eMedicine Specialties > Emergency Medicine > Infectious Diseases

Staphylococcal Scalded Skin Syndrome

Author: Randall W King, MD, Assistant Clinical Professor of Emergency Medicine, The University of Toledo College of Medicine; Program Director Emergency Medicine Residency, Associate Chair, Department of Emergency Medicine, St Vincent Mercy Medical Center
Coauthor(s): Paul R de Saint Victor, MD, FACEP, Chief of Staff, St Vincent Mercy Medical Center, Toledo
Contributor Information and Disclosures

Updated: Jun 18, 2009

Introduction

Background

Staphylococcal scalded skin syndrome (SSSS), also known as Ritter von Ritterschein disease (in newborns), Ritter disease, and staphylococcal epidermal necrolysis, encompasses a spectrum of superficial blistering skin disorders caused by the exfoliative toxins of some strains of Staphylococcus aureus.

It is a syndrome of acute exfoliation of the skin typically following an erythematous cellulitis. Severity of staphylococcal scalded skin syndrome varies from a few blisters localized to the site of infection to a severe exfoliation affecting almost the entire body.

Pathophysiology

Staphylococcal scalded skin syndrome (SSSS) is caused by an exfoliative toxin produced by roughly 5% of Staphylococcus aureus. As the syndrome evolves, an initial infection occurs, commonly at a site such as the oral or nasal cavities, throat, or umbilicus.1  Epidermolytic toxins are produced by the infecting Staphylococcus species; these toxins act at a remote site leading to a red rash and separation of the epidermis beneath the granular cell layer. Bullae form, and diffuse sheetlike desquamation occurs. Two types of staphylococcal scalded skin syndrome are thought to exist: a localized form, in which there is only patchy involvement of the epidermis, and a generalized form, in which significant areas of are involved, remote from the initial site of infection. 

Two exfoliative toxins (ETA and ETB) have been isolated and characterized, but the exact mechanism by which they cause exfoliation had until recently been uncertain. The toxins likely act as proteases that target the protein desmoglein-1 (DG-1), an important cell-to-cell attachment protein found only in the superficial epidermis.2,3,4 The relative quantity of DG-1 in the skin differs with age and may partially explain the increased frequency of staphylococcal scalded skin syndrome in children younger than 5 years. It is theorized that immature renal function in this age group may contribute to impaired clearance of circulating exotoxins, contributing to more extensive disease. Another theory suggests that the exfoliative toxins may possess a superantigenic activity.

The decrease in frequency of staphylococcal scalded skin syndrome (SSSS) in adults is thought to be explained by the presence of antibodies specific for exotoxins and also improved renal clearance of toxins that are produced. 

Initial studies suggested that phage lytic group II S aureus (subtypes 3A, 3B, 3C, 55 and 71) were solely responsible for exfoliative toxin production, but it is now known that all phage groups are able to produce exfoliative toxin and cause staphylococcal scalded skin syndrome. 

Staphylococcal scalded skin syndrome differs from bullous impetigo. Both are blistering skin diseases caused by staphylococcal exfoliative toxin. However, in bullous impetigo, the exfoliative toxins are restricted to the area of infection, and bacteria can be cultured from the blister contents. In staphylococcal scalded skin syndrome, the exfoliative toxins are spread hematogenously from a localized source potentially causing epidermal damage at distant sites.

Staphylococcal scalded skin syndrome differs from the more severe toxic epidermal necrolysis (TEN), in that the cleavage site in staphylococcal scalded skin syndrome is intraepidermal, as opposed to TEN, which involves necrosis of the full epidermal layer (at the level of the basement membrane).

Frequency

United States

Staphylococcal scalded skin syndrome (SSSS) is most common in children and neonates. Staphylococcal scalded skin syndrome is rarer in adults, but it has been described in adults with renal failure, immunologic deficiency, and other chronic illness.5

International

Internationally, predominance is in children as well. Overall incidence is higher in developing countries and wherever the incidence of staphylococcal infections is higher. Additionally, some geographic difference exists in the incidence of staphylococcal strains and the types of exotoxins produced.6

Mortality/Morbidity

The mortality rate from staphylococcal scalded skin syndrome (SSSS) in children is very low (1-5%), unless associated sepsis or an underlying serious medical condition exists. The mortality rate in adults is higher (as high as 50-60%), although this may be a reflection of the underlying disorder, which increased susceptibility to SSSS, and not SSSS itself.7,8,9 Significant morbidity can result from hematologic or local spread of infection.10 Complications are usually the result of sepsis, superinfection, and dehydration or electrolyte imbalance due to denuded skin.

Sex

No gender predilection is documented in children. In adults, the male-to-female ratio is approximately 2:1.

Age

Staphylococcal scalded skin syndrome (SSSS) primarily is a disease of children.

  • Children are more at risk because of lack of immunity and immature renal clearance capability (exfoliative toxins are renally excreted). Maternal antibodies transferred to infants in breastmilk are thought to be partially protective, but neonatal disease can still occur possibly as a result of inadequate immunity or immature renal clearance of exotoxin. 
  • SSSS can occur individually or as outbreaks in nurseries.  Outbreaks are usually due to asymptomatic carriers who spread the disease to susceptible individuals. 
  • Most children (62%) are younger than 2 years, and almost all (98%) are younger than 6 years. 
  • SSSS is rare in adults, with fewer than 50 cases formally reported in the literature. Adults with SSSS are most often chronically ill, are immunocompromised, or have renal failure. SSSS can also appear in adults in cases with a high burden of staphylococcal infection where the quantity of exotoxin is significant.

