Brazilian Purpuric Fever

Brazilian purpuric fever
Classification and external resources
Specialty	infectious disease
ICD-10	A48.4
DiseasesDB	35123
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Brazilian purpuric fever (BPF) is an illness of children caused by the bacterium Haemophilus influenzae biogroup aegyptius which is ultimately fatal due to sepsis. BPF was first recognized in the São Paulo state of Brazil in 1984. At this time, young children between the ages of 3 months and 10 years were contracting a strange illness which was characterized by high fever and purpuric lesions on the body. These cases were all fatal, and originally thought to be due to meningitis. It was not until the autopsies were conducted that the cause of these deaths was confirmed to be infection by H. influenzae aegyptius. Although BPF was thought to be confined to Brazil, other cases occurred in Australia and the United States during 1984–1990.

Haemophilus species are non-spore-forming gram-negative coccobacilli . They lack motility and are aerobic or facultatively anaerobic. They require preformed growth factors that are present in blood, specifically X factor(hemin) and V factor(NAD or NADP). The bacterium grows best at 35–37 °C and has an optimal pH of 7.6. Haemophilus species are obligate parasites and are part of the normal flora of the human upper respiratory tract.

In documented BPF cases, the symptoms include high fever (101.3 degrees F or higher), nausea, vomiting, severe abdominal pain, septic shock, and ultimately death. A history of conjunctivitis 30 days prior to the onset of fever was also present in the documented BPF cases. The physical presentation of children infected with BPF include purpuric skin lesions affecting mainly the face and extremities, cyanosis, rapid necrosis of soft tissue, particularly the hands, feet, nose, and ears. Analysis of the fatalities due to BPF showed hemorrhage in the skin, lungs, and adrenal glands. Histopathology showed hemorrhage, intravascular microthrombi and necrosis in the upper dermis, renal glomeruli, lungs, and hepatic sinusoids.

A positive BPF diagnosis includes the clinical symptoms (mainly the fever, purpuric lesions, and rapid progression of the disease), isolation of Haemophilus Influenzae Biogroup aegyptius from blood, and negative laboratory tests for Neisseria meningitidis. The negative tests for Neisseria meningitidis rules out the possibility of the symptoms being caused by meningitis, since the clinical presentations of the two diseases are similar.

The eye gnat (Liohippelates) was thought to be the cause of the conjunctivitis epidemic which occurred in Mato Grosso do Sul in 1991. These gnats were extracted from the conjunctival secretions of the children who were infected with conjunctivitis. 19 of those children developed BPF following the conjunctivitis. Other modes of transmission include contact with the conjunctival discharges of infected people, ophthalmic instruments which have not been properly sterilized, sharing eye makeup applicators or multiple-dose eye medications.

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