When rash and fever become an emergency
Rashes and fevers are among the most common complaints seen in the pediatrician’s office. The differential diagnosis is often large and ranges from entities the pediatrician sees commonly such as erythema infectiosum to the less common diseases such as Kawasaki syndrome, to more potentially serious conditions such as vaccine preventable illnesses and everything in between.
Rashes and fevers are among the most common complaints seen in the pediatrician’s office. The differential diagnosis is often large and ranges from entities the pediatrician sees commonly such as erythema infectiosum to the less common diseases such as Kawasaki syndrome, to more potentially serious conditions such as vaccine preventable illnesses and everything in between.
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Although most rashes and fevers are from relatively benign viral causes, some rashes constitute real emergencies and demand prompt identification and action from the pediatrician. This article will review several diseases that represent a rash and fever emergency in that the disease needs to be recognized promptly, and failure to recognize and treat may lead to poor outcomes for the patient.
Meningococcal infection
Neisseria meningitidis can lead to a life-threatening infection in children and adolescents that most commonly presents as: 1) meningitis; 2) meningitis with meningococcemia; or 3) meningococcemia without meningitis. Known risk factors include both clinical and social parameters such as1-5:
· Complement deficiency-either congenital or functional as in lupus or nephrotic syndrome;
· Splenectomy;
· Inhabiting confined spaces such as daycare or dormitories;
· Human immunodeficiency virus infection;
· Intimate kissing with multiple partners;
· Recent viral illness or influenza infection;
· Being a college student;
· Attendance at a bar;
· Smoking.
Prompt identification of this rash is essential because the disease can be rapidly fatal with mortality rates of 10% to 25%. The time frame from initial symptoms to death can be relatively short. In a review of 448 hospital admissions for meningococcal disease, the median time from symptom onset to hospital admission was less than 24 hours, and younger age was associated with the shortest time frames. Nonspecific symptoms compatible with a self-limiting viral illness (eg, fever, sore throat, loss of appetite) were the earliest symptoms in the clinical presentation.6
One significant problem is that this disease is relatively uncommon in clinical practice, with pediatricians possibly seeing only a few cases over their careers. Additionally, the classic symptoms of petechial fever, hemorrhagic rash, meningismus, and altered mental status appear late in the illness.6
RASH
In the previously mentioned study, rash was the first classic symptom of meningococcal disease. The meningococcal rash typically is petechial with small (1mm to 2 mm) red or purple spots that do not blanch with pressure. The rash may progress to a palpable purpura with larger red or purple patches that look like bruises. Whereas any portion of the body can be affected, the trunk and extremities are most commonly involved. In severe cases, the lesions may evolve into necrosis.6
The rash also correlates with the clinical picture to some degree. Although studies of different groups report slightly different numbers, 1 study reported 75% of patients presenting with generalized maculopapular or petechial rash; 11% with peripheral purpuric/ ecchymotic lesions; and 14% with no rash at all. Meningitis was more common in patients with maculopapular, petechial, or no rash, whereas shock and disseminated intravascular coagulation were more common in patients presenting with peripheral purpuric or ecchymotic lesions. Mortality in the former group was 3% and in the latter was 44%.7
Read more: FDA approves first meningococcal B vaccine
Symptoms of early sepsis include leg pain, cold hands and feet, and abnormal skin signs such as mottling or pallor. Presence of these symptoms warrants close monitoring of the pediatric patient.6
WHO IS AT RISK?
Adolescents aged between 16 and 21 years have higher rates of meningococcal disease. As a result, the Advisory Committee on Immunization Practices recommends routine administration of the meningococcal conjugate vaccine between the ages of 11 and 18 years. Adolescents should receive the meningococcal vaccine when aged 11 to 12 years with a booster dose at age 16 years.8,9
Infants also are at risk but vaccination is not recommended unless they have a complement deficiency, anatomic asplenia, or functional asplenia.8,9
See “Helpful information for physicians” for useful links to more information on meningococcal disease.
NEXT: Infective endocarditis
Infective endocarditis
The rash and other clinical manifestations associated with infective endocarditis (IE) may help you make a diagnosis in this rash and fever emergency.
