11/28/2017 Scarlet Fever: Background, Pathophysiology, Etiology

Scarlet Fever
Updated: Jun 09, 2017
Author: Bahman Sotoodian, MD; Chief Editor: William D James, MD more...

OVERVIEW

Background
Scarlet fever (known as scarlatina in older literature references) is a syndrome characterized by
exudative pharyngitis (see the image below), fever, and bright-red exanthem. It is caused by
streptococcal pyrogenic exotoxins (SPEs) types A, B, and C produced by group A beta-hemolytic
streptococci (GABHS) found in secretions and discharge from the nose, ears, throat, and skin.
Scarlet fever may follow streptococcal wound infections or burns, as well as upper respiratory tract
infections. Food-borne outbreaks have been reported. [1, 2] Reemergence of the condition is being
recognized, perhaps because of newer virulence of the streptoccocal bacteria. [3, 4, 5]

The exudative pharyngitis typical of scarlet fever. Although the tongue is somewhat out of focus, the whitish
coating observed early in scarlet fever is visible.

See 15 Back-to-School Illnesses You Should Know, a Critical Images slideshow, to help identify
conditions that may occur in young patients after they return to the classroom.

Ordinarily, scarlet fever evolves from a tonsillar/pharyngeal focus, although the rash develops in
less than 10% of cases of “strep throat.” The site of bacterial replication tends to be inconspicuous
compared to the possible dramatic effects of released toxins. Exotoxin-mediated streptococcal
infections range from localized skin disorders (eg, bullous impetigo) to the widespread eruption of
scarlet fever to the uncommon but highly lethal streptococcal toxic shock syndrome.

Pathophysiology
As the name “scarlet fever” implies, an erythematous eruption is associated with a febrile illness.
The circulating toxin, produced by GABHS and often referred to as erythemogenic or erythrogenic
toxin, causes the pathognomonic rash as a consequence of local production of inflammatory
mediators and alteration of the cutaneous cytokine milieu. This results in a sparse inflammatory
response and dilatation of blood vessels, leading to the characteristic scarlet color of the rash. [6]

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11/28/2017 Scarlet Fever: Background, Pathophysiology, Etiology

Usually, the sites of GABHS replication in scarlet fever are the tonsils and pharynx. Clinically
indistinguishable, scarlet fever may follow streptococcal infection of the skin and soft tissue,
surgical wounds (ie, surgical scarlet fever), or the uterus (ie, puerperal scarlet fever).

Etiology
Scarlet fever is a streptococcal disease. Streptococci are gram-positive cocci that grow in chains.
They are classified by their ability to produce a zone of hemolysis on blood agar and by differences
in carbohydrate cell wall components (A-H and K-T). They may be alpha-hemolytic (partial
hemolysis), beta-hemolytic (complete hemolysis), or gamma-hemolytic (no hemolysis).

Group A streptococci are normal inhabitants of the nasopharynx. Group A streptococci can cause
pharyngitis, skin infections (including erysipelas pyoderma and cellulitis), pneumonia, bacteremia,
and lymphadenitis.

Most streptococci excrete hemolyzing enzymes and toxins. The erythrogenic toxins produced by
GABHS are the cause of the rash of scarlet fever. The erythema-producing toxin was discovered
by Dick and Dick in 1924. Scarlet fever is usually associated with pharyngitis; however, in rare
cases, it follows streptococcal infections at other sites.

Although infections may occur year-round, the incidence of pharyngeal disease is highest in
school-aged children during winter and spring and in a setting of crowding and close contact.
Person-to-person spread by means of respiratory droplets is the most common mode of
transmission. It can rarely be spread through contaminated food, as seen in an outbreak in China.
[2]

The organism is able to survive extremes of temperature and humidity, which allows spread by
fomites. Geographic distribution of skin infections tends to favor warmer or tropical climates and
occurs mainly in summer or early fall in temperate climates.

