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American Journal of Clinical Dermatology

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01-03-2003 | Therapy in Practice

Staphylococcal Scalded Skin Syndrome

Diagnosis and Management

Authors: Dr Girish K. Patel, Andrew Y. Finlay

Published in: American Journal of Clinical Dermatology | Issue 3/2003

Abstract

Staphylococcal scalded skin syndrome (SSSS) is a common disorder that is usually seen in infants and children and rarely seen in adults. SSSS usually presents with a prodrome of sore throat or conjunctivitis. Extremely tender flaccid bullae, which are Nikolsky sign-positive, develop within 48 hours and commonly affect the flexures; occasionally, large areas of the skin may be involved. The bullae enlarge and rupture easily to reveal a moist erythematous base, which gives rise to the scalded appearance.

SSSS in adults is a rare disorder, though there are now over 50 documented cases. Usually SSSS occurs in predisposed individuals, but not all adults have an underlying illness. Whereas mortality in childhood SSSS is approximately 4%, the mortality rate in adults is reported to be greater than 60%.

SSSS is caused by an infection with a particular strain of Staphylococcus aureus, which leads to blistering of the upper layer of the skin, by the release of a circulating exotoxin. It has recently been demonstrated that the exfoliative exotoxin responsible for SSSS leads to the cleavage of desmoglein 1 complex, an important desmosomal protein. The same toxins that are responsible for causing SSSS also cause bullous impetigo. There appears to be a relationship between the disease extent, the amount of toxin produced and whether the toxin is released locally or systemically. As a result there is likely to be a spectrum of disease and there are likely to be a number of milder cases of adult SSSS that go undiagnosed.

Social improvements and hygiene have led to a dramatic fall in the number of cases of SSSS. Treatment is usually straightforward, when there is no coexistent morbidity and the presentation is mild, but can be demanding if the patient is particularly ill. SSSS is still associated with mortality, particularly when it occurs in adults.

Ritter von Rittershain G. Die exfoliativa dermatitis jüngerer Säulinge. Centralz Kinderheilk 1878; 2: 3–23

Lyell A. Toxic epidermal necrolysis: an eruption resembling scalding of the skin. Br J Dermatol 1956; 68: 355–361PubMedCrossRef

Lyell A, Dick HM, Alexander JO. Outbreak of toxic epidermal necrolysis associated with staphylococci. Lancet 1969; I: 787–789CrossRef

Lyell A. A review of toxic epidermal necrolysis in Britain. Br J Dermatol 1967; 79: 662–671PubMedCrossRef

Jefferson J. Lyell’s toxic epidermal necrolysis: a staphylococcal aetiology? BMJ 1967; 555: 802–804CrossRef

Tyson RG, Ushinski SC, Kisilevsky R. Toxic epidermal necrolysis (the scalded skin syndrome): its association in two cases with pathogenic staphylococci and its similarity in infancy to Ritter’s disease. Am J Dis Child 1966; 111: 386–392PubMed

Messaritakis J. Toxic epidermal necrolysis in children: description of 4 cases. Ann Paediatr 1966; 207 (4): 236–246PubMed

Melish ME, Glasgow LA. The staphylococcal scalded-skin syndrome. N Engl J Med 1970; 282: 1114–1119PubMedCrossRef

Kapral FA, Miller MM. Product of Staphylococcus aureus responsible for the scalded skin syndrome. Infect Immun 1971; 4: 541–545PubMed

10.

Melish ME, Glasgow LA, Turner MD. Staphylococcal scalded skin syndrome: isolation and partial characterisation of the exfoliative toxin. J Infect Dis 1972; 125: 129–140PubMedCrossRef

11.

Elias PM, Fritsch P, Mittermayer H, et al. Experimental staphylococcal toxic epidermal necrolysis (TEN) in adult humans and mice. J Lab Clin Med 1974; 84: 414–424PubMed

12.

Lyell A. The staphylococcal scalded skin syndrome a historical perspective: emergence of dermopathic strains of Staphylococcal aureus and the discovery of the epidermolytic toxin. J Am Acad Dermatol 1983; 9: 285–294PubMedCrossRef

13.

Levine G, Norden CW. Staphylococcal scalded skin syndrome in an adult. N Engl J Med 1972; 287: 1339–1340PubMedCrossRef

14.

Patel GK, Varma S, Finlay AY. Staphylococcal scalded skin syndrome in healthy adults. Br J Dermatol 2000; 142 (6): 1253–1255PubMedCrossRef

15.