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Infective endocarditis is now more commonly an acute illness versus a chronic health condition, but this is variable. A pediatrician might expect to see either acute or subacute presentations of IE. Most children with IE have an identifiable risk factor such as:
· Congenital heart disease (CHD). Children with cyanotic heart disease such as Tetralogy of Fallot are at greater risk, with 35% to 60% of children developing IE also having a history of CHD. Additionally, a recent surgery is a significant risk factor in this group of pediatric patients. However, other preexisting heart conditions lead to increased risk of IE including cardiomyopathy or placement of an intracardiac device such as a defibrillator or pacemaker.10-13
· Central venous catheters (CVC). Critically ill children, premature infants, and those undergoing chronic treatment for cancer and other diseases such as cystic fibrosis are commonly in need of intermediate or long-term venous access. With a push to move care out of hospitals, venous access is becoming more and more common. Any patient with a CVC presenting with symptoms should be evaluated for IE.10,14-17
· Rheumatic heart disease (RHD). Although RHD was a common risk factor for IE in the past, the incidence of RHD has declined significantly and is now an uncommon cause of IE.11 However, many practicing pediatricians today have never seen RHD, which could lead to a potential delay in diagnosis and treatment.
Risk factors that are common in adults such as intravenous drug abuse and degenerative heart disease are not common in children.
SUBACUTE VS ACUTE PRESENTATION
Patients with either of the following presentations and the previously mentioned risk factors should be evaluated for IE.
In a subacute presentation, patients will experience low-grade fevers over a prolonged period of time. Other variable nonspecific complaints may include arthralgia; diaphoresis; fatigue; poor or worsening exercise capacity; myalgia; or weight loss.18
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Acute IE is not subtle but progressive and fulminant in many cases. Patients will appear severely or critically ill with fever. Patients often are hemodynamically unstable and at risk of heart valve destruction, abscess formation, and emboli.18
SKIN MANIFESTATION IN IE
Infective endocarditis is classically associated with a number of different skin lesions or lesions of the mucus membranes. These include:
· Petechiae. This 1-mm to 2-mm nonblanching skin lesion is a nonspecific finding for IE but it is the most common skin manifestation. Petechiae are nonblanching because they are caused by a minor hemorrhage in the skin. They may be found on the extremities or on mucosal membranes in the mouth or eyes.
· Splinter hemorrhages. These red or reddish-brown lines of blood under the nails also are a nonspecific finding for IE
· Janeway lesions. These erythematous macules are not painful and are found on the palms and soles.
· Osler nodes. These painful, violaceous nodules are found on the fingers and toes. Osler nodes are more common in subacute IE.
· Roth spots. These are nonpainful retinal hemorrhages with a central white spot.
Treatment is directed at the underlying bacterial cause. See “Helpful information for physicians” for useful links to more information on IE.
NEXT: Rocky Mountain spotted fever
Rocky Mountain spotted fever
Rocky Mountain spotted fever (RMSF) is a crucial rash and fever emergency because the development of the rash occurs later in the illness course, and there often is a short time period between the onset of the rash and more severe clinical symptoms. The illness can be fatal within the first 8 days, even among previously healthy people, and disease progression is highly variable. With early identification and treatment with appropriate antibiotics, many patients are successfully treated as outpatients, while others may require intravenous antibiotics, hospitalization, and intensive care.19
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Rocky Mountain spotted fever is a tick-borne illness from Rickettsia rickettsii, and although RMSF can be encountered anywhere with the United States, more than 60% of reported cases come from Arkansas, Missouri, North Carolina, Oklahoma, and Tennessee. In these states, the responsible tick is the American dog tick. Although RMSF occurs in every month of the year, most cases occur in the summer months with a peak in June and July. This can differ slightly in different parts of the country because of weather patterns and the particular tick involved.19
TYPICAL PRESENTATION
Patients usually have a viral-like illness initially consisting of fever and headache prominently with associated myalgia, fatigue, and conjunctival injection. Symptoms usually occur from 2 to 14 days after a tick bite, but patients or parents usually will not recall the tick bite. It is not unusual for patients to have several visits to 1 or multiple doctors because of the initial nonspecific symptoms. Additional symptoms patients may experience include abdominal pain, nausea, and vomiting. Children are less likely to complain of headache but more likely to have an associated rash compared with adults. Abdominal pain, conjunctival injection, and altered mental status also are more likely to occur in children.19
Rash typically follows initial symptoms by about 1 week. The rash is initially erythematous and maculopapular but it can progress to become petechial and purpuric in severe cases.19
RASH
Typically the rash will begin on wrists and ankles before spreading to the palms and soles and then centrally to include arms, legs, face, and trunk. The rash is described as maculopapular consisting of small, pink nonpruritic lesions, but this is highly variable. In 30% to 60% of patients, the rash will progress to petechiae, generally 6 or more days after the onset of symptoms. Development of petechiae is a marker of severe disease and treatment is best started before this progression when possible.19
More than 90% of patients have some form of rash during their illness, but some do not develop it until relatively late and 10% will never have a rash.
