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Epidemiologic Notes and Reports Preliminary Report: Epidemic Fatal Purpuric Fever Among Children -- Brazil

From October 14, to December 15, 1984, an outbreak of an unusual illness occurred in a small town (population 20,000) in the state of Sao Paulo, Brazil. Ten children, aged 3 months to 8 years, developed acute onset of high fever associated with vomiting and abdominal pain. Within 12-48 hours after onset of fever, these children developed purpura, followed by vascular collapse and necrosis of peripheral tissues. All 10 children died.

In all cases, laboratory examinations demonstrated only nonspecific findings, and cerebrospinal fluid (CSF) examinations did not suggest meningitis. White blood cell counts, elevated initially, were depressed immediately before death; hematocrits were normal; and thrombocytopenia was present. Bacterial cultures were negative, although antibiotics had been administered before obtaining cultures in most, if not all, instances. Autopsy examination characteristically demonstrated cerebral edema without meningitis, adrenal hemorrhage, lymphocyte depletion in lymphoid tissue, early fatty changes in the liver, fibrin deposition in small blood vessels, and changes consistent with acute shock in other organs. No organisms were visualized on microscopic examination by Gram or Dieterle staining.

At the same time the outbreak was occurring, another group of children in the same town developed what may have been a milder form of the same illness consisting of high fever, vomiting, and abdominal pain without progression to purpura or shock. No other illnesses were occurring in the town at above background level; no cases of meningitis occurred during this period; and no increase in any illnesses among older children or adults was noted.

The parents of children who died were extensively interviewed. No evidence was uncovered to suggest a common toxic exposure or preceding vaccination and no secondary transmission of the illness occurred. One consistent finding was noted--in most instances, a self-limited purulent conjunctivitis had preceded the onset of other symptoms by 3-15 days. A case-control study confirmed that children who died or children with suspected disease were significantly more likely than control children to have had conjunctivitis during the time of the outbreak (18/20 cases and suspected cases, compared with 9/20 controls (p = 0.006)). A systematic survey of households with children 10 years of age or under documented that a small epidemic of purulent conjunctivitis, distinguishable from previous epidemics of viral hemorrhagic conjunctivitis, had occurred in the low income peripheral sections of the town during October and November. Haemophilus aegyptius was the most common organism isolated from conjunctival cultures taken from children with purulent conjunctivitis during this time. No cultures of conjunctivitis were obtained from children who developed severe illness; however, H. aegyptius was isolated from a nonaseptically obtained skin scraping of a petechia from one case-patient.

In May 1984, another cluster of deaths among children occurred in a city in the neighboring state of Parana. Although coexisting background meningococcal disease made recognition at the time difficult, retrospective evaluation of these cases revealed that the age distribution, symptoms, clinical presentation, history of preceding conjunctivitis, and laboratory findings were similar to the cases in Sao Paulo State.

Epidemic disease in two geographically distinct areas suggested the presence of more widespread illness. To identify additional cases of this illness, a case definition for sporadic disease was established: Brazilian Purpuric Fever (BPF) (Febre Purpurica do Brasil)

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

    1. Fever 38.5 C (101.3 F) or higher.

    2. Abdominal pain and/or vomiting.

    3. Development of petechiae and/or purpura within 72 hours after onset of fever.

    4. No evidence of meningitis.

  2. History of conjunctivitis within the 15 days preceding 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.*

  4. In fatal cases, pathologic exam negative for microorganisms.

  5. Other laboratory data, if obtained:

    1. Cerebrospinal fluid containing 100 or fewer leukocytes mm((3)) and negative by culture or antigen detection for pathogenic bacteria.

    2. Blood cultures taken before antibiotic administration negative for known pathogenic bacteria.

    3. Serologic studies, if obtained, negative for known pathogens.

    Using this definition, sporadic cases of BPF occurring at times

other than the outbreak periods were identified in both towns and in five additional cities. A BPF task force, consisting of Brazilian laboratorians, clinicians, pathologists, and epidemiologists, as well as CDC investigators, has been created to further study the illness. High priority has been placed on collecting acute and convalescent sera, urines, autopsy tissues, and clinical material. Reported by the Brazilian Purpuric Fever Task Force, Sao Paulo, Brazil.

Editorial Note

Editorial Note: BPF, a serious illness affecting healthy young children, has not been previously recognized in Brazil. Epidemiologic and laboratory evaluations have not yet revealed an etiologic agent. Mortality has been approximately 70% for sporadic and epidemic cases identified to date. Transmission of illness from one child to another has not been observed. With clusters of cases, recognition of disease is not difficult; however, study of sporadic illness is hindered by the difficulty of differentiating cases of BPF from sporadic cases of meningococcemia, particularly in areas with poor laboratory support. Clinically, the illness is best categorized as purpura fulminans (1), a rare syndrome seen most commonly in children and characterized by purpura, fever, and hypotension. Purpura fulminans has been described in two forms: acute, resulting from overwhelming bacteremia, and delayed, most often occurring several days to weeks after a preceding viral or bacterial infection. There have been no reports of this second form of purpura fulminans occurring in epidemic form.

At present, the incidence of BPF and its geographic distribution are unknown. Additional laboratory and epidemiologic studies are being conducted by Brazilian health authorities and CDC. Questions about the syndrome or reports of similar illnesses should be directed to the Respiratory and Special Pathogens Epidemiology Branch, Division of Bacterial Diseases, CDC; telephone (404) 329-3687.

Reference

  1. Spicer TE, Rau JM. Purpura fulminans. Am J Med 1976;61:566-71. *If necessary, these samples can be stored for subsequent testing at a reference laboratory.



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