Cases reported "Xanthomatosis"

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1/620. 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. ( info)

2/620. 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. ( info)

3/620. 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. ( info)

4/620. necrobiotic xanthogranuloma with paraproteinaemia.

    A 51-year-old woman developed multiple periorbital nodules. The subsequent demonstration of IgG lambda paraproteinaemia and the histological features of necrobiotic xanthogranulomatous inflammation confirmed the clinical diagnosis of necrobiotic xanthogranuloma with paraproteinaemia. ( info)

5/620. 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. ( info)

6/620. Ultrasonic demonstration of xanthogranulomatous pyelonephritis.

    An ultrasound examination performed on a young woman with a long history of urinary tract infection demonstrated multiple subcapsular sonolucencies suggestive of multiple abscesses. The combination of the ultrasonic appearance, the clinical history, and a large nonfunctioning kidney with calculi on intravenous pyelography allowed us to suggest the diagnosis of xanthogranulomatous pyelonephritis. Ultrasound plays an even greater role in patients who are allergic to iodinated contrast material in whom intravenous pyelograms and arteriograms may be contraindicated. ( info)

7/620. Xanthogranulomatous cholecystitis associated with a xanthogranulomatous pseudotumour on the left diaphragm.

    We present a case of xanthogranulomatous cholecystitis associated with a xanthogranulomatous vegetation on the left diaphragm with breakthrough into the thoracic cavity. A similar case has not previously been reported. ( info)

8/620. Xanthogranulomatous oophoritis--a case report.

    This is a case of a 25 year old unmarried women who presented with intermittent fever and lower abdominal pain. laparotomy revealed a large cystic left sided tuboovarian mass adherent to surrounding structures and containing foul smelling fluid. microscopy showed extensive replacement of the ovary by a chronic inflammatory exudate composed predominantly of foamy macrophages. ( info)

9/620. Symptomatic Rathke's cleft cyst coexisting with central diabetes insipidus and hypophysitis: case report.

    We describe a 48-year-old female with acute onset of central diabetes insipidus followed by mild anterior pituitary dysfunction. magnetic resonance imaging (MRI) revealed enlargement of the hypophysis-infundibulum accompanied by a cystic component. She underwent a transsphenoidal exploration of the sella turcica. Histological examination showed foreign body type xanthogranulomatous inflammation in the neurohypophysis which might have been caused by rupture of a Rathke's cleft cyst. The MRI abnormalities and anterior pituitary dysfunction improved after a short course of corticosteroid administration, but the diabetes insipidus persisted. The histological findings in this case indicated the site of RCC rupture and the direction of the progression of RCC induced neurohypophysitis and adenohypophysitis. ( info)

10/620. 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. ( info)
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