The incubation period for scarlet fever ranges from 12 hours to 7 days. Patients are contagious
during the acute illness and during the subclinical phase.

Epidemiology
As many as 10% of the population contracts group A streptococcal pharyngitis. Of this group, as
many as 10% then develop scarlet fever.

In the past century, the number of cases of scarlet fever has remained high, with marked decrease
in case-mortality rates secondary to widespread use of antibiotics. Transmission usually occurs via
airborne respiratory particles that can be spread from infected patients and asymptomatic carriers.

The infection rate increases in overcrowded situations (eg, schools, institutional settings) and it
peaks during late fall, winter, and spring in temperate environments. Immunity, which is type
specific, may be induced by a carrier state or overt infection. In adulthood, incidence decreases
markedly as immunity develops to the most prevalent serotypes. Complications (eg, rheumatic
fever) are more common in recent immigrants to the United States.

Scarlet fever predominantly occurs in children aged 1-10 years, though it can also occur in older
children and adults. By the time children are 10 years old, 80% have developed lifelong protective
antibodies against streptococcal pyrogenic exotoxins, which prevent future disease manifestation.
Scarlet fever is rare in children younger than 1 year because of the presence of maternal
antiexotoxin antibodies and lack of prior sensitization.

Leslie et al suggest from a case-control study that antecedent streptococcal infection can increase
the likelihood of children developing certain neuropsychiatric disorders, including Tourette
syndrome, attention-deficit/hyperactivity disorder, and major depressive disorder. [7]
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11/28/2017 Scarlet Fever: Background, Pathophysiology, Etiology

Males and females are affected equally. No racial or ethnic predilection is reported for group A
streptococcal infection.

Prognosis
When the condition is identified in a timely fashion, the prognosis is excellent. Most patients
recover fully after 4-5 days, with resolution of skin symptoms over several weeks. Attacks may
recur.

In the preantibiotic era, infections due to GABHS were major causes of mortality and morbidity.
Historically, scarlet fever resulted in death in 15-20% of those affected. However, scarlet fever is no
longer associated with the deadly epidemics that made it so feared in the 1800s. Since the advent
of antibiotic therapy, the mortality rate for scarlet fever has been less than 1%. However, school
outbreaks still occur owing to significant close proximity of susceptible children in a limited and
confined area, which can lasts for weeks.

Today, as a result not only of antibiotic therapy but also of enhanced immune status of the
population and improved socioeconomic conditions, scarlet fever usually follows a benign course.
Any undue morbidity and mortality are more likely to arise from suppurative complications (eg,
peritonsillar abscess, sinusitis, bronchopneumonia, and meningitis) or problems associated with
immune-mediated sequelae, rheumatic fever, or glomerulonephritis. Very rare complications, such
as septic shock with multisystem organ failure, have been reported. [8]

Known complications, such as septicemia, vasculitis, hepatitis, or rheumatic fever, should be

considered on a case-by-case basis as determined by the presence of clinical history and
examination findings suggestive of those diseases. [9, 10] Localized soft tissue infections may
suggest the presence of underlying osteomyelitis, but scarlet fever may occur from cellulitis alone.
[11] When scarlet fever has been determined to be due to a soft tissue infection over or near bone,
evaluation for bony involvement should be considered.

Patient Education
Patients must be instructed to complete the entire course of antibiotics, even if symptoms resolve.
They should be advised to follow general good hygiene precautions, especially in households with
other small children.

Patients should be warned that they will have generalized exfoliation over the next 2 weeks. In
particular, they should be warned about signs of complications of streptococcal infection, such as
persistent fever, increased throat or sinus pain, and generalized swelling.

For patient education resources, see the Children’s Health Center and the Ear, Nose, and Throat
Center, as well as Strep Throat and Skin Rashes in Children.

Clinical Presentation

References

1. Dong H, Xu G, Li S, Song Q, Liu S, Lin H, et al. Beta-haemolytic group A streptococci

emm75 carrying altered pyrogenic exotoxin A linked to scarlet fever in adults. J Infect. 2008
Apr. 56(4):261-7. [Medline].

