1/42. 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 = sclerosis (Clic here for more details about this article) |
2/42. Autosomal dominant craniometaphyseal dysplasia with atypical features.Craniometaphyseal dysplasia (CMD) is a rare genetic disorder of bone modelling characterised by hyperostosis and sclerosis of the craniofacial bones, and abnormal modelling of the metaphyses. Clinically, autosomal dominant (AD) CMD is characterised by facial distortion and cranial-nerve compression. The goals of surgical treatment for AD CMD are cosmetic recontouring of the sclerotic craniofacial bones, correction of nasal obstruction and correction or prevention of neurological manifestations. We describe the successful correction of AD CMD craniofacial manifestations in an individual with atypical findings, and outline an approach for correcting the craniofacial deformities associated with this rare disorder.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
3/42. Brick tea fluoride as a main source of adult fluorosis.An epidemiological survey was conducted in Naqu County, tibet in September 2001 to investigate the manifestations of fluorosis in adults caused by the habitual consumption of brick tea. Profiles were obtained for the total daily fluoride intake, environmental fluoride levels and average urinary fluoride concentration, and a physical examination and a skeletal radiographic study were conducted. One hundred and eleven 30-78-year-old adults were enrolled. It was found that the fluoride level of water sources in Naqu County was 0.10 /-0.03 mg/l; no evidence of fluoride air pollution was found, but the brick tea water processed foods--zamba and buttered tea--had fluoride contents of 4.52 /-0.74 mg/kg and 3.21 /-0.65 mg/l, respectively. The adult daily fluoride intake reached 12 mg, of which 99% originated from the brick tea-containing foods. The positive rate of clinical symptoms by physical examination was 89%; furthermore, 42 of the 111 subjects were diagnosed by X-ray. The positive examination rate was 83%. Although the osteosclerosis-type skeletal fluorosis (overall increased bone matrix density) affected 74%, arthropathy and arthritis affected a significant number of the patients, resulting in functional disability. The results suggest that this brick tea-type fluorosis had even more severe adverse effects on human health compared with both the water-type and coal combustion-type fluorosis that occurred in other areas of china.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
4/42. Multimodality imaging features of primary xanthoma of the calcaneus.Secondary xanthomatous features are histologically observed in various bone lesions, but primary xanthoma of bone is rare. We present a primary xanthoma of the right calcaneus in a 51-year-old woman who had no aberrant lipid metabolism. Roentgenograms showed a small osteolytic lesion in the calcaneal triangle, partially surrounded by bone sclerosis. Computed tomographic scans of the calcaneus showed multiple osteolytic areas, with an irregular trabecular pattern in the surrounding sclerotic bone. T1-weighted magnetic resonance images showed a lesion with central low signal intensity, surrounded by a peripheral ring with high signal intensity. The entire lesion showed high signal intensity on T2-weighted images, partially surrounded by areas with low signal intensity, concordant with reactive bone sclerosis. Histologically, the lesion consisted of numerous lipid-laden histiocytes arranged in sheets, scattered multinucleated giant cells and lymphocytes, and granulation tissues. There was no evidence of pre-existing lesions. Total excision of the tumor was curative.- - - - - - - - - - ranking = 2keywords = sclerosis (Clic here for more details about this article) |
5/42. Chinese herbs and bone disease.We treated a patient with an unusual bone disease at least partly associated with Chinese herbs. Seven years after 65-year-old man had begun to consume Chinese herbs, multifocal osteoarthralgias were noted, and the patient was hospitalized for renal dysfunction (serum creatinine, 2.8 mg/dl; urea nitrogen, 19 mg/dl). fanconi syndrome also was apparent. A renal biopsy specimen showed tubulo-interstitial fibrosis. Chinese herbs were discontinued and prednisolone was started, but bone and joint pain as well as renal function gradually worsened. Four years later, creatinine was 9.0 mg/dl and alkaline phosphatase was 571 IU/l. As bone scintigraphy revealed localized asymmetric lesions, Paget's disease of bone was suspected at first. However, neither osteosclerosis nor hypertrophy was seen in radiographs. Based on a bone specimen histology we diagnosed as mixed-type renal osteodystrophy including osteomalacia and osteitis fibrosa. Mosaic pattern of cement lines was not present. This case was not compatible with either Paget's disease or typical renal osteodystrophy as seen in dialysis patients. Etidronate disodium was effective in alleviating bone symptoms. The patient's bone disorder may be a new disease at least partly related to Chinese herbs independently of nephropathy.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
