Fatal purpura fulminans and Waterhouse-Friderichsen syndrome from fulminant Streptococcus pneumoniae sepsis in an asplenic young adult

Abstract

Asplenic patients are at increased risk for sepsis and fulminant infection. Sepsis in these patients is typically secondary to encapsulated bacteria, with Streptococcus pneumoniae being the most frequent pathogen. Rare complications of severe sepsis include purpura fulminans and bilateral adrenal hemorrhage (Waterhouse-Friderichsen syndrome).We present the case of a 36-year-old woman, healthy except for splenectomy years prior for idiopathic thrombocytopenic purpura treatment, who presented with fever. Upon presentation to our hospital, three hours after symptoms onset, she had purpura fulminans and shock. Despite timely antimicrobials and maximal resuscitative efforts, her disease progressed and she expired 12 hours after symptoms onset. Autopsy revealed bilateral adrenal hemorrhage; acute adrenal crisis likely contributed to her refractory shock. Prior to her presentation, she had not received guideline-based post-splenectomy care.Sepsis in asplenic patients can be fulminant and rapidly fatal. Streptococcus pneumoniae remains the most frequent cause, despite decreasing rates in recent years related to widespread pneumococcal vaccination. Guideline-based vaccinations and “pill-in-pocket” therapy can be life-saving for asplenic patients. Purpura fulminans represents an extreme manifestation of disseminated intravascular coagulation, is more common in asplenic patients, and portends a poor prognosis. Waterhouse-Friderichsen syndrome can be seen concurrently with purpura fulminans and further portends a poor prognosis; pre-mortem diagnosis requires a high index of suspicion.

Sepsis and septic shock: endothelial molecular pathogenesis associated with vascular microthrombotic disease

“…MODS occur as a result of microthrombosis that causes reversible hypoxia in organs unless it advances to terminal multiorgan failure. Organ dysfunction syndrome in sepsis, including fulminant hepatic failure (FHF)/acute liver failure [92], acute necrotizing pancreatitis [93], myocardial infarction [95], acute adrenal insufficiency [50, 96, 97], and rhabdomyolysis [98] may be reversible with improvement of sepsis unless necrotic and gangrenous change develops as seen in tissue gangrene [90] and peripheral digit ischemic syndrome [91]. More serious combined phenotypic syndromes, which underlying pathogenesis is still being debated, include hepatic encephalopathy [92], hepato-renal syndrome [99, 100], hepatic coagulopathy [1–3, 101], and cardio-pulmonary syndrome [102].…”

“…For example, the tropism of pathogen Neisseria meningitides to the adrenal glands causes Waterhouse-Friderichsen syndrome [50, 96, 97, 106]. Indeed, “DIC”, which presently is being renamed as TTP-like syndrome or EA-VMTD/DIT [1–4, 20], has been associated with Waterhouse-Friderichsen syndrome [96, 97, 106, 107]. The role of AAI in septic shock has been established [107–110].…”

Septic Shock and Purpura Fulminans Due to Streptococcus pneumoniae Bacteremia in an Unvaccinated Immunocompetent Adult: Case Report and Review

“…Only a few cases have reported this dramatic of a presentation in otherwise healthy patients [11,[35][36][37]. Asplenia is a significant risk factor, as illustrated in the case of an otherwise healthy 36-yearold mother of 2 who died 12 hours after symptom onset despite timely and appropriate therapy [38]. Patients with PF may also progress to develop bilateral adrenal hemorrhages, known as Waterhouse-Friderichsen syndrome.…”

Pathology of Gangrene

“…"Purpura fulminans" represents an extreme skin manifestation of DIC and Waterhouse-Friderichsen syndrome (caused by bilateral adrenal hemorrhage). The disease is often seen in splenectomized or immunosuppressed patients [133][134][135], while it is also observed in healthy patients without a history of splenectomy [136].…”