Case of Pseudo-xanthoma Elasticum Presenting with Ischaemic Claudication

Nonatherosclerotic Conditions


A Case of Pseudo-xanthoma Elasticum Presenting with Ischaemic Claudication


Lamb CM, Johns RA, Gallagher PJ, et al (Southampton Univ Hosps NHS Trust, UK) Eur J Vasc Endovasc Surg 43:478–479, 2012§



B.W. Starnes, MD



Evidence Ranking


• D



Expert Rating


• 3



Abstract


A 37-year-old man presented with symptoms of intermittent claudication. Investigations revealed atypical calf vessel disease but no obvious aetiology. Ten years later he re-presented with worsening symptoms. CT angiography confirmed the atypical pattern of lower limb arterial disease but also noted calcification of the renal parenchyma, myocardium and scrotum. A diagnosis of pseudo-xanthoma elasticum was confirmed by skin biopsy. Pseudo-xanthoma elasticum is a rare condition that presents infrequently to vascular surgeons. Early recognition should prompt aggressive risk factor management to slow accelerated atherosclerosis. Clinicians should be aware of the clinical features of this condition to allow early diagnosis (Fig 2).


image

Figure 2 (A) A low power view of a skin biopsy, stained with haematoxylin and eosin, taken from the neck lesion. Note that the epithelium is normal. The large mass of sub epithelial collagen shows degenerative change (Scale bar 1000 μm). A high power view of the abnormal collagen is shown in the upper left inset. The normal arrangement of the collagen bundles has been lost and many are clearly fragmented (Scale bar 250 μm). (B) Photograph showing typical PXE skin lesion superior to right clavicle. The patient, when questioned, had initially attributed this lesion to a childhood scald. (Reprinted from European Journal of Vascular and Endovascular Surgery, Lamb CM, Johns RA, Gallagher PJ, et al. A case of pseudo-xanthoma elasticum presenting with ischaemic claudication. Eur J Vasc Endovasc Surg. 2012;43:478–479. Copyright 2012, with permission from the European Society for Vascular Surgery.)

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Apr 1, 2017 | Posted by in CARDIOLOGY | Comments Off on Case of Pseudo-xanthoma Elasticum Presenting with Ischaemic Claudication

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