Introduction
Background
Scarlet fever (known as scarlatina in older literature references) is an exotoxin-mediated disease arising from group A beta-hemolytic streptococcal infection. Ordinarily, scarlet fever evolves from a tonsillar/pharyngeal focus, although the rash develops in fewer 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 systemic rash of scarlet fever to the uncommon but highly lethal streptococcal toxic shock syndrome.
Pathophysiology
Usually, the sites of group A beta-hemolytic streptococcal 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).
Group A beta-hemolytic streptococci secrete a number of toxins, enzymes, and erythrogenic toxins. Release of erythrogenic toxin causes the pathognomonic rash of scarlet fever. Local lesions reveal a characteristic inflammatory reaction, specifically hyperemia, edema, and polymorphonuclear cell infiltration.
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.
Frequency
United States
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). 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.
Mortality/Morbidity
Scarlet fever is no longer associated with the deadly epidemics that made it so feared in the 1800s.
- Today, scarlet fever infection usually follows a benign course, and any undue morbidity and mortality are more likely to arise from suppurative complications, such as 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.1
- Risk of acute rheumatic fever following an untreated streptococcal infection has been estimated at 3% in epidemic situations and approximately 0.3% in endemic scenarios.
- If a nephritogenic strain of group A beta-hemolytic streptococci causes infection, the individual has a 10-15% chance of developing glomerulonephritis. A lethal form of streptococcal infection is capable of producing the toxic streptococcal syndrome.
Sex
- Males and females are affected equally.
Age
- Peak incidence of scarlet fever occurs in children aged 4-8 years.
- By the time children are 10-years-old, 80% have developed lifelong protective antibodies against streptococcal pyrogenic exotoxins.
- Scarlet fever is rare in children younger than 2 years because of the presence of maternal antiexotoxin antibodies and lack of prior sensitization.
Clinical
History
- Scarlet fever generally has a 1- to 4-day incubation period.
- Emergence of the illness tends to be abrupt, usually heralded by sudden onset of fever associated with sore throat, headache, nausea, vomiting, abdominal pain, myalgias, and malaise.
- The characteristic rash appears 12-48 hours after onset of fever.
- In the untreated patient, fever peaks by the second day (temperature as high as 103-104°F) and gradually returns to normal in 5-7 days.
- Fever abates within 12-24 hours after initiation of antibiotic therapy.
- Recent history of exposure to another individual with a "strep" infection may aid in the diagnosis.
Physical
- Exudative tonsillitis preceding scarlet fever often is accompanied by erythematous oral mucous membranes, along with petechiae and punctate red macules on the hard and soft palate and uvula (ie, Forchheimer spots).
- On day 1 or 2, a white coating covers the dorsum of the tongue with reddened papillae projecting through, giving rise to the white strawberry tongue. This white coating is shown in the image below.
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.
- By day 4 or 5, the white coating disappears, revealing the representative raspberry tongue.
- Generally, the rash develops 12-48 hours after the onset of fever, first appearing as erythematous patches below the ears, chest, and axilla.
- Dissemination to the trunk and extremities occurs over 24 hours.
- Typically, the rash consists of scarlet macules over generalized erythema (boiled lobster appearance).
- As the skin lesions evolve and become more diffuse, they turn punctate and resemble a sunburn with goose pimples.
- Numerous punctate lesions the size of pinheads give the skin a rough sandpaperlike texture.
- Lesions tend to be accentuated in the skin folds, particularly in the region of the neck, axilla, antecubital fossae, and inguinal and popliteal creases.
- Rupture of fragile capillaries at these sites displays linear arrays of petechiae (ie, Pastia lines) that may persist for 1-2 days after resolution of the generalized rash.
- Another distinctive facial finding is circumoral pallor.
- In severe disease, small vesicular lesions termed miliary sudamina may appear on the abdomen, hands, and feet.
- Mitigation of the exanthem occurs in approximately 1 week.
- Desquamation, shown in the image below, one of the most distinctive features of scarlet fever, begins 7-10 days after resolution of the rash and may continue up to 6 weeks.
Desquamation of the palms is a frequently observed self-limited manifestation of scarlet fever present in the healing period following resolution of the infection and acute eruption.
- Peeling of the skin is most prominent in the axilla, groin, and tips of the fingers and toes.
- Extent and duration of desquamation is directly proportional to initial intensity of the rash.
Causes
Infection of group A beta-hemolytic streptococci causes scarlet fever.
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| Follow-up: Scarlet Fever |
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References
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Further Reading
Keywords
scarlet fever, scarlatina, group A beta-hemolytic streptococci, group A streptococci, strep throat, bullous impetigo, streptococcal toxic shock syndrome, toxic streptococcal syndrome, surgical scarlet fever, puerperal scarlet fever, rheumatic fever
peritonsillar abscess, sinusitis, bronchopneumonia, meningitis, glomerulonephritis, hepatitis, vasculitis, uveitis, Forchheimer spots, white strawberry tongue, raspberry tongue, Pastialines