2. Yang SG, Dong HJ, Li FR, Xie SY, Cao HC, Xia SC, et al. Report and analysis of a scarlet
fever outbreak among adults through food-borne transmission in China. J Infect. 2007 Nov.
55(5):419-24. [Medline].

3. Brinker A. Scarlet Fever. N Engl J Med. 2017 May 18. 376 (20):1972. [Medline].
https://emedicine.medscape.com/article/1053253-overview#a5 3/8
11/28/2017 Scarlet Fever: Background, Pathophysiology, Etiology

4. Andrey DO, Posfay-Barbe KM. Re-emergence of scarlet fever: old players return?. Expert
Rev Anti Infect Ther. 2016 Aug. 14 (8):687-9. [Medline].

5. Park DW, Kim SH, Park JW, Kim MJ, Cho SJ, Park HJ, et al. Incidence and Characteristics of
Scarlet Fever, South Korea, 2008-2015. Emerg Infect Dis. 2017 Apr. 23 (4):658-661.
[Medline].

6. Cunningham MW. Pathogenesis of group A streptococcal infections. Clin Microbiol Rev. 2000
Jul. 13(3):470-511. [Medline]. [Full Text].

7. Leslie DL, Kozma L, Martin A, Landeros A, Katsovich L, King RA, et al. Neuropsychiatric
disorders associated with streptococcal infection: a case-control study among privately
insured children. J Am Acad Child Adolesc Psychiatry. 2008 Oct. 47(10):1166-72. [Medline].
[Full Text].

8. Sandrini J, Beucher AB, Kouatchet A, Lavigne C. [Scarlet fever with multisystem organ failure
and hypertrophic gastritis]. Rev Med Interne. 2009 May. 30(5):456-9. [Medline].

9. Gómez-Carrasco JA, Lassaletta A, Ruano D. [Acute hepatitis may form part of scarlet fever].
An Pediatr (Barc). 2004 Apr. 60(4):382-3. [Medline].

10. Güven A. Hepatitis and hematuria in scarlet fever. Indian J Pediatr. 2002 Nov. 69(11):985-6.
[Medline].

11. Lau SK, Woo PC, Yuen KY. Toxic scarlet fever complicating cellulitis: early clinical diagnosis
is crucial to prevent a fatal outcome. New Microbiol. 2004 Apr. 27(2):203-6. [Medline].

12. Finnish Medical Society Duodecim. Sore throat and tonsillitis. EBM Guidelines. Evidence-
Based Medicine. Feb 2 2007Helsinki, Finland: Wiley Interscience. John Wiley & Sons. [Full
Text].

13. Gidaris D, Zafeiriou D, Mavridis P, Gombakis N. Scarlet Fever and hepatitis: a case report.
Hippokratia. 2008 Jul. 12(3):186-7. [Medline]. [Full Text].

14. Reddy UP, Albini TA, Banta JT, Davis JL. Post-streptococcal vasculitis. Ocul Immunol
Inflamm. 2008 Jan-Feb. 16(1):35-6. [Medline].

15. Wilson PF, Wannemuehler TJ, Matt BH. Invasive group A Streptococcus resulting in sepsis
and abdominal wall abscess after adenotonsillectomy. Laryngoscope. 2015 May. 125
(5):1230-2. [Medline].

16. Warnier H, Depuis Z, Nyamugabo K, Desprechins B, Seghaye MC. [An rare complication of
scarlet fever : invasive group A streptococcal infection with streptococcal toxic shock
syndrome]. Rev Med Liege. 2017 Mar. 72 (3):132-137. [Medline].

17. Gaston DA, Zurowski SM. Arcanobacterium haemolyticum pharyngitis and exanthem. Three
case reports and literature review. Arch Dermatol. 1996 Jan. 132(1):61-4. [Medline].