Amagai M, Matsuyoshi N, Wang ZH, et al. Toxin in bullous impetigo and staphylococcal scalded skin syndrome targets desmoglein 1. Nature Med 2000; 6: 1275–1277PubMedCrossRef

16.

Elias PM, Fritsch P, Epstein EV. Staphylococcal scalded skin syndrome: clinical features, pathogenesis, and recent microbiological and biochemical developments. Arch Dermatol 1977; 113: 207–219PubMedCrossRef

17.

Elias PM, Levy SW. Bullous impetigo: occurrence of scalded skin syndrome in an adult. Arch Dermatol 1976; 112: 856–858PubMedCrossRef

18.

Falk DK, King LE. Criteria for the diagnosis of Staphylococcal scalded skin syndrome in adults. Cutis 1983; 31: 431–434

19.

Lowney ED, Baublis JV, Kreye GM, et al. The scalded skin syndrome in small children. Arch Dermatol 1967; 95: 359–369PubMedCrossRef

20.

Moss C, Gupta E. The Nikolsky sign in staphylococcal scalded skin syndrome [letter]. Arch Dis Child 1998; 79: 290PubMedCrossRef

21.

Shelley ED, Shelley WB, Talanin NY. Chronic staphylococcal scalded skin syndrome. Br J Dermatol 1998; 139: 319–324PubMedCrossRef

22.

Reid LH, Weston WL, Humbert JR. Staphylococcal scalded skin syndrome: adult patient with deficient cell mediated immunity. Arch Dermatol 1976; 112: 1275–1279CrossRef

23.

Fine JD, Harrist TJ, Radford MJ. Adult scalded skin syndrome fatally complicated by mixed gram-negative sepsis and cellulitis. Cutis 1981; 27: 162–167PubMed

24.

Norden CW, Mendelow H. Staphylococcal scalded skin syndrome in adults [letter]. N Engl J Med 1974; 290: 577PubMed

25.

Todd JK. Staphylococcal toxin syndromes. Annu Rev Med 1985; 36: 337–347PubMedCrossRef

26.

Amon RB, Dimond RL. Toxic epidermal necrolysis: rapid differentiation between staphylococcal- and drug-induced disease. Arch Dermatol 1975; 111: 1433–1437PubMedCrossRef

27.

Dimond RL, Wolff HH, Braun-Falco O. The staphylococcal scalded skin syndrome: an experimental histochemical and electron microscopy study. Br J Dermatol 1977; 96: 483–492PubMedCrossRef

28.

Robinson ND, Hashimoto T, Amagai M, et al. The new pemphigus variants. J Am Acad Dermatol 1999; 40: 649–671PubMedCrossRef

29.

Breathnach SM, McGibbon DH, Ives FE, et al. Carbamazepine (Tegretol) and toxic epidermal necrolysis: report of three cases with histopathological observations. Clin Exp Dermatol 1982; 7: 585–591PubMedCrossRef

30.

Gillespie WA, Pope RC, Simpson K. Pemphigus neonatorum caused by Staphylococcal aureus type 71. BMJ 1957; 1: 1044–1046PubMedCrossRef

31.

Willard D, Montell H, Piermont Y, et al. Exfoliatine dans les staphylococcies neonatales. Nouv Presse Med 1982; 11: 3769–3771PubMed

32.

Parker MT. Some cultural characteristics of Staphylococcus aureus strains from superficial skin infections. J Hyg (Lond) 1958; 56: 238–253CrossRef

33.

Arbuthnott JP, Kent K, Lyell A, et al. Toxic epidermal necrolysis produced by an extracellular product of Staphylococcus aureus. Br J Dermatol 1971; 85: 145–149PubMedCrossRef

34.

Lina G, Gillet Y, Vadenesch F, et al. Toxin involvement in Staphylococcal scalded skin syndrome. Clin Infect Dis 1997; 25: 1369–1373PubMedCrossRef

35.

Arbuthnott JP, Gemmel CG, Kent J, et al. Haemolysin and enzyme patterns of coagulase-positive staphylococci isolated from toxic epidermal necrolysis, Ritter’s disease and impetigo contagiosa. J Med Microbiol 1969; 2: 479–487PubMedCrossRef

36.

Dancer SJ, Garratt R, Saldanha J, et al. The epidermolytic toxins are serine proteinases. FEBS Lett 1990; 268: 129–132PubMedCrossRef

37.

Wiley BB, Allman S, Rogolsky M, et al. Staphylococcal scalded skin syndrome: potentiation by immunosuppression in mice; toxin-mediated exfoliation in a healthy adult. Infect Immun 1974; 9: 636–640PubMed

38.

