Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Staphylococcal Scalded Skin Syndrome

  • Author: Randall W King, MD, FACEP; Chief Editor: Jeter (Jay) Pritchard Taylor, III, MD  more...
 
Updated: Jun 15, 2016
 

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]

Next

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.

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).

Previous
Next

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]

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]

Previous
 
 
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)

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, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

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.

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

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: 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.

References
  1. Hubiche T, Bes M, Roudiere L, Langlaude F, Etienne J, Del Giudice P. Mild staphylococcal scalded skin syndrome: an underdiagnosed clinical disorder. Br J Dermatol. 2012 Jan. 166(1):213-5. [Medline].

  2. Adhisivam B, Mahadevan S. Abscess of the nasal septum with staphylococcal scalded skin syndrome. Indian Pediatr. 2006 Apr. 43(4):372-3. [Medline].

  3. Amagai M, Yamaguchi T, Hanakawa Y, Nishifuji K, Sugai M, Stanley JR. Staphylococcal exfoliative toxin B specifically cleaves desmoglein 1. J Invest Dermatol. 2002 May. 118(5):845-50. [Medline].

  4. Hanakawa Y, Stanley JR. Mechanisms of blister formation by staphylococcal toxins. J Biochem. 2004 Dec. 136(6):747-50. [Medline].

  5. Ladhani S. Understanding the mechanism of action of the exfoliative toxins of Staphylococcus aureus. FEMS Immunol Med Microbiol. 2003 Nov 28. 39(2):181-9. [Medline].

  6. Patel GK, Finlay AY. Staphylococcal scalded skin syndrome: diagnosis and management. Am J Clin Dermatol. 2003. 4(3):165-75. [Medline].

  7. Rydzewska-Rosolowska A, Brzosko S, Borawski J, Mysliwiec M. Staphylococcal scalded skin syndrome in the course of lupus nephritis. Nephrology (Carlton). 2008 Jun. 13(3):265-6. [Medline].

  8. Murono K, Fujita K, Yoshioka H. Microbiologic characteristics of exfoliative toxin-producing Staphylococcus aureus. Pediatr Infect Dis J. 1988 May. 7(5):313-5. [Medline].

  9. Hayward A, Knott F, Petersen I, Livermore DM, Duckworth G, Islam A. Increasing hospitalizations and general practice prescriptions for community-onset staphylococcal disease, England. Emerg Infect Dis. 2008 May. 14(5):720-6. [Medline]. [Full Text].

  10. Decleire PY, Blondiaux G, Delaere B, Glupczynski Y. Staphyloccocal scalded skin syndrome in an adult. Acta Clin Belg. 2004 Nov-Dec. 59(6):365-8. [Medline].

  11. Oono T, Kanzaki H, Yoshioka T, Arata J. Staphylococcal scalded skin syndrome in an adult. Identification of exfoliative toxin A and B genes by polymerase chain reaction. Dermatology. 1997. 195(3):268-70. [Medline].

  12. Shirin S, Gottlieb AB, Stahl EB. Staphylococcal scalded skin syndrome in an immunocompetent adult: possible implication of low-dosage prednisone. Cutis. 1998 Nov. 62(5):223-4. [Medline].

  13. Wong GW, Oppenheimer SJ, Evans RM, Leung SS, Cheng JC. Pyomyositis and staphylococcal scalded skin syndrome. Acta Paediatr. 1993 Jan. 82(1):113-5. [Medline].

  14. Norbury WB, Gallagher JJ, Herndon DN, Branski LK, Oehring PE, Jeschke MG. Neonate twin with staphylococcal scalded skin syndrome from a renal source. Pediatr Crit Care Med. 2010 Mar. 11(2):e20-3. [Medline].

  15. Kadam S, Tagare A, Deodhar J, Tawade Y, Pandit A. Staphylococcal scalded skin syndrome in a neonate. Indian J Pediatr. 2009 Oct. 76(10):1074. [Medline].

