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Child with Rocky Mountain spotted fever has the rash that is characteristic but typically does not appear until several days after fever onset.

Fatal Cases of Rocky Mountain Spotted Fever in Family Clusters - Three States, 2003

Posted 06/01/2004, medscape.com

Content

Rocky Mountain spotted fever (RMSF), a tickborne infection caused by Rickettsia rickettsii and characterized by a rash (Figure), has a case-fatality rate as high as 30% in certain untreated patients.[1] Even with treatment, hospitalization rates of 72% and case-fatality rates of 4% have been reported.[1-3] This report summarizes the clinical course of three fatal cases of RMSF in children and related illness in family members during the summer of 2003. These cases underscore the importance of 1) prompt diagnosis and appropriate antimicrobial therapy in patients with RMSF to prevent deaths and 2) consideration of RMSF as a diagnosis in family members and contacts who have febrile illness and share environmental exposures with the patient.

Case Reports

Oklahoma. In late May, a female child aged 7 years was taken to an emergency department (ED) with 2 days of fever (102.7 F [39.3 C]), malaise, abdominal pain, nausea, and vomiting. Viral gastroenteritis was diagnosed, and the patient was released. Four days later, the patient reported to a second ED with persistent fever, anorexia, irritability, photophobia, cough, diffuse myalgias, nausea, and vomiting. Physical examination showed hepatosplenomegaly and an erythematous papular rash with scattered petechiae on the trunk, arms, legs, palms, and soles. Laboratory results included an elevated white blood cell (WBC) count of 11.4 x 109 cells/L (normal range: 3.0-9.1 x 109 cells/L), thrombocytopenia (19 x 109 platelets/L [normal range: 150-350 x 109 platelets/L]), elevated aspartate aminotransferase (AST) of 279 U/L (normal: </=42 U/L), and elevated alanine aminotransferase (ALT) of 77 U/L (normal: </=48 U/L). In the ED, the patient was treated with intravenous (IV) doxycycline for suspected RMSF and transferred to a pediatric intensive care unit at a tertiary care medical center, where she had declining mental status, metabolic acidosis, and respiratory failure; the patient died 6 days after initially seeking treatment. IgG antibodies reactive with R. rickettsii at a reciprocal titer of 128 were demonstrated by using an indirect immunofluorescence antibody (IFA) assay in a serum specimen collected 2 days before death. Spotted fever group rickettsiae (SFGR) were detected by immunohistochemical (IHC) staining at CDC in autopsy specimens from the brain, skin, heart, lung, spleen, and kidney.

On June 1, the child's sister, aged 3 years, had fever, headache, myalgias, and vomiting; on the following day, she had an erythematous maculopapular rash on the trunk, extremities, palms, and soles. RMSF was diagnosed, and the child was treated with doxycycline; she recovered. Seroconversion of IgG antibodies reactive with R. rickettsii was demonstrated in acute and convalescent phase serum specimens obtained during illness and 5 months later. Both children played frequently in grassy areas near their home. No history of tick bite was reported, although ticks were frequently observed on the family's pet dogs and often were manually removed by members of the household.

Kentucky. In early August, a male child aged 2 years was taken to a pediatrician after 1 day of fever (101.0 F [38.3 C]) with a papular rash on his legs, arms, trunk, and back. An unspecified viral syndrome was diagnosed, and the child was treated with nonsteroidal anti-inflammatory drugs. During the next 2 days, the child continued to have fevers, spiking to 102.0 F-103.0 F (38.9 C-39.4 C), and variable rash. The child was examined in an ED and discharged with a diagnosis of viral infection. Four days after initial treatment, the child was again evaluated by a pediatrician because of lethargy and refusal to walk. Laboratory tests showed thrombocytopenia (42 x 109 platelets/L), a WBC count of 3.3 x 109 cells/L, anemia (hemoglobin 10.4 g/dL [normal range: 13.8-17.2 g/dL]), and hyponatremia (134 mmol sodium/L [normal range: 135-145 mmol sodium/L]). The next day, the child was admitted and treated with IV ceftriaxone and methylprednisolone. Two days later, the child was transferred to a tertiary care hospital. Physical examination at admission revealed a fine petechial rash on the groin, trunk, ankles, and palms. The patient was treated with IV vancomycin, cefotaxime, and doxycycline. His condition continued to deteriorate; 8 days after initial treatment, he died from multiple system failure. A serum specimen collected 2 days earlier tested positive by enzyme immunoassay for IgM antibodies reactive with R. rickettsii at 9.4 index value units (index values >2.0 were considered reactive by the testing laboratory). SFGR were detected by IHC stain in autopsy specimens of the brain, skin, heart, lung, spleen, kidney, lung, and adrenal gland.