Rogolosky M, Wiley BB, Glasgow LA. Phage group II Staphylococcal strains with chromosomal and extra-chromosomal genes for exfoliative toxin production. Infect Immun 1976; 13: 44–52

39.

Oono T, Kanzaki H, Yoshioka T, et al. Staphylococcal scalded skin syndrome in an adult: identification of exfoliative toxin A and B genes by polymerase chain reaction. Dermatology 1997; 195: 268–270PubMedCrossRef

40.

Criber B, Piemont Y, Grosshans E. Staphylococcal scalded skin syndrome in adults. J Am Acad Dermatol 1994; 30: 319–324CrossRef

41.

Kawabata A, Ichiyama S, Iinuma Y, et al. Exfoliative toxin detection using reverse passive latex agglutination: clinical and epidemiologic application. J Clin Microbiol 1997; 35: 1984–1987PubMed

42.

Sarai Y, Nikahara H, Ishikawa T, et al. A bacteriological study on children with staphylococcal toxic epidermal necrolysis in Japan. Dermatologica 1977; 154: 161–167PubMedCrossRef

43.

Murono K, Fujita K, Yoshika H. Microbiological characteristics of exfoliative toxin-producing Staphylococcus aureus. Paediatr Infect Dis J 1988; 7: 313–315CrossRef

44.

De Azevedo JCS, Arbuthnott JP. Assays for epidermolytic toxin of Staphylococcus aureus. Methods Enzymol 1988; 165: 333–338CrossRef

45.

Rifai S, Barbancon V, Prevost G, et al. Synthetic exfoliative toxin A and B DNA probes for detection of toxigenic Staphylococcus aureus strains. J Clin Microbiol 1989; 27: 504–506PubMed

46.

Murono K, Fujita K, Yoskioka H. Detection of staphylococcal exfoliative toxin by slide latex agglutination. J Clin Microbiol 1988; 26: 271–274PubMed

47.

Hoeger PH, Elsner P. Staphylococcal scalded skin syndrome: transmission of the exfoliatin-producing Staphylococcus aureus by asymptomatic carriers. Pediatr Infect Dis 1988; 7: 340–342CrossRef

48.

Dave J, Reith S, Nash JQ, et al. A double outbreak of exfoliative toxin-producing strains of Staphylococcus aureus in a maternity unit. Epidemiol Infect 1994; 112: 103–114PubMedCrossRef

49.

Curran JP, Al-Sahili FL. Neonatal staphylococcal scalded skin syndrome: massive outbreak due to an unusual phage type. Paediatrics 1980; 66: 285–290

50.

Opal SM, Johnson-Winegar AD, Cross AS. Staphylococcal scalded skin syndrome in two immunocompetent adults caused by exfoliating B-producing Staphylococcus aureus. J Clin Microbiol 1988; 26: 1283–1286PubMed

51.

Diem E, Konrad K, Graninger W. Staphylococcal scalded skin syndrome in an adult with fatal disseminated sepsis. Acta Derm Venereol (Stockh) 1982; 62: 295–299

52.

Petzelbauer P, Konrad K, Wolff K. Staphylococcal scalded skin syndrome in 2 adults with acute kidney failure. Hautarzt 1989; 40: 90–93PubMed

53.

Rothenberg R, Renna F, Drew T. Staphylococcal scalded skin syndrome in an adult. Arch Dermatol 1973; 108: 408–410PubMedCrossRef

54.

Neefe LI, Tuazon CV, Cardella TA, et al. Staphylococcal scalded skin syndrome in adults: case report and review of the literature. Am J Med Sci 1979; 277: 99–110PubMedCrossRef

55.

Borchers SL, Gomez EC, Isseroff RR. Generalized staphylococcal scalded skin syndrome in an anephric boy undergoing hemodialysis. Arch Dermatol 1984; 120: 912–918PubMedCrossRef

56.

Donohue D, Robinson B, Goldberg NS. Staphylococcal scalded skin syndrome in a woman with chronic renal failure exposed to human immunodeficiency virus. Cutis 1991; 47: 317–318PubMed

57.

Elsner P, Hartmann AA. Epidemiology of ETA- and ETB- producing staphylococci in dermatological patients [abstract]. Zentralbl Bakteriol Mikrobiol Hyg [A] 1988; 268: 534

58.

Arbuthnott JP. Characterisation of the epidermolytic toxins of Staphylococcus aureus. In: Macdonald A, Smith G, editors. The Staphylococci: proceedings of the Alexander Ogston Centennial Conference. Aberdeen: Aberdeen University Press, 1981: 109–118

59.