6/42. Systemic sclerosis could mask the presentation of psoriasis in a patient with symptomatic and bilateral sacroiliitis.psoriasis is rarely associated with systemic sclerosis (SSc), and sacroiliitis associated to connective tissue diseases is also rather rare. In this report we describe a case of a young woman with SSc who developed symptomatic and bilateral sacroiliitis. The clinical pattern of sacroiliac involvement of this patient resembles that of psoriatic sacroiliitis. HLA typing was compatible with both SSc and psoriasis. Psoriatic sacroiliitis could not be diagnosed, but, on the basis of these observations, the patient described could represent a case of PsA without psoriasis. and psoriatic cutaneous involvement masked by the presence of SSc.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
7/42. osteogenesis imperfecta of the temporal bone: CT and MR imaging in Van der Hoeve-de Kleyn syndrome.We report the progressive otic capsule demineralization around the membranous labyrinth and facial nerve in an adult patient with osteogenesis imperfecta tarda. Whereas the initial CT scan showed bandlike, undermineralized pericochlear areas, 2 years later, repeat CT performed because of hearing deterioration showed progression of these findings to the promontory, the round window niche, and the labyrinthine and tympanic segments of the facial nerve canal. MR imaging demonstrated enhancement of the abnormal otic capsule and of the intratemporal and canalicular facial nerve. The differential diagnosis of osteogenesis imperfecta tarda affecting the temporal bone includes otosclerosis, Paget disease, otosyphilis, and Camurati-Engelmann disease.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
8/42. Phalangeal microgeodic syndrome resulting in rapid digital shortening.Microgeodic phalangeal syndrome is a rare condition affecting the fingers in children. Radiographically, the affected phalanges show sclerosis with multiple small areas of osteolysis. The pathogenesis of microgeodic phalangeal syndrome is considered to be a transient disturbance of the peripheral circulation caused by cold temperatures. In most cases, the symptoms and the radiographic changes return to normal within several months without any treatment. We present a rare case of microgeodic phalangeal syndrome in the middle phalanx of the right index finger in an 8-year-old girl, in whom the affected phalanx shortened within 6 weeks of the initial presentation. It was speculated that minor trauma to the finger might have contributed to the collapse and shortening of the phalanx. The current case indicates that the use of a splint for the affected finger in the early period after onset of symptoms might be recommended to avoid digital shortening.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
9/42. Case report 705. Osteosclerotic sarcoidosis.In summary, sarcoidosis is a rare but well-recognized cause of diffuse osteosclerosis. The differential diagnosis of osteosclerosis is limited and includes osteoblastic metastases, sclerotic myeloma, myelofibrosis, and less common infiltrative bone marrow processes such as mastocytosis and sarcoidosis. In all of these entities the sclerosis is found most often in the axial (red marrow) skeleton. In this regard, it is interesting that cases of osseous sarcoid are usually lytic and located in the peripheral skeleton. In patients with osteosclerotic sarcoidosis, the diagnosis may be suggested by a past history of the disease or ancillary signs such as hilar node enlargement and subtle skin involvement. However, the specific diagnosis usually requires bone marrow biopsy with the demonstration of extensive, noncaseating granulomas.- - - - - - - - - - ranking = 3keywords = sclerosis (Clic here for more details about this article) |
10/42. The value of ultrasound in diagnosis and follow-up of fibrous cortical defect.Fibrous cortical defects are local disturbances of ossification, most commonly seen in the metaphysis of tubular bones in growing children. These lesions are usually clinically silent, and most of them heal by being replaced with normal bone. Along with typical age, location and absence of clinical symptoms, plain radiographic films allow the diagnosis of fibrous cortical defect to be made. Because of rare but occasionally occurring complications, follow-up examinations are necessary. In a child with fibrous cortical defect the ultrasound examination demonstrated that fibrous cortical defects display a rather typical sonographic image. One finds clearly delineated defects of the bone located in the vicinity of the knee joint, filled with hypo-echogenic soft tissue, also displaying prominent internal vascularisation on colour-Doppler. We followed this patient up with repeated US examinations revealing the lesions to be shrinking in size and becoming shallower. Echogenic foci within the lesions appeared. It could be presumed that these changes represent the healing process and are comparable to the areas of sclerosis seen on CT scans. We believe that ultrasound with all its benefits is a powerful complementary method for the diagnosis and follow-up of fibrous cortical defects in children.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
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