“…Skin biopsies of affected areas fail to show granulomatous inflammation or fibri noid necrosis, demonstrating instead, when appropriately stained, medial calcinosis usually accompanied by inti mai proliferation and ischemic epidermolysis [2,5,15,[20][21][22]24], Stains for immunoglobulins and complement arc also negative [2], The peripheral gangrene frequently leads to surgical amputation of one or more extremities, or, in lesions of terminal phalanges, to eschar formation, demarcation and eventually self-amputation. In addition to acral gangrene and ischemic necrosis of skin and sub cutaneous fat tissue, the most characteristic clinical man ifestations, myopathy, fever, hypotension, dementia, and central nervous system dysfunction, cerebral, myocardial and bowel infarction have also been described [8,22,26,33]. The course of the disease is frequently rapidly pro gressive and sepsis, resulting from secondary infection of skin lesions, refractory hypotension and cerebrovascular accidents arc the leading causes of death.…”