Chronic meningococcemia in a 16-year-old boy: a case report

Abstract

Herein, we present a case of meningococcal disease in a patient presenting with of a three-week history of fever, cutaneous vasculitis and joint pain, in whom chronic meningococcemia was retained as presumptive diagnosis, after the disease evolved towards meningitis. This unusual case illustrates the great heterogeneity in possible clinical presentations of Neisseria meningitidis infections and underlines that diagnosis should always be evocated when facing the triad of fever, vasculitic skin eruption and big joints arthralgia, in a person in otherwise good general condition.

Chronic meningococcemia

“…We present an unusual case of meningococcemia without meningitis, that also fits in the definition of chronic meningococcemia as a syndrome affecting mostly healthy young adults and characterized by a clinical course lasting for at least a week [4,7]. Serogroup B N. meningitidis , as it is the case of our patient, has been associated the most often with this clinical manifestation [4].…”

“…Chronic meningococcemia is a rare condition [[4], [5], [6], [7], [8], [9]], characterized by a clinical triad of rash, arthralgia and fever [5,6,8,10], that may be continuous, although in most cases is described as relapsing or recurrent with afebrile periods of variable duration (afebrile periods as long as 10 days have been described) [[2], [3], [4]]. The skin rash is typically maculopapular, but has also been described as nodular, petechial, polymorphous or even necrotic [2,11,12].…”

“…Infection by Neisseria meningitidis can produce a wide array of clinical manifestations, ranging from asymptomatic carrier status to a fulminant disease, in which death occurring a few hours after the first symptoms [[1], [2], [3], [4]]. Along this spectrum, there are rare descriptions of mild meningococcal disease, without organ failure, which may present as bacteremia without sepsis (typically mimicking an upper respiratory tract infection or a self-limited viral exanthema), meningococcemia without meningitis (in which the patient presents with skin rash, malaise, weakness, headache, leucocytosis and, potentially, hypotension) and meningitis without meningococcemia [1].…”

“…Rarely, N. meningitidis can also cause persistent or relapsing bacteremia associated with low-grade fever, rash and arthralgia, with or without arthritis, a clinical situation known as chronic meningococcemia [[4], [5], [6], [7], [8], [9]].…”

Chronic Meningococcemia

“…This condition is characterized by abrupt onset of fever and a petechial or purpuric rash, often associated with hypotension, shock, acute adrenal hemorrhage, and multiorgan failure [10]. Alongside the classic acute forms of the disease, there are rare chronic (benign) forms, defined as a meningococcal sepsis of at least one week duration without meningeal symptoms and characterized by a prolonged clinical course with intermittent fever, rash and migratory arthralgia in healthy individuals [7]. Chronic meningococcemia may be self-limiting, but meningitis and death can occur as a late complication [8].…”