Rocky Mountain spotted fever sometimes can be difficult to distinguish from meningococcemia. However, a history of a tick bite, activities that would expose the patient to ticks, or residing in or visiting an area with RMSF ticks can help elucidate a diagnosis. Additional clinical clues include onset of rash 4 or more days after fever as well as relative leukopenia, hyponatremia, and elevated liver function tests.
WHO IS AT RISK?
Patients at higher risk of RMSF infection include19:
· Males;
· American Indians;
· Age over 40;
· Frequent exposure to dogs;
· Living in wooded or high-grass areas.
Patients at increased risk of dying of a RMSF infection include19:
· People with delayed treatment;
· Children aged younger than 10 years;
· American Indians;
· Immunocompromised persons.
TREATMENT
Standard treatment for RMSF is doxycycline (2.2 mg/kg body weight given twice a day for children weighing less than 45 kg (100 lb). Patients should be treated for at least 3 days after the fever subsides and until there is demonstrable clinical improvement. A total treatment course of 7 to 14 days is recommended. Use of antibiotics other than doxycycline is associated with an increased risk of death.
See “Helpful information for physicians” for useful links to more information on RMSF.
NEXT: Toxic-shock syndrome
Toxic-shock syndrome
Toxic-shock syndrome (TSS) has declined significantly since it was first described with an incidence of more than 13 per 100,000 in 1980 to 1.5 per 100,000 in 1986, with the rate not significantly declining further since then.20 Deaths from TSS also declined from 5.5% in 1980 to 1.8% in 1996.21 Initial cases of TSS were described in pediatric patients in the late 1970s, and most early cases were attributed to tampon use. With the removal from the market of certain brands of tampons, there was a steady decline in TSS cases, and an associated increase in nonmenstrual etiologies was noted.20-24
The syndrome can result from infection with either Staphylococcus aureus or Streptococcus pyogenes.
TYPICAL PRESENTATION
The case definition used for surveillance of TSS includes presentation with fever, hypotension, desquamating rash, and multiorgan system disease with involvement of at least 3 organ systems (eg, vomiting or diarrhea at presentation; myalgia or increased creatinine phosphokinase; mucus membrane involvement; abnormalities of renal function; abnormal liver enzymes; low platelets; abnormal mental status).25,26
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Although symptoms usually develop rapidly, there are case reports of a significant lag in time between inciting event and symptoms. Toxic-shock syndrome has been noted within 48 hours of menstruation or after surgery.27,28
WHO IS AT RISK?
Cases associated with menstruation and tampon use were common from the 1970s to the 1990s. However, a large number of cases today are because of other risk factors such as20,23,24,27,29-32:
· Cuts and bruises on the skin or other cutaneous lesions;
· Recent surgery or postoperative wound infection;
· Postpartum wound infection;
· Recent viral infection such as influenza or varicella;
· Infections such as sinusitis, mastitis, osteomyelitis;
· Burns; and
· Foreign body such as packing for a nosebleed.
RASH
The rash of TSS is typically described as a diffuse erythroderma that is macular and sunburn-like. It also may be fleeting and subtle. Scleral hemorrhages are common as well as hyperemia of mucus membranes. In surgical cases, the rash often is more pronounced near the wound. Desquamation of the palms and soles usually occurs 1 to 3 weeks from symptom onset.
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TREATMENT
Empiric treatment includes antibiotics directed at the likely cause (staphylococcal, clindamycin plus vancomycin; streptococcal, clindamycin plus a carbapenem such as imipenem or meropenem or a penicillin with beta-lactamase inhibitor such as ticarcillin-clavulanate or piperacillin-tazobactam) as well as local measures (eg, debridement of infectious focus) and fluid resuscitation.
See “Helpful information for physicians” for useful links to more information on TSS.
