(Last Updated On: December 14, 2018)

Authors: 

Abstract

Introduction: Calcemic uremic arteriolopathy (CUA), or usually known as calciphylaxis, is an uncommon, highly morbid disorder presenting with skin ischemia and necrosis. It predominantly occurs, but not exclusively, in patients with end-stage renal disease (ESRD).

Case Summary: We report two Filipino females with end-stage renal disease on chronic dialysis with non-healing ulcers. A 39-year old Filipino female, diagnosed with chronic kidney disease on hemodialysis for 8 years, presented with a 2-week history of initially erythematous tender plaques in the right medial thigh which eventually became violaceous. A 6-mm punch biopsy of the center of the violaceous plaque showed extensive dermal necrosis and calcifications of the vessels of the subcutaneous fat with fat necrosis. A 40-year old Filipino female, known diabetic and hypertensive, with chronic kidney disease on hemodialysis for 5 years, presented with a one-month history of non-healing rapidly developing multiple tender, brown to slightly erythematous subcutaneous nodules on both legs, that eventually became large ulcers with black eschar. A 5-mm punch biopsy of a subcutaneous nodule showed multiple amorphous basophilic material in the subcutaneous fat with necrosis of the adipocytes and sclerosis surrounding the calcifications. Some vessels of the panniculus showed medial calcifications with refractile red blood cells within the lumen.

Conclusion: CUA histologically appears to be a morphologic spectrum of a calcific thrombogenic microangiopathy, beginning with medial vascular calcification. Key histopathologic findings are diverse and include calcifications (both distinct and microcalcifications) in dermal and subcutaneous vessels and vascular thrombosis with subsequent ischemic necrosis of the dermis and panniculus.

 

Citation

 

Keywords

calciphylaxis, vascular calcification, ulcer, end-stage renal disease

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