Acland KM, Darvay A, Griffin C, et al. Staphylococcal scalded skin syndrome in an adult associated with methicillin-resistant Staphylococcus aureus. Br J Dermatol 1999; 140: 518–520PubMedCrossRef

60.

McLay ALC, Arbuthnott JP, Lyell A. Action of Staphylococcal epidermolytic toxin on mouse skin: an electron microscopy study. J Invest Dermatol 1975; 65: 423–428PubMedCrossRef

61.

Sato H, Matsumori Y, Tanabe T, et al. A new type of staphylococcal exfoliative toxin from a Staphylococcus aureus strain isolated from a horse with phlegmon. Infect Immun 1994; 62: 3780–3785PubMed

62.

Lillibridge CB, Melish ME, Glasgow LA. Site of action of exfoliative toxin in the staphylococcal scalded skin syndrome. Pediatrics 1972; 50: 728–738PubMed

63.

Kapral FA, Miller NM. Product of Staphylococcal aureus responsible for the staphylococcal scalded skin syndrome. Infect Immun 1971; 4: 541–545PubMed

64.

Arbuthnott JP, Kent J, Lyell A, et al. Studies of staphylococcal toxins in relation to toxic epidermal necrolysis: the scalded skin syndrome [supplement]. Br J Dermatol 1972; 86: s35–s39CrossRef

65.

Wuepper KD, Dimond RL, Knutson DD. Studies of the mechanism of epidermal injury by Staphylococcal epidermolytic toxin. J Invest Dermatol 1975; 65: 191–200PubMedCrossRef

66.

Melish ME, Chen FS, Sprouse S, et al. Epidermolytic toxin in staphylococcal infection: toxin levels and host response. Zentralbl Bakteriol Mikrobiol Hyg Suppl 1981; 10: 287–298

67.

Bailey CJ, de Azavedo J, Arbuthnott JP. A comparative study of two serotypes of epidermolytic toxin from Staphylococcus aureus. Biochim Biophys Acta 1980; 624: 111–120PubMedCrossRef

68.

Kondo I, Sakurai S, Sarai Y. New type of exfoliatin obtained from staphylococcal strains, belonging to phage groups other than group II, isolated from patients with impetigo and Ritter’s disease. Infect Immun 1974; 10: 851–861PubMed

69.

Sakurai S, Suzuki H, Kondo I. DNA sequencing of the eta gene coding for Staphylococcal exfoliative toxin serotype A. J Gen Microbiol 1988; 134: 711–717PubMed

70.

Lee CY, Schmidt JJ, Johnson-Winegar AD, et al. Sequence determination and comparison of exfoliative toxin A and B genes from Staphylococcal aureus. J Bacteriol 1987; 169: 3904–3909PubMed

71.

O’Toole PW, Foster TJ. Nucleotide sequence of the epidermolytic toxin A gene of Staphylococcus aureus. J Bacteriol 1987; 169: 3910–3915PubMed

72.

Sakuria S, Suzuki H, Kondo I. Cloning of the gene coding for Staphylococcal exfoliative toxin A and its expression in Escherichia coli. FEMS Microbiol Lett 1987; 42: 63–67CrossRef

73.

Smith TP, John DA, Bailey CJ. The binding of epidermolytic toxin from staphylococcus aureus to mouse epidermal tissue. Histochem J 1987; 19: 135–149CrossRef

74.

Gentihomme E, Faure M, Piemont Y, et al. Action of staphylococcal exfoliative toxins on epidermal cell cultures and organotypic skin. J Dermatol 1990; 17: 526–532

75.

Vath GM, Earhart CA, Rago JV, et al. The structure of the superantigen exfoliative toxin A suggests a novel regulation as a serine protease. Biochemistry 1997; 36: 1559–1566PubMedCrossRef

76.

Prevost G, Rifai S, Chaix ML, et al. Functional evidence that Ser-195 residue of Staphylococcal exfoliative toxin A is essential for biological activity. Infect Immun 1991; 59: 3337–3339PubMed

77.

Redpath MB, Foster TJ, Bailey CJ. The role of serine protease active site in the mode of action of epidermolytic toxin of S. aureus. FEMS Microbiol Lett 1991; 81: 151–156CrossRef

78.

Jaulhac B, Piemont Y, Prevost G. Staphylococcal exfoliative toxins induce proteolytic activity when combined with epidermis. In: Mollby R, Flock JI, Nord CE, et al., editors. Staphylococci and staphylococcal infections. New York: Gustav Fischer Verlag, 1994: 280–282

79.