18. Sanz JC, Bascones Mde L, Martín F, Sáez-Nieto JA. [Recurrent scarlet fever due to recent
reinfection caused by strains unrelated to Streptococcus pyogenes]. Enferm Infecc Microbiol
Clin. 2005 Jun-Jul. 23(6):388-9. [Medline].

19. Gerber MA, Baltimore RS, Eaton CB, Gewitz M, Rowley AH, Shulman ST, et al. Prevention of
rheumatic fever and diagnosis and treatment of acute Streptococcal pharyngitis: a scientific
statement from the American Heart Association Rheumatic Fever, Endocarditis, and
Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the
Interdisciplinary Council on Functional Genomics and Translational Biology, and the
Interdisciplinary Council on Quality of Care and Outcomes Research: endorsed by the
American Academy of Pediatrics. Circulation. 2009 Mar 24. 119(11):1541-51. [Medline].

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11/28/2017 Scarlet Fever: Background, Pathophysiology, Etiology

20. Gerber MA, Shulman ST. Rapid diagnosis of pharyngitis caused by group A streptococci. Clin
Microbiol Rev. 2004 Jul. 17(3):571-80, table of contents. [Medline]. [Full Text].

21. Bass JW. Antibiotic management of group A streptococcal pharyngotonsillitis. Pediatr Infect
Dis J. 1991 Oct. 10(10 Suppl):S43-9. [Medline].

22. Derrick CW, Dillon HC. Erythromycin therapy for streptococcal pharyngitis. Am J Dis Child.
1976 Feb. 130(2):175-8. [Medline].

23. Stock I. [Streptococcus pyogenes--much more than the aetiological agent of scarlet fever].
Med Monatsschr Pharm. 2009 Nov. 32(11):408-16; quiz 417-8. [Medline].

Media Gallery

The exudative pharyngitis typical of scarlet fever. Although the tongue is somewhat out of
focus, the whitish coating observed early in scarlet fever is visible.

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Contributor Information and Disclosures

Author

Bahman Sotoodian, MD Resident Physician, Department of Dermatology, University of Alberta

Faculty of Medicine and Dentistry, Canada

Disclosure: Nothing to disclose.

Coauthor(s)

Jaggi Rao, MD, FRCPC Clinical Professor of Medicine, Division of Dermatology and Cutaneous
Sciences, Director of Dermatology Residency Program, University of Alberta Faculty of Medicine
and Dentistry

Jaggi Rao, MD, FRCPC is a member of the following medical societies: American Academy of
Dermatology, American Society for Dermatologic Surgery, American Society for Laser Medicine
and Surgery, Canadian Medical Association, Pacific Dermatologic Association, Royal College of
Physicians and Surgeons of Canada, Canadian Medical Protective Association, Canadian
Dermatology Association

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency

Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of

Dermatology, Society for Investigative Dermatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for:
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11/28/2017 Scarlet Fever: Background, Pathophysiology, Etiology

Naked Biome<br/>Received income in an amount equal to or greater than $250 from: Elsevier;
WebMD<br/>StatPearls; Editor.

Additional Contributors

Edward J Zabawski, Jr, DO Medical and Surgical Dermatology

Edward J Zabawski, Jr, DO is a member of the following medical societies: American Osteopathic
Association, New England Dermatological Society

Disclosure: Nothing to disclose.

Acknowledgements

Jerry Balentine, DO Professor and Chair of Emergency Medicine, New York Institute of
Technology College of Osteopathic Medicine; Executive Vice President, St Barnabas Hospital

Jerry Balentine, DO is a member of the following medical societies: American College of

Emergency Physicians, American College of Osteopathic Emergency Physicians, American
College of Physician Executives, American Osteopathic Association, and New York Academy of
Medicine

Disclosure: Nothing to disclose.

Peter Bloomfield, MD, MPH Clinical Instructor, Department of Emergency Medicine, Olive View-
UCLA Medical Center

Disclosure: Nothing to disclose.