  16. Kapoor V, Travadi J, Braye S. Staphylococcal scalded skin syndrome in an extremely premature neonate: a case report with a brief review of literature. J Paediatr Child Health. 2008 Jun. 44(6):374-6. [Medline].

  17. O'Connell NH, Mannix M, Philip RK, MacDonagh-White C, Slevin B, Monahan R, et al. Infant Staphylococcal scalded skin syndrome, Ireland, 2007--preliminary outbreak report. Euro Surveill. 2007 Jun 14. 12(6):E070614.5. [Medline].

  18. Duijsters CE, Halbertsma FJ, Kornelisse RF, Arents NL, Andriessen P. Recurring staphylococcal scalded skin syndrome in a very low birth weight infant: a case report. J Med Case Reports. 2009. 3:7313. [Medline]. [Full Text].

  19. El Helali N, Carbonne A, Naas T, Kerneis S, Fresco O, Giovangrandi Y, et al. Nosocomial outbreak of staphylococcal scalded skin syndrome in neonates: epidemiological investigation and control. J Hosp Infect. 2005 Oct. 61(2):130-8. [Medline].

  20. Brewer JD, Hundley MD, Meves A, Hargreaves J, McEvoy MT, Pittelkow MR. Staphylococcal scalded skin syndrome and toxic shock syndrome after tooth extraction. J Am Acad Dermatol. 2008 Aug. 59(2):342-6. [Medline].

  21. Moss C, Gupta E. The Nikolsky sign in staphylococcal scalded skin syndrome. Arch Dis Child. 1998 Sep. 79(3):290. [Medline].

  22. Uzun S, Durdu M. The specificity and sensitivity of Nikolskiy sign in the diagnosis of pemphigus. J Am Acad Dermatol. 2006 Mar. 54(3):411-5. [Medline].

  23. Acland KM, Darvay A, Griffin C, Aali SA, Russell-Jones R. Staphylococcal scalded skin syndrome in an adult associated with methicillin-resistant Staphylococcus aureus. Br J Dermatol. 1999 Mar. 140(3):518-20. [Medline].

  24. Ito Y, Funabashi Yoh M, Toda K, Shimazaki M, Nakamura T, Morita E. Staphylococcal scalded-skin syndrome in an adult due to methicillin-resistant Staphylococcus aureus. J Infect Chemother. 2002 Sep. 8(3):256-61. [Medline].

  25. Noguchi N, Nakaminami H, Nishijima S, Kurokawa I, So H, Sasatsu M. Antimicrobial agent of susceptibilities and antiseptic resistance gene distribution among methicillin-resistant Staphylococcus aureus isolates from patients with impetigo and staphylococcal scalded skin syndrome. J Clin Microbiol. 2006 Jun. 44(6):2119-25. [Medline].

  26. Kaplan SL, Deville JG, Yogev R, Morfin MR, Wu E, Adler S, et al. Linezolid versus vancomycin for treatment of resistant Gram-positive infections in children. Pediatr Infect Dis J. 2003 Aug. 22 (8):677-86. [Medline].

  27. Kimberlin DW, Brady MT, Jackson MA, Long SS. Staphylococcal infections. American Academy of Pediatrics Red Book. 30th. 2015. 22: 715.

  28. Al-Dhalimi MA. Neonatal and infantile erythroderma: a clinical and follow-up study of 42 cases. J Dermatol. 2007 May. 34(5):302-7. [Medline].

  29. Baartmans MG, Maas MH, Dokter J. Neonate with staphylococcal scalded skin syndrome. Arch Dis Child Fetal Neonatal Ed. 2006 Jan. 91(1):F25. [Medline]. [Full Text].

  30. Chi CY, Wang SM, Lin HC, Liu CC. A clinical and microbiological comparison of Staphylococcus aureus toxic shock and scalded skin syndromes in children. Clin Infect Dis. 2006 Jan 15. 42(2):181-5. [Medline].

  31. Cribier B, Piemont Y, Grosshans E. Staphylococcal scalded skin syndrome in adults. A clinical review illustrated with a new case. J Am Acad Dermatol. 1994 Feb. 30(2 Pt 2):319-24. [Medline].