Barbosa JARG, Saldanaha JW, Garratt RC. Novel features of serine protease active sites and specificity pockets: sequence analysis and modelling studies of glutamate-specific endopeptidases and epidermolytic toxins. Protein Eng 1996; 9: 591–601PubMedCrossRef

80.

Rago JV, Vath GM, Tripp TJ, et al. Staphylococcal exfoliative toxins cleave alphaand beta-melanocyte-stimulating hormones. Infect Immun 2000; 68: 2366–2368PubMedCrossRef

81.

Mahoney MG, Wang Z, Rothenberger K, et al. Explanation for the clinical and microscopic localization of lesions in pemphigus foliaceous and vulgaris. J Clin Invest 1999; 103: 461–468PubMedCrossRef

82.

Marrack P, Kappler J. The staphylococcal enterotoxins and their relatives. Science 1990; 346: 471–473

83.

Khuong MA, Chosidow O, Solh NE, et al. Staphylococcal scalded skin syndrome in an adult: possible influence of non-steroidal anti-inflammatory drugs. Dermatology 1993; 186: 153–154PubMedCrossRef

84.

Haas Baker D, Wuepper KD, Rasmussen JE. Staphylococcal scalded skin syndrome: detection of antibody to epidermolytic toxin by a primary binding assay. Clin Exp Dermatol 1978; 3: 17–24CrossRef

85.

Wiley B, Glasgow L, Rogolsky M. Studies on staphylococcal scalded skin syndrome (SSSS): isolation and purification of toxin and development of a radioimmunobinding assay for antibodies to exfoliative toxin (ET). In: Jeljaszewicz J, editor. Staphylococci and staphylococcal diseases. Stuttgart: Gustav Fischer Verlag, 1976: 499–516

86.

Beer B, Wilson B. Adult staphylococcal scalded skin syndrome. Int J Dermatol 1990; 29: 428–429CrossRef

87.

Herzog JL, Sexton M. Desquamative rash in an immunocompromised adult. Arch Dermatol 1990; 26: 815–819CrossRef

88.

Trentham DE, Silvermann HA, Townes AS. Cause of adult toxic epidermal necrolysis [letter]. N Engl J Med 1975; 292: 870PubMed

89.

Saiag P, Caumes E, Roujeau JC, et al. Epidermolyse staphylococcique (syndrome de Lyell staphylococcique) de l’adult: 2 cas. Ann Dermatol Venereol 1988; 115: 1164–1166PubMed

90.

Fritsch P, Elias P, Varga J. The fate of Staphylococcal exfoliatin in newborn and adult mice. Br J Dermatol 1976; 95: 275–284PubMedCrossRef

91.

Kirmani N, Tauzon CU, Murray HW, et al. Staphylococcus aureus carriage rate of patients receiving long term haemodialysis. Arch Intern Med 1978; 138: 467–475CrossRef

92.

Rothenburg R, Renna FS, Drew TM, et al. Staphylococcal scalded skin syndrome in an adult. Arch Dermatol 1973; 108: 408–410CrossRef

93.

Epstein Jr EH, Flynn P, Davis RS. Adult toxic epidermal necrolysis and fatal staphylococcal septicaemia. JAMA 1974; 229: 425–427PubMedCrossRef

94.

O’Keefe R, Dagg JH, Mackie RM. The staphylococcal scalded skin syndrome in two elderly immunocompromised patients. BMJ 1987; 295: 179–180PubMedCrossRef

95.

Roeb E, Schonfelder T, Matern S, et al. Staphylococcal scalded skin syndrome in an immunocompromised adult. Eur J Clin Microbiol Infect Dis 1996; 15: 499–503PubMedCrossRef

96.

Strumia R, Bedetti A, Cavazzini L. Staphylococcal scalded skin syndrome in corso di AIDS. G Ital Dermatol Venereol 1990; 125: 461–464PubMed

97.

Farrell AM, Ross JS, Umasankar S, et al. Staphylococcal scalded skin syndrome in an HIV-1 seropositive man. Br J Dermatol 1996; 134: 962–965PubMedCrossRef

98.

Hawley HB, Aronson MD. Scalded skin syndromes in adults. N Engl J Med 1972; 288: 130

99.

Melish ME, Chen FS, Sprouse S, et al. Epidermolytic toxin in staphylococcal infection: toxin levels and host response. Zentralbl Bakteriol Mikrobiol Hyg Suppl 1981; 10: 