Purpura fulminans secondary to Xanthomonas maltophilia sepsis in an adult with aplastic anemia.
Purpura fulminans is a rare disease characterized by purpura ecchymosis, hypotension, and fever associated with disseminated intravascular coagulation. It often begins as a benign infectious process and subsequently progresses to a severe, catastrophic outcome. It is recognized to originate from congenital or acquired protein C deficiency. We present an unusual case of an adult with Xanthomonas maltophilia sepsis that subsequently developed into purpura fulminans with involvement of the four extremities. We discuss the importance of the protein C system in coagulation homeostasis and its relationship to purpura fulminans.
Department of Dermatology, Otaru City General Hospital, Japan.
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Kato
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Morioka
D000741:Anemia, Aplastic / Q000150:complications*; D005121:Extremities; D005734:Gangrene; D006801:Humans; D008297:Male; D008875:Middle Aged; D020151:Protein C Deficiency; D011693:Purpura / Q000209:etiology* / Q000473:pathology; D018805:Sepsis / Q000150:complications*; D014973:Xanthomatosis / Q000150:complications* / Q000175:diagnosis; D014974:Xanthomonas