NEXT: Conclusion and references
Conclusion
Although most cases of rash and fever are benign, there are various situations in which a rash becomes an emergency that must be recognized promptly and treated quickly to ensure the best outcome for the patient.
REFERENCES
1. Fischer M, Hedberg K, Cardosi P, et al. Tobacco smoke as a risk factor for meningococcal disease. Pediatr Infect Dis J. 1997;16(10):979-983.
2. Cookson ST, Corrales JL, Lotero JO, et al. Disco fever: epidemic meningococcal disease in northeastern Argentina associated with disco patronage. J Infect Dis. 1998;178(1):266-269.
3. Imrey PB, Jackson LA, Ludwinski PH, et al. Outbreak of serogroup C meningococcal disease associated with campus bar patronage. Am J Epidemiol. 1996;143(6):624-630.
4. Tully J, Viner RM, Coen PG, et al. Risk and protective factors for meningococcal disease in adolescents: matched cohort study. BMJ. 2006;332(7539):445-450.
5. Ferguson LE, Hormann MD, Parks DK, Yetman RJ. Neisseria meningitidis: presentation, treatment, and prevention. J Pediatr Health Care. 2002;16(3):119-124.
6. Thompson MJ, Ninis N, Perera R, et al. Clinical recognition of meningococcal disease in children and adolescents. Lancet. 2006;367(9508):397-403.
7. Toews WH, Bass JW. Skin manifestations of meningococcal infection; an immediate indicator of prognosis. Am J Dis Child. 1974;127(2):173-176.
8. Centers for Disease Control and Prevention (CDC). Infant meningococcal vaccination: Advisory Committee on Immunization Practices (ACIP) recommendations and rationale. MMWR Morb Mortal Wkly Rep. 2013;62(3):52-54.
9. Strikas RA, Centers for Disease Control and Prevention (CDC); Advisory Committee on Immunization Practices (ACIP); ACIP Child/Adolescent Immunization Work Group. Advisory Committee on Immunization Practices recommended immunization schedules for persons aged 0 through 18 years-United States, 2015. MMWR Morb Mortal Wkly Rep. 2015;64(4):93-94.
10. Uwaydah MM, Weinberg AN. Bacterial endocarditis--a changing pattern. N Engl J Med. 1965;273(23):1231-1235.
11. Van Hare GF, Ben-Shachar G, Liebman J, Boxerbaum B, Riemenschneider TA. Infective endocarditis in infants and children during the past 10 years: a decade of change. Am Heart J. 1984;107(6):1235-1240.
12. Johnson DH, Rosenthal A, Nadas AS. A forty-year review of bacterial endocarditis in infancy and childhood. Circulation. 1975;51(4):581-588.
13. Ajdakar S, Elbouderkaoui M, Rada N, Drais G, Bouskraoui M. Multiple pulmonary emboli complicating infective endocarditis in a child with congenital heart disease [French]. Arch Pediatr. 2015;22(4):401-404.
14. García-Teresa MA, Casado-Flores J, Delgado Domínguez MA, et al; Spanish Central Venous Catheter Pediatric Study Group. Infectious complications of percutaneous central venous catheterization in pediatric patients: a Spanish multicenter study. Intensive Care Med. 2007;33(3):466-476.
15. Daher AH, Berkowitz FE. Infective endocarditis in neonates. Clin Pediatr (Phila). 1995;34(4):198-206.
16. Rech A, Loss JF, Machado A, Brunetto AL. Infective endocarditis (IE) in children receiving treatment for cancer. Pediatr Blood Cancer. 2004;43(2):159-163.
17. Day MD, Gauvreau K, Shulman S, Newburger JW. Characteristics of children hospitalized with infective endocarditis. Circulation. 2009;119(6):865-870
18. Ferrieri P, Gewitz MH, Gerber MA, et al. Unique features of infective endocarditis in childhood. Pediatrics. 2002;109(5):931-943.
19. Centers for Disease Control and Prevention. Rocky Mountain spotted fever (RMSF). Symptoms, diagnosis, and treatment. Available at: http://www.cdc.gov/rmsf/symptoms/index.html. Updated September 5, 2013. Accessed April 10, 2015.
20. DeVries AS, Lesher L, Schlievert PM, et al. Staphylococcal toxic shock syndrome 2000-2006: epidemiology, clinical features, and molecular characteristics. PLoS One. 2011;6(8):e22997.
