Treating A Patient With Sepsis And Full Thickness Necrosis
Protein C. Research has shown that both genetic and acquired deficiencies in the natural anticoagulant protein C contribute to the development of purpura fulminans.14 Several studies have shown the clinical benefit of protein C supplementation with recombinant human activated protein C.12,15-18 Smith and colleagues showed administration of protein C in patients with purpura fulminans produced a 0 percent mortality rate despite a published mortality rate for purpura fulminans ranging from 40 to 80 percent.15
Antithrombin III. Administration of antithrombin III has the potential to reverse the procoagulant state that occurs in purpura fulminans. Studies have shown that antithrombin III has anti-inflammatory properties and decreases the inflammatory response of mononuclear cells and endothelial cells.19
Topical nitroglycerin. Topical nitroglycerin can convert to nitric oxide, a powerful natural vasodilator. Several studies have shown improved blood flow to the skin with topical nitroglycerin application.20,21 Reduction in pain resulting from poor tissue perfusion can also occur with topical nitroglycerin use.6
Surgical management. Researchers have proposed that aggressive fluid resuscitation and endotoxin-induced fluid shifts lead to tissue edema and subsequent compartment syndrome, which further decreases the blood supply in tissue already subject to vascular compromise. Warner and coworkers showed that performing fasciotomies early in the management of patients with purpura fulminans reduced the depth of necrosis and decreased the extent of amputations.4
Surviving patients typically require surgical treatment of skin necrosis and extremity gangrene. One manages skin necrosis similarly to frostbite and burns by delaying amputation until maximal collateral circulation has developed and demarcation of non-viable tissue has taken place.3 Researchers have described multiple techniques for the treatment of full thickness skin necrosis including skin grafting, synthetic skin grafts and amputation.2-4,22
What About The Prognosis Of Patients With Purpura Fulminans?
While the mortality rate of purpura fulminans has decreased with current treatment modalities, it remains a disabling condition with complex wounds complicated by secondary infections, often requiring major amputations.
A study by Warner and colleagues reviewing 70 cases of purpura fulminans showed a 40 percent mortality rate, 90 percent of patients required surgical intervention with skin grafting/amputation and 25 percent of patients required amputations of upper and lower extremities.4 Davis and coworkers reported 12 cases of purpura fulminans associated with peripheral gangrene with a 25 percent mortality rate, a 67 percent single limb amputation rate and a 33 percent four-limb amputation rate.23 Childers and colleagues reported a mortality rate of 43 percent in patients with purpura fulminans.5 Researchers have shown that rapid diagnosis, ICU management and early surgical intervention can decrease mortality in patients with purpura fulminans.5
Dr. Hoffman is in private practice in Boulder, Colo.
1. Marciniak E, Wilson HD, Marlar RA. Neonatal purpura fulminans: a genetic disorder related to the absence of protein C in blood. Blood. 1985; 65(1):15–20.
2. Edlich RF, Cross CL, Dahlstrom JJ, Long WB 3rd. Modern concepts of the diagnosis and treatment of purpura fulminans. J Environ Pathol Toxicol Oncol. 2008; 27(3):191-6.
3. Chu DZ, Blaisdell FW. Purpura fulminans. Am J Surg. 1982; 143(3):356-62.
4. Warner PM, Kagan RJ, Yakuboff KP, et al. Current management of purpura fulminans: a multicenter study. J Burn Care Rehab. 2003; 24(3):129-126.
5. Childers BJ, Cobanov B. Acute infections purpura fulminans: a 15-year retrospective review of 28 consecutive cases. Am Surg. 2003; 69(1):86-90.
6. Nolan J, Sinclair R. Review of management of purpura fulminans and two case reports. Br J Anaesth. 2001; 86(4):581-586.
7. Darmstadt GL. Acute infectious purpura fulminans: pathogenesis and medical management. Ped Dermatol. 1998; 15(3):169-83.