - ranking = 0.66666666666667keywords = xanthoma (Clic here for more details about this article) |
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