21. Hajjeh RA, Reingold A, Weil A, Shutt K, Schuchat A, Perkins BA. Toxic shock syndrome in the United States: surveillance update, 1979-1996. Emerg Infect Dis. 1999;5(6):807-810.
22. Centers for Disease Control and Prevention (CDC). Toxic-shock syndrome, United States, 1970-1982. MMWR Morb Mortal Wkly Rep. 1982;31(16):201-204.
23. Reingold AL, Hargrett NT, Shands KN, et al. Toxic shock syndrome surveillance in the United States, 1980 to 1981. Ann Intern Med. 1982;96(6 pt_2):875-880.
24. Reingold AL, Hargrett NT, Dan BB, Shands KN, Strickland BY, Broome CV. Nonmenstrual toxic shock syndrome: a review of 130 cases. Ann Intern Med. 1982;96(6 pt 2):871-874.
25. Wharton M, Chorba TL, Vogt RL, Morse DL, Buehler JW. Case definitions for public health surveillance. MMWR Recomm Rep. 1990;39(RR-13):1-43.
26. Case definitions for infectious conditions under public health surveillance. Centers for Disease Control and Prevention. MMWR Recomm Rep. 1997;46(RR-10):1-55.
27. Reingold AL, Dan BB, Shands KN, Broome CV. Toxic-shock syndrome not associated with menstruation. A review of 54 cases. Lancet. 1982;1(8262):1-4.
28. Bartlett P, Reingold AL, Graham DR, et al. Toxic shock syndrome associated with surgical wound infections. JAMA. 1982;247(10):1448-1450.
29. MacDonald KL, Osterholm MT, Hedberg CW, et al. Toxic shock syndrome. A newly recognized complication of influenza and influenzalike illness. JAMA. 1987;257(8):1053-1058.
30. Ferguson MA, Todd JK. Toxic shock syndrome associated with Staphylococcus aureus sinusitis in children. J Infect Dis. 1990;161(5):953-955.
31. Abram AC, Bellian KT, Giles WJ, Gross CW. Toxic shock syndrome after functional endonasal sinus surgery: an all or none phenomenon? Laryngoscope. 1994;104(8 pt 1):927-931.
32. Dann EJ, Weinberger M, Gillis S, Parsonnet J, Shapiro M, Moses AE. Bacterial laryngotracheitis associated with toxic shock syndrome in an adult. Clin Infect Dis. 1994;18(3):437-439.
HELPFUL INFORMATION FOR PHYSICIANS
MENINGOCOCCAL INFECTION
CDC: Meningococcal disease
bit.ly/CDC-meningococcal-disease
CDC: Meningococcal vaccination
CDC: Clinical information
bit.ly/CDC-meningococcal-clinic
Immunization Action Coalition: Meningococcal photos
bit.ly/IAC-meningococcal-photos
AAP: Immunization/Meningococcal
bit.ly/AAP-meningococcal-immune
ROCKY MOUNTAIN SPOTTED FEVER
CDC: Symptoms, diagnosis, and treatment
Clinical Directors Network (CDN): Webcast: Recognizing and treating tick-borne diseases
CDC: Tick removal
CDC: New research on doxycycline
INFECTIVE ENDOCARDITIS
AHA: Infective endocarditis
bit.ly/IE-guidelines-AHA
Canadian Paediatric Society: AHA guidelines
Allen U. Infective endocarditis: updated guidelines. Can J Infect Dis Med Microbiol. 2010;21(2):74-77.
Cochrane Collaboration: Antibiotic treatment
Glenny A, Oliver R, Roberts GJ, Hooper L, Worthington HV. Antibiotics for the prevention of bacterial endocarditis (severe infection or inflammation of the lining of the heart chambers) in dentistry. Published October 9, 2013.
TOXIC-SHOCK SYNDROME
CDC: Streptococcal toxic-shock syndrome (STSS; Streptococcus pyogenes). 2010 case definition
bit.ly/CDC-streptococcal-TSS
DermNet NZ: Toxic-shock syndrome
Abbreviations: AAP, American Academy of Pediatrics; AHA, American Heart Association; CDC, Centers for Disease Control and Prevention.
Dr Bass is chief medical information officer and associate professor of medicine and pediatrics, Louisiana State University Health Science Center–Shreveport. The author has nothing to disclose in regard to affiliations with or financial interests in any organizations that may have an interest in any part of this article.
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