  32. El Helali N, Carbonne A, Naas T, et al. Nosocomial outbreak of staphylococcal scalded skin syndrome in neonates: epidemiological investigation and control. J Hosp Infect. 2005 Oct. 61(2):130-8. [Medline].

  33. Ginsburg CM. Staphylococcal toxin syndromes. Pediatr Infect Dis J. 1991 Apr. 10(4):319-21. [Medline].

  34. Greenwood JE, Dunn KW, Davenport PJ. Experience with severe extensive blistering skin disease in a paediatric burns unit. Burns. 2000 Feb. 26(1):82-7. [Medline].

  35. Hedrick J. Acute bacterial skin infections in pediatric medicine: current issues in presentation and treatment. Paediatr Drugs. 2003. 5 Suppl 1:35-46. [Medline].

  36. Hochman MA, Mayers M. Stevens-Johnson syndrome, epidermolysis bullosa, staphylococcal scalded skin syndrome, and dermatitis herpetiformis. Int Ophthalmol Clin. 1997 Spring. 37(2):77-92. [Medline].

  37. Johnston GA. Treatment of bullous impetigo and the staphylococcal scalded skin syndrome in infants. Expert Rev Anti Infect Ther. 2004 Jun. 2(3):439-46. [Medline].

  38. Ladhani S, Evans RW. Staphylococcal scalded skin syndrome. Arch Dis Child. 1998 Jan. 78(1):85-8. [Medline].

  39. Ladhani S, Joannou CL, Lochrie DP, Evans RW, Poston SM. Clinical, microbial, and biochemical aspects of the exfoliative toxins causing staphylococcal scalded-skin syndrome. Clin Microbiol Rev. 1999 Apr. 12(2):224-42. [Medline].

  40. Lina G, Gillet Y, Vandenesch F, Jones ME, Floret D, Etienne J. Toxin involvement in staphylococcal scalded skin syndrome. Clin Infect Dis. 1997 Dec. 25(6):1369-73. [Medline].

  41. Payne AS, Hanakawa Y, Amagai M, Stanley JR. Desmosomes and disease: pemphigus and bullous impetigo. Curr Opin Cell Biol. 2004 Oct. 16(5):536-43. [Medline].

  42. Prevost G, Couppie P, Monteil H. Staphylococcal epidermolysins. Curr Opin Infect Dis. 2003 Apr. 16(2):71-6. [Medline].

  43. Resnick SD. Staphylococcal toxin-mediated syndromes in childhood. Semin Dermatol. 1992 Mar. 11(1):11-8. [Medline].

  44. Rodriguez Prieto MA, Manchado Lopez P, Ruiz Gonzalez I. Toxic epidermal necrolysis associated with scalded skin syndrome in an adult. Int J Dermatol. 1997 Nov. 36(11):875-6. [Medline].

  45. Shelley ED, Shelley WB, Talanin NY. Chronic staphylococcal scalded skin syndrome. Br J Dermatol. 1998 Aug. 139(2):319-24. [Medline].

  46. Veien NK. The clinician's choice of antibiotics in the treatment of bacterial skin infection. Br J Dermatol. 1998 Dec. 139 Suppl 53:30-6. [Medline].

  47. Whittock NV, Bower C. Targetting of desmoglein 1 in inherited and acquired skin diseases. Clin Exp Dermatol. 2003 Jul. 28(4):410-5. [Medline].

  48. Williams RE, MacKie RM. The staphylococci. Importance of their control in the management of skin disease. Dermatol Clin. 1993 Jan. 11(1):201-6. [Medline].

  49. Wooldridge WE. Managing skin infections in children. Postgrad Med. 1991 Mar. 89(4):109-12. [Medline].

  50. Yamasaki O, Yamaguchi T, Sugai M, et al. Clinical manifestations of staphylococcal scalded-skin syndrome depend on serotypes of exfoliative toxins. J Clin Microbiol. 2005 Apr. 43(4):1890-3. [Medline].

 
Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.