Clinical

History

  • Staphylococcal scalded skin syndrome (SSSS) presents as a red rash followed by diffuse epidermal exfoliation. 
  • A prodromal localized S aureus infection of the skin, throat, nose, mouth, umbilicus, or GI tract occurs. Such an infection often is not apparent before the SSSS rash appears.
  • General malaise
  • Fever
  • Irritability
  • Skin tenderness

Physical

  • Fever, although patients may be afebrile
  • Tenderness to palpation
  • Warmth to palpation
  • Diffuse erythematous rash (see Media file 1)


Staphylococcal scalded skin syndrome. Photograph ...

Staphylococcal scalded skin syndrome. Photograph by David Effron, MD, FACEP.

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Staphylococcal scalded skin syndrome. Photograph ...

Staphylococcal scalded skin syndrome. Photograph by David Effron, MD, FACEP.

    • Often begins centrally
    • Sandpaperlike, progressing into a wrinkled appearance
    • Accentuated in flexor creases (see Media file 2)


Staphylococcal scalded skin syndrome. Photograph ...

Staphylococcal scalded skin syndrome. Photograph by David Effron, MD, FACEP.

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Staphylococcal scalded skin syndrome. Photograph ...

Staphylococcal scalded skin syndrome. Photograph by David Effron, MD, FACEP.


Staphylococcal scalded skin syndrome. Photograph ...

Staphylococcal scalded skin syndrome. Photograph by David Effron, MD, FACEP.

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Staphylococcal scalded skin syndrome. Photograph ...

Staphylococcal scalded skin syndrome. Photograph by David Effron, MD, FACEP.

    • Flaccid
    • Ill-defined
  • Nikolsky sign (gentle stroking of the skin causes the skin to separate at the epidermis)11,12
  • Exfoliation of skin, which may be patchy or sheetlike in nature (see Media files 4-6)
  • Facial edema
  • Perioral crusting
  • Most patients do not appear severely ill.
  • Dehydration may be present and significant.

Causes

  • Infection by group 2 phage S aureus (several types) leads to release of exotoxin.
    • Exotoxin is a protein and is classified as either type A or B. Most are type A.
    • Exotoxin causes separation of the epidermis beneath the granular cell layer.
    • Cases of staphylococcal scalded skin syndrome (SSSS) have been reported among infants who have breastfed from mothers with S aureus breast abscess.
    • A case has been reported of neonatal staphylococcal scalded skin syndrome secondary to maternal-fetal transmission at birth.
    • Outbreaks have been reported in neonatal and newborn nurseries.
    • Reports implicating MRSA and community-acquired methicillin Staphylococcus aureus (CA-MRSA) as a cause of staphylococcal scalded skin syndrome are increasing.13,14,15

More on Staphylococcal Scalded Skin Syndrome

Overview: Staphylococcal Scalded Skin Syndrome
Differential Diagnoses & Workup: Staphylococcal Scalded Skin Syndrome
Treatment & Medication: Staphylococcal Scalded Skin Syndrome
Follow-up: Staphylococcal Scalded Skin Syndrome
Multimedia: Staphylococcal Scalded Skin Syndrome
References
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References

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Further Reading

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Keywords

staphylococcal scalded skin syndrome, SSSS, blistering skin, Ritter von Ritterschein disease, Ritter disease, Ritter’s disease, Lyell disease, Lyell’s disease, skin disease, skin condition, treatment, symptoms, causes, exfoliative toxins, toxin-mediated syndrome, staphylococcal epidermal necrolysis, acute exfoliation of the skin, staphylococcal infection, erythematous cellulitis, phage group 2 Staphylococcus aureus, S aureus, epidermolytic toxins

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Contributor Information and Disclosures

Author

Randall W King, MD, Assistant Clinical Professor of Emergency Medicine, The University of Toledo College of Medicine; Program Director Emergency Medicine Residency, Associate Chair, Department of Emergency Medicine, St Vincent Mercy Medical Center
Randall W King, MD is a member of the following medical societies: American College of Emergency Physicians, American Medical Association, Council of Emergency Medicine Residency Directors, Ohio State Medical Association, and Society for Academic Emergency Medicine
Disclosure: Challenger corporation None Other

Coauthor(s)

Paul R de Saint Victor, MD, FACEP, Chief of Staff, St Vincent Mercy Medical Center, Toledo
Paul R de Saint Victor, MD, FACEP is a member of the following medical societies: American College of Emergency Physicians, American Heart Association, National Association of EMS Physicians, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Medical Editor

Daniel J Dire, MD, FACEP, FAAP, FAAEM, Clinical Associate Professor, Department of Emergency Medicine, University of Texas-Houston
Daniel J Dire, MD, FACEP, FAAP, FAAEM is a member of the following medical societies: American Academy of Clinical Toxicology, American Academy of Emergency Medicine, American Academy of Pediatrics, American College of Emergency Physicians, and Association of Military Surgeons of the US
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Jeter (Jay) Pritchard Taylor III, MD, Compliance Officer, Attending Physician, Emergency Medicine Residency, Department of Emergency Medicine, Palmetto Health Richland, University of South Carolina; Medical Director, Department of Emergency Medicine, Palmetto Health Baptist
Jeter (Jay) Pritchard Taylor III, MD is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, American Medical Association, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

Rick Kulkarni, MD, Medical Director, Assistant Professor of Surgery, Section of Emergency Medicine, Yale-New Haven Hospital
Rick Kulkarni, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, American Medical Association, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: WebMD Salary Employment

 
 
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