Brazilian purpuric fever (BPF) was first recognized in late 1984 in the town of Promissao, Sao Paulo State, Brazil (1). The disease was characterized by the acute onset of high fever, vomiting, and abdominal pain, followed by purpura, vascular collapse, and death in children 3 months to 8 years of age. There was no evidence of meningitis, and blood cultures were negative when obtained, although some patients may have received antibiotics. Haemophilus aegyptius (Haemophilus influenzae, biotype III) was isolated from a nonaseptically obtained skin scraping of a petechia from an affected child.

Although the etiology could not be determined at the time of the outbreak, an epidemiologic investigation indicated disease was associated with preceding purulent conjunctivitis. H. aegyptius was the most commonly isolated organism from children with purulent conjunctivitis in Promissao; however, conjunctival cultures had not been obtained from children who subsequently developed BPF. Surveillance for BPF also identified other cases, including an outbreak of 17 cases that had occurred in 1984 in a town in the neighboring state of Parana. In addition, 12 sporadic cases in early 1985 and a cluster of eight cases in February 1986 all occurred in towns in Sao Paulo State.

In March 1986, an outbreak of purulent conjunctivitis occurred in Serrana, Sao Paulo State. Because of surveillance established for BPF and the development of protocols for collecting specimens, blood cultures were obtained from children in Serrana with fever and concomitant or recent histories of conjunctivitis and from those with clinical presentations consistent with BPF.

Ten children, 20 months to 6 years of age, had blood (eight) or cerebrospinal fluid (CSF) (two) cultures positive for H. aegyptius. However, none had evidence of meningitis, and there was evidence that the two culture-positive CSF specimens may have been contaminated with blood. All had fever; only five had petechiae and/or purpura. Four of the 10 died. Five of the 10 fit the previously established case definition of BPF (1), and nine had recent histories of conjunctivitis. The majority had received antibiotic eye drops for treatment of conjunctivitis. Among the 10 culture-confirmed cases and an additional case that fit the BPF case definition, patients who received intravenous antibiotics (generally ampicillin with or without chloramphenicol) before the development of petechiae or purpura (five of six) were more likely to survive than those who did not (one of five). Four additional patients with BPF and blood cultures positive for H. aegyptius were reported from four other towns in Sao Paulo State between March and June 1986.

Based on these findings, the case definition of BPF has been revised:

1. A febrile illness in a child with isolation of H. aegyptius from a normally sterile body site (e.g., blood, CSF).

   OR

1. An acute illness in a child aged 3 months to 10 years characterized by:

Fever of 38.5 C (101.3 F) or higher.

b. Abdominal pain and/or vomiting.

c. Development of petechiae and/or purpura.

d. No evidence of meningitis.

2. History of conjunctivitis within the 30 days preceding

the onset of fever. 3. At least one of the following two tests negative for

Neisseria meningitidis:

1. Blood cultures taken before antibiotic administration.

2. Serum or urine antigen detection.

Other laboratory data if obtained:

1. CSF containing 100 or fewer leukocytes per mm((3)) and

negative culture or antigen detection for pathogenic bacteria other than H. aegyptius.

b. Blood cultures taken before antibiotic administration negative for known pathogenic bacteria other than H. aegyptius.

c. Serologic studies, if obtained, negative for known pathogens other than H. aegyptius. Reported by Brazilian Purpuric Fever Task Force, Sao Paulo, Brazil, and Atlanta, Georgia.

Editorial Note

Editorial Note: BPF is a serious systematic illness that accumulating evidence suggests is due to invasive H. aegyptius disease. The illness characteristically begins with purulent conjunctivitis caused by H. aegyptius and progresses in a small percentage of patients to fever and other systemic manifestations due to disseminated H. aegyptius infection. If untreated, some patients may develop petechiae and purpura and die from overwhelming endotoxemia and shock. The clinical presentation of BPF is similar to meningococcemia.

The observation that the majority of patients had initially received local antibiotic therapy for treatment of conjunctivitis suggests that topical treatment of conjunctivitis may be inadequate in preventing BPF. However, use of systemic antibiotics to treat BPF before development of hemorrhagic skin lesions may be effective in preventing progression of the disease and reducing the case-fatality rate.

It is unknown whether BPF occurs in areas other than southern Brazil. In many areas, blood cultures may not be drawn if cases are treated empirically for presumed meningococcemia. However, the occurrence of clusters in areas separated by 250 miles suggests the potential for spread.

Questions about BPF or reports of similar illnesses should be directed to the Meningitis and Special Pathogens Branch, Division of Bacterial Diseases, Center for Infectious Diseases, CDC; telephone (404) 329-3687.

Reference

1. CDC. Preliminary report: epidemic fatal purpuric fever among children--Brazil. MMWR 1985;34:217-9.

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