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Diffuse dermal angiomatosis with clinical features simulating calciphylaxis in the setting of end-stage renal disease

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INTRODUCTION Reactive angioendotheliomatosis refers to a group of benign disorders characterized by intravascular or extravascular endothelial hyperplasia without cytologic atypia. Diffuse dermal angiomatosis is a rare, acquired, and typically localized… Click to show full abstract

INTRODUCTION Reactive angioendotheliomatosis refers to a group of benign disorders characterized by intravascular or extravascular endothelial hyperplasia without cytologic atypia. Diffuse dermal angiomatosis is a rare, acquired, and typically localized form of reactive angioendotheliomatosis. Vascular hyperplasia in diffuse dermal angiomatosis results from ischemia or inflammation that upregulates vascular endothelial growth factor. Diffuse dermal angiomatosis can also be observed in the setting of atherosclerosis, which can result in embolization to distant vessels supplying the skin. Clinically, diffuse dermal angiomatosis presents as erythematous, violaceous, and purpuric patches or plaques. Extensive disease can result in central ulceration, necrosis, and subsequent widespread involvement. Calciphylaxis is a life-threatening vasculopathy characterized by necrosis resulting from calcification of arterioles in the subcutis, most commonly observed in the setting of end-stage renal disease. Rarely, diffuse dermal angiomatosis has been observed in association with calciphylaxis. Herein, we present a case of diffuse dermal angiomatosis in the setting of endstage renal disease, with clinical features simulating calciphylaxis.

Keywords: diffuse dermal; dermal angiomatosis; calciphylaxis; renal disease

Journal Title: JAAD Case Reports
Year Published: 2020

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