Synonym: calcification-cutaneous necrosis syndrome, uremic gangrene syndrome, calcific uremic arteriolopathy, calcific azotemic arteriopathy, ischemic tissue necrosis
Pathologic features:
(1) progressive cutaneous necrosis
(2) calcification of small arteries
Cutaneous findings:
(1) flaccid or hemorrhagic bullae
(2) extremely painful purpuric nodules and plaques
(3) retiform purpura (livedo reticularis-like purpura)
(4) cutaneous ulceration with eschar
Distribution of lesions:
(1) acral (toes, fingers)
(2) distal (forearms, lower legs)
(3) proximal (buttocks, thighs, trunk)
(4) systemic (multi-organ involvement)
Features of systemic disease - involvement of noncutaneous tissues with:
(1) hypotension
(2) myopathy
(3) central nervous system infarction
(4) myocardial infarction
(5) bowel infarction
(6) tongue infarction
(7) multi-organ failure
Widespread systemic or proximal disease often has a poor prognosis.
