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Mysterious Brazilian purpuric fever, a disease from a rural area

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Abraham Eisenstein, M.D. ▼ | September 1, 2018
Brazilian purpuric fever
Children   A disease that came and go. And came again

Back in 1984, an outbreak of a deadly new disease started to kill children in Brazil. Something that started with conjunctivitis became a disease with 90% fatality rate. And then it disappeared.

Brazilian purpuric fever (BPF) is a Haemophilus aegyptius–caused hemorrhagic illness of children that begins with conjunctivitis.

Haemophilus species are coccobacilli, a type of bacterium. They are parasites and part of the normal flora of the human upper respiratory tract and humans are the only natural host of them.

H. aegyptius was first described by Koch and then Weeks and the organism known as the Koch-Weeks bacillus was, after a number of other names, called H. aegyptius, or Haemophilus influenzae biogroup aegyptius.

When the doctors examined 13 children with a similar disease they thought it is part of a concomitant meningococcal disease outbreak. But there was no meningitis and an intriguing fact was that conjunctivitis was present before the more serious situation.

Brazilian purpuric fever begins with conjunctivitis caused by H. aegyptius. It then progresses in a small percentage of patients to fever and other systemic manifestations.

If left untreated, some patients may develop petechiae and purpura and die from endotoxemia (the presence of toxins in the blood) and shock.

The problem is BPF is similar to meningococcemia, a rare infection caused by the Neisseria meningitidis bacteria that also can cause meningitis.

Doctors then learned that antibiotics were not the complete solution: they were received for conjunctivitis but BPF still developed. Good news it that using antibiotics before development of hemorrhagic skin lesions may reduce the fatality rate.

The problem here is that it is often hard to obtain a proper diagnosis.

The disease starts as a common conjunctivitis and while we are treating it, the condition progresses to BPF. That means that valuable time is lost. Now, we can treat BPF with many common antibiotics but it develops fast to the point of no return.

There were many hypotheses why the disease appears but we do know that children under 5 years of age and those living in warmer geographic areas have been associated with a higher risk an infection.

Also, it was thought that BPF is something specific for Brazil but some cases were discovered in the U.S. and as far away as Australia.

The symptomps of a BPF infections are high fever, skin lesions, abdominal pain, nausea, vomiting, then followed by septic shock and death.

The most visible characteristic are purpuric skins lesions mostly on the patient's face and extremities. There are also rapid necrosis of tissues, especially on the hands, feet, nose, and ears.

There are many unaswered questions about BPF.

Why it started in agricultural cities? Why didn't it spread to large cities nearby? How come it didn't spread with people movement? If it's endemic to Brazil, how come it appeared in Australia? How come other infectious diseases spread very easily and BPF came, lasted for ten years or so and then disappeared?

Another mystery is Haemophilus aegyptius itself.

In short, more than 100 years after its discovery, we are not completely sure is Haemophilus influenzae different from Haemophilus aegyptius although a lot of efforts went into research. So, today H. aegyptius has been classified as a biogroup of H. influenzae.

We know that Haemophilus influenzae can't live long in the environment but can survive more than 18 hours in mucous and 12 hours on plastic.

If we add to that that a study shown a connection between day care attendance and contraction of BPF, we may conclude that day care facilities may be a good ground for the transmission of BPF not just by a direct contact but by other means.

The problem is, after the outbreak was done, new cases, although just a few of them, were reported in distant cities a few years later which means the infection can spread much further than initially thought, and after another calm period, sporadic cases were reported again.

It may very well be that there were other cases unrecognized by the medical staff not closely familiar with the infection.

BPF is a disease that requires mandatory reporting in Brazil because new outbreaks may still occur.

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