Loss of Flow and Purple Hands: That's What I Call Fulminans

Document Type

Conference Proceeding

Publication Date

5-1-2025

Publication Title

Am J Respir Crit Care Med

Keywords

antibiotic agent, antihypertensive agent, antithrombin, autoantibody, D dimer, fibrinogen, heparin, placebo, protein C, all cause mortality, anticoagulation, blister, blood culture, blood flow, case report, child, clinical article, conference abstract, continuous renal replacement therapy, diagnosis, disseminated intravascular clotting, drug therapy, dyspnea, female, fulminating purpura, human, hypotension, medical history, mortality rate, multiple organ failure, nose mucosa, partial thromboplastin time, patient comfort, protein deficiency, prothrombin time, purpura, rash, sepsis, Streptococcus group A, Streptococcus pyogenes

Abstract

Our patient was a 78-year old female with no known medical history who presented with shortness of breath and a painful rash of the right arm. The rash began approximately two weeks prior to respiratory symptoms. She rapidly deteriorated within several hours of presentation and became febrile and hypotensive. Within 48 hours, she developed diffuse, flaccid bullae and purpura over all extremities and anterior trunk. Blood cultures resulted positive for Group A Streptococcus pyogenes within 24 hours. Despite prompt initiation of broad spectrum and antitoxic antibiotics, multiorgan failure rapidly ensued. Shortly thereafter she developed necrosis of the oral and nasal mucosa, digits of the hands and feet, as well as small vessel thrombi leading to loss of blood flow to her feet and hands. It was also noted her CRRT filter clotted at an abnormally rapid rate, approximately 6-8 hours. Her clinical picture was consistent with purpura fulminans (PF), an even more rapidly progressive subtype of disseminated intravascular coagulation (DIC) that has an overwhelming mortality rate. The mechanism of PF remains poorly understood. Despite all that is unknown about PF, there is a wide consensus that PF occurs predominantly in children. Of the limited literature that exists, PF is believed to result from a number of etiologies including sepsis, inherited deficiency of proteins C (PC) and S (PS), or in the post-infectious period due to acquired autoantibodies against PC and PS. Unsurprisingly, there is not a general consensus regarding work up for PF. Multiple case reports and limited data suggest that lab findings of PF are largely consistent with DIC, which include prolonged PTT and PT, elevated D-dimer, and decreased fibrinogen. Interestingly, our patient's fibrinogen remained within normal limits but with prolonged PTT and PT. A small study by Lorelle et al. found a linear relationship between antithrombin (AT) and PC, which raises the question: can PC concentrate or anticoagulation treat PF? Again, no PF-specific studies exist to answer this question, but a study by Zarychanski et al investigating infection-associated DIC found no change in all cause mortality in those treated with heparin versus placebo or usual care. Unfortunately, our patient declined too rapidly for these investigations to be performed before her family elected to initiate comfort care measures. Especially considering the inevitably unfavorable outcome of PF, rapid recognition of PF is paramount and goes without saying that potential therapies including protein C supplementation and anticoagulation need be explored.

Volume

211

Issue

Supplement 1

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