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December 2, 1998

Adult Purpura Fulminans and Digital Necrosis Associated With Sepsis and the Factor V Mutation

Author Affiliations

Margaret A.WinkerMD, Senior EditorIndividualAuthorPhil B.FontanarosaMD, Senior EditorIndividualAuthor

JAMA. 1998;280(21):1829-1830. doi:10-1001/pubs.JAMA-ISSN-0098-7484-280-21-jbk1202

To the Editor.—Two adults with purpura fulminans related to sepsis were found to be heterozygous for the Factor V Leiden mutation. Each patient survived disseminated intravascular coagulation (DIC), shock, and digital necrosis, but eventually required digit amputations. We believe the underlying congenital hypercoaguable risk factor contributed to the severity of the clinical course.

After 6 hours of fever, chills, nausea, and headache, a previously healthy 42-year-old man presented to the emergency department. Splenectomy had been performed for childhood trauma. Striking cyanosis of the face, nose, ears, and extremities was present with severe hypotension. Blood cultures grew Streptococcus pneumoniae, and ceftriaxone was administered within the first hour after admission. Disseminated intravascular coagulation was evidenced by low platelet count (46,000), prolonged prothrombin time (19 seconds) and partial thromboplastin time (78 seconds, INR 2.5), and elevated fibrin split products. Penicillin was begun 12 hours after admission. On the second day, lobar pneumonia was evident in the lower right lung. Renal failure, oliguria, seizures, rhabdomyolysis, and respiratory failure followed. Dialysis was required for 2 weeks and mechanical ventilation for 3 weeks.