The child's mother, aged 40 years, was hospitalized 2 days before her son's death with 2 days of diplopia, dizziness, headache, and fever. Oral doxycycline and IV ceftriaxone were administered; she was discharged after 5 days. Seroconversion of IgG antibodies reactive with R. rickettsii was demonstrated in acute and convalescent phase serum specimens obtained during illness and 2 weeks later. The family lived near a lake with woods. The mother did not recall any recent tick bites, travel, or participation in outdoor activities, by herself or her son prior to illness onset.

Arizona. In mid-August, a male child aged 14 months was taken to a community health clinic after 1 day of fever (103.7 F [39.8 C]), with a maculopapular rash, including the palms and soles, and thick white exudates on the tongue. Chest radiographic evaluation showed a possible right lower lobe infiltrate. The child was treated with intramuscular cefotaxime, acetominophen, and antifungal medication for presumptive thrush. The next day, the child visited the clinic with nausea, vomiting, anorexia, and dehydration. The patient was transferred to a referral hospital for treatment of pneumonia, roseola infantum, and thrush; on admission, the patient had a temperature of 105.7 F (41 C). After 3 days, he was transferred to a tertiary care hospital with a diagnosis of sepsis and disseminated intravascular coagulopathy. The patient was treated with IV ceftazidime and vancomycin. Laboratory findings included an elevated WBC count (16.2 x 109 cells/L), thrombocytopenia (46 x 109 platelets/L), and elevated levels of AST (291 U/L) and ALT (99 U/L). Six days after initial treatment, the child died of pulmonary hemorrhage; an autopsy was not performed. A serum specimen obtained 5 days before the child's death tested negative by IFA for IgM and IgG antibodies reactive with R. rickettsii; however, R. rickettsii DNA was amplified from serum by polymerase chain reaction (PCR) assay. A serum specimen obtained from a brother, aged 5 years, showed IgM and IgG antibodies reactive to R. rickettsii, indicating recent exposure. The children lived in a rural environment with low shrubs and grasses and frequently interacted with free-roaming dogs with ticks; however, neither child had a history of recent tick bite.

Reported by: C Levy, MS, J Burnside, MS, T Tso, Arizona Dept of Health Svcs. S Englender, MD, M Auslander, DVM, S Billings, DVM, Div of Epidemiology and Health Planning, Kentucky Dept for Public Health. K Bradley, DVM, J Bos, MPH, L Burnsed, MPH, Div Communicable Diseases, Oklahoma Dept of Health. J Brown, MD, D Mahoney, MD, K Chamberlain, M Porter, C Duncan, B Johnson, R Ethelbah, K Robinson, M Wessel, S Savoia, MD, C Garcia, J Dickson, D Kvamme, D Yost, MD, M Traeger, MD, Indian Health Svc. J Krebs, MS, C Paddock, MD, W Shieh, MD, J Guarner, MD, S Zaki, MD, D Swerdlow, MD, J McQuiston, DVM, WL Nicholson, PhD, Div of Viral and Rickettsial Diseases, National Center for Infectious Diseases; L Demma, PhD, EIS Officer, CDC

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