Pamela L Dyne, MD Professor of Clinical Medicine/Emergency Medicine, University of California,

Los Angeles, David Geffen School of Medicine; Attending Physician, Department of Emergency
Medicine, Olive View-UCLA Medical Center

Pamela L Dyne, MD is a member of the following medical societies: American Academy of

Emergency Medicine, American College of Emergency Physicians, and Society for Academic
Emergency Medicine

Disclosure: Nothing to disclose.

Craig A Elmets, MD Professor and Chair, Department of Dermatology, Director, UAB Skin
Diseases Research Center, University of Alabama at Birmingham School of Medicine

Craig A Elmets, MD is a member of the following medical societies: American Academy of

Dermatology, American Association of Immunologists, American College of Physicians, American
Federation for Medical Research, and Society for Investigative Dermatology

Disclosure: Palomar Medical Technologies Stock None; Astellas Consulting fee Review panel
membership; Massachusetts Medical Society Salary Employment; Abbott Laboratories
Grant/research funds Independent contractor; UpToDate Salary Employment; Biogen
Grant/research funds Independent contractor; Clinuvel Independent contractor; Covan Basilea
Pharmaceutical Grant/research funds Independent contractor; ISDIN None Consulting; TenX
BIopharma Grant/research funds Independent contractor

Daniel P Lombardi, DO Clinical Assistant Professor, New York College of Osteopathic Medicine;
Attending Physician, Associate Department Director and Program Director, Department of
Emergency Medicine, St Barnabas Hospital

Daniel P Lombardi, DO is a member of the following medical societies: American College of

Emergency Physicians, American College of Osteopathic Emergency Physicians, and American

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Osteopathic Association

Disclosure: Nothing to disclose.

Robert E O'Connor, MD, MPH Professor and Chair, Department of Emergency Medicine,
University of Virginia Health System

Robert E O'Connor, MD, MPH is a member of the following medical societies: American Academy
of Emergency Medicine, American College of Emergency Physicians, American College of
Physician Executives, American Heart Association, American Medical Association, Medical Society
of Delaware, National Association of EMS Physicians, Society for Academic Emergency Medicine,
and Wilderness Medical Society

Disclosure: Nothing to disclose.

Joseph A Salomone III, MD Associate Professor and Attending Staff, Truman Medical Centers,
University of Missouri-Kansas City School of Medicine; EMS Medical Director, Kansas City,
Missouri

Joseph A Salomone III, MD is a member of the following medical societies: American Academy of
Emergency Medicine, National Association of EMS Physicians, and Society for Academic
Emergency Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical
Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech

University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain
View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of

Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas
Medical Association

Disclosure: Nothing to disclose.

Eric L Weiss, MD, DTM&H Medical Director, Office of Service Continuity and Disaster Planning,
Fellowship Director, Stanford University Medical Center Disaster Medicine Fellowship, Chairman,
SUMC and LPCH Bioterrorism and Emergency Preparedness Task Force, Clinical Associate
Progressor, Department of Surgery (Emergency Medicine), Stanford University Medical Center

Eric L Weiss, MD, DTM&H is a member of the following medical societies: American College of
Emergency Physicians, American College of Occupational and Environmental Medicine, American
Medical Association, American Society of Tropical Medicine and Hygiene, Physicians for Social
Responsibility, Southeastern Surgical Congress, Southern Association for Oncology, Southern
Clinical Neurological Society, and Wilderness Medical Society

Disclosure: Nothing to disclose.

Garry Wilkes, MBBS, FACEM Director of Emergency Medicine, Calvary Hospital, Canberra, ACT;
Adjunct Associate Professor, Edith Cowan University, Western Australia

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center
College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
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Disclosure: Nothing to disclose.

Grace M Young, MD Associate Professor, Department of Pediatrics, University of Maryland

Medical Center

Grace M Young, MD is a member of the following medical societies: American Academy of

Pediatrics and American College of Emergency Physicians

Disclosure: Nothing to disclose.

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