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Cholesterol Embolization Syndrome With an Atypical Proximal Presentation Simulating Calciphylaxis

Bao Anh Patrick Tran, BA1; Robert Egbers, MD2; Lori Lowe, MD2,3; Yolanda R. Helfrich, MD2; Frank Wang, MD2
[+] Author Affiliations
1University of Michigan Medical School, Ann Arbor
2Department of Dermatology, University of Michigan, Ann Arbor
3Department of Pathology, University of Michigan, Ann Arbor, Michigan
JAMA Dermatol. 2014;150(8):903-905. doi:10.1001/jamadermatol.2013.8846.
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Cholesterol embolization syndrome (CES) is associated with endovascular procedures or anticoagulation, which disrupt atheromatous plaques within large arteries releasing cholesterol crystals.1 These crystals lodge in the vasculature of various organs, most commonly the skin.2,3 Cutaneous involvement classically presents in the distal lower extremities as livedo reticularis (49%), gangrene (35%), cyanosis (28%), ulceration (17%), nodules (10%), purpura (9%), and splinter hemorrhages.4 We report a case of CES with proximal cutaneous lesions mimicking calciphylaxis.

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Figure 1.
Clinical Images of Cholesterol Embolization Syndrome (CES) With an Atypical Proximal Presentation

A and B, Violaceous livedoid plaques on the bilateral flanks, buttock, and proximal thighs, with deep ulcerations on the lower back and buttocks. Although not shown in these images, livedoid changes were also present on the bilateral upper arms, and, in contrast to the classic presentation of CES, the toes were notably spared.

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Figure 2.
Histopathologic Findings of Cholesterol Embolization Syndrome

A 6-mm punch biopsy specimen from an ulcer on the left buttock shows cholesterol clefts within the lumen of deep dermal arteries, consistent with cholesterol embolization syndrome (hematoxylin-eosin, original magnification ×200).

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