| A case of pseudoxanthoma elasticum of the left internal mammary artery from a 61-year-old male who underwent coronary artery bypass grafting is reported. Intraoperative evaluation of the left internal mammary artery revealed partial stenosis. Histologically, the stenotic portion showed pseudoxanthoma elasticum. This represents the first reported case of pseudoxanthoma elasticum in the internal mammary artery. ( info) |
2/151. Late onset focal dermal elastosis: a distinct clinicopathologic entity? Two patients, aged 72 and 69 years, had skin lesions clinically resembling pseudoxanthoma elasticum (PXE). On histologic examination, the lesions showed an increased accumulation of normal-appearing elastic fibers lacking the abnormalities that characterize PXE. Similar lesions have been described previously in two elderly patients by Tojima et al. (1995). The clinical and histologic differential diagnosis of these lesions is discussed, and it is concluded that they represent a clinicopathologic entity with undetermined prevalence and etiopathogenesis. ( info) |
3/151. Detection of choroidal calcium by computed tomography scan quantitative computerized tomography: a clinically useful diagnostic tool. OBJECTIVE: Quantitative computerized tomography (QCT), a scanning method routinely used to classify tissues based on their radio density, was used to detect choroidal calcification. DESIGN: case reports. PARTICIPANTS: Two patients with known or suspected choroidal calcium-choroidal osteoma (CO) and pseudoxanthoma elasticum (PXE) and five normal control subjects were assessed. INTERVENTION: The CT scan of choroid, B-scan ultrasound, fluorescein angiography. MAIN OUTCOME MEASURES: The QCT scan used to detect the presence of calcium based on a readout greater than 90. RESULTS: calcium was detected bilaterally, as predicted, in PXE, and in both eyes of CO, although only one eye had a clinically noted lesion. No control eye gave a scale readout consistent with calcium. No findings suggestive of calcium were noted with B-scan ultrasound or fluorescein angiography in PXE or CO. CONCLUSION: The QCT can detect calcium in the choroid when B-scan ultrasonography and CT scan fine sectioning fail to identify calcium despite high clinical suspicion. ( info) |
| In patients with pseudoxanthoma elasticum, severe organic coronary artery stenosis often occurs without coronary risk factors. However, this report presents the case of a 49-year-old woman with pseudoxanthoma elasticum who had coronary artery spasm with an angiographically normal coronary artery. In addition, coronary artery spasm was provoked with dipyridamole thallium-201 cardiac imaging. ( info) |
| pseudoxanthoma elasticum is a cause of abnormal skin laxity. A case of lower face and neck skin laxity is presented with its treatment by cutaneous rhytidectomy. ( info) |
6/151. Enlarged gamma band response of neuromagnetic auditory evoked fields in a visually impaired subject. Under acoustic stimulation a phase-locked response in the gamma band (near 40 Hz) in the latency range between 20 and 130 ms is evoked. We report on a considerably visually impaired woman with Gronblad-Strandberg syndrome which involves degeneration at the level of retina, but has no overt central nervous component to the degeneration. The subject exhibited an extraordinarily high power in the phase-locked gamma band response (GBR) which was found to be more than three, and sometimes more than four, standard deviations above the average of a group of 25 subjects with normal vision. Furthermore, the dipoles of her mismatch reaction and M200 were found to be located posteriorly to the dipoles of the M100. overall, both enlarged GBR and changed cortical representation could be results of cortical plasticity related to visual impairment. ( info) |
| A 68-year-old woman with pseudoxanthoma elasticum (PXE) was treated with oral vitamin d(3). After 2 weeks, new papules developed on the pre-existing plaques. biopsy specimens of a fresh papule showed more abundant calcium deposition than before therapy. Electron microscopy revealed electron-lucent areas, which suggested unusual mineralization. From these findings, we propose that in patients with PXE, certain cutaneous tissues are highly sensitive to vitamin d(3), resulting in microdeposition of calcium salts. ( info) |
| An 18 year old girl with pseudoxanthoma elasticum, a 7 year history of angina pectoris and evidence of an old anteroseptal myocardial infarction was found on coronary angiography to have three vessel coronary artery disease. A triple coronary artery-saphenous vein bypass graft was performed, and she has been asymptomatic for 1 year. Histologic examination of a segment of the right coronary artery revealed changes consistent with the vascular lesion of pseudoxanthoma elasticum. ( info) |
| pseudoxanthoma elasticum (PXE) is a heritable disorder of elastic fibers characterized by yellowish, coalescing papules on the loose and wrinkled flexural area. There have been no reports of osteoma cutis associated with PXE. A 17-year-old Korean girl presented cutis laxa-like marked wrinkling on the flexural area, and a skin biopsy specimen revealed multiple foci of ossification with irregularly clumped, basophilic-stained elastic fibers in the reticular dermis and calcium deposits along the elastic fibers. Ultrasonographic evaluation showed multiple tiny osteomas diffusely scattered along the entire abdominal wall, axillae, and medial aspect of the upper arms. We report the first case of osteoma cutis coexisting with cutis laxa-like PXE. ( info) |
| A 42-year-old white woman presented with clinical and histologic manifestations of both calcinosis cutis and pseudoxanthoma elasticum: discrete milia-like calcifications at the anterior aspect of the neck, a funduscopic examination with classic eye findings, peripheral vascular disease, and a mottled appearance of the skin at the axillae, groin, and lateral aspects of the neck. A younger sibling had similar skin lesions and deteriorating visual acuity. The patient was normocalcemic and normophosphatemic. This case may represent the coincidental occurrence of two rare entities in the same person or may be suggestive of a pattern of dystrophic calcification associated with pseudoxanthoma elasticum. ( info) |