10/26/2017 Staphylococcal Scalded Skin Syndrome (SSSS): Background, Pathophysiology, Epidemiology

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Staphylococcal Scalded Skin Syndrome

(SSSS)
Updated: May 18, 2017
Author: Randall W King, MD, FACEP; Chief Editor: Jeter (Jay) Pritchard Taylor, III, MD more...

OVERVIEW

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. A mild form of the illness involving
desquamation of just the skin folds following impetigo has been described. [1]

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. [2] 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 keratinocyte cell-to-cell
attachment protein found only in the superficial epidermis. [3, 4, 5] 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.

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10/26/2017 Staphylococcal Scalded Skin Syndrome (SSSS): Background, Pathophysiology, Epidemiology

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. Therefore, cultures of the
bullous material are sterile.

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).

Epidemiology
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. [6, 7]

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. [8] Some recent reports show an increase in hospitalizations and prescriptions for
staphylococcal disease, including SSSS, in England. [9]

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. [10, 11, 12] Significant morbidity can
result from hematologic or local spread of infection. [13] 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. [14, 15, 16, 17] There is at least one
report of recurrent SSSS in a neonate. [18]

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SSSS can occur individually or as outbreaks in nurseries. Outbreaks are usually due to
asymptomatic carriers who spread the disease to susceptible individuals. [19]

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. There is also a case report of SSSS in an adult after tooth extraction. [20]

Clinical Presentation

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Media Gallery

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

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

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Contributor Information and Disclosures

Author

Randall W King, MD, FACEP Assistant Clinical Professor of Emergency Medicine, University of
Toledo College of Medicine; Director, Emergency Medicine Residency Program, Department of
Emergency Medicine, Chief Medical Information Officer, Chief of Staff Elect, Mercy St Vincent
Medical Center

Randall W King, MD, FACEP is a member of the following medical societies: American College of
Emergency Physicians, Council of Emergency Medicine Residency Directors, American Medical
Association, Ohio State Medical Association, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Heather Lyn Carone, MD Attending Physician, Department of Emergency Medicine, St Vincent

Mercy Medical Center

Heather Lyn Carone, MD is a member of the following medical societies: American College of
Emergency Physicians, American Medical Association, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

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

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10/26/2017 Staphylococcal Scalded Skin Syndrome (SSSS): Background, Pathophysiology, Epidemiology

Physicians, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Jeter (Jay) Pritchard Taylor, III, MD Assistant Professor, Department of Surgery, University of
South Carolina School of Medicine; Attending Physician, Clinical Instructor, Compliance Officer,
Department of Emergency Medicine, Palmetto Richland Hospital

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, Columbia Medical Society, Society for Academic Emergency Medicine, South
Carolina College of Emergency Physicians, South Carolina Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for:
Employed contractor - Chief Editor for Medscape.

Additional Contributors

Daniel J Dire, MD, FACEP, FAAP, FAAEM Clinical Professor, Department of Emergency
Medicine, University of Texas Medical School at Houston; Clinical Professor, Department of
Pediatrics, University of Texas Health Sciences Center San Antonio

Daniel J Dire, MD, FACEP, FAAP, FAAEM is a member of the following medical societies:
American Academy of Clinical Toxicology, American Academy of Pediatrics, American Academy of
Emergency Medicine, American College of Emergency Physicians, Association of Military
Surgeons of the US

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the medical review of this
article by Joseph U Becker, MD.

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