Abstract

Purpura Fulminans (PF) is a rare dermatologic emergency of intravascular thrombosis often associated with disseminated intravascular coagulation, multi-organ failure, and a high morbidity and mortality. PF is caused by the activation of inflammatory pathways, thrombus formation, and inhibition of fibrinolysis leading to widespread clot formation. It frequently occurs with severe bacterial sepsis, most commonly meningococcemia. Clinically it presents as widespread purpura that can rapidly progress to cutaneous hemorrhage and necrosis, often leading to limb amputations. Due to the rapid progression and critically ill patients, these lesions are frequently difficult to biopsy. Here, we describe a case of Purpura Fulminans biopsied in its early stages.

A 55-year-old female presented to the hospital and admitted to the MICU with septic shock secondary to a urinary tract infection and acute on chronic kidney disease. Additionally, she was in acute respiratory failure with a lactic acidosis. Infectious workup was notable for Citrobacter and Enterobacter in her urine and Neisseria in her blood. The dermatology team was consulted for progressive diffuse purpura of 2-3 days duration. On physical examination, the patient had diffuse retiform purpura with areas of necrosis covering the abdomen, chest, and back most prominently, but also involving the extremities, genitals/groin, and forehead. Histopathologic sections showed diffuse dermal hemorrhage without vasculitis and with scattered fibrin thrombi in the vessels. Additionally, there was variable necrosis of the epidermis and eccrine glands. Clinicopathologic correlation strongly supported a diagnosis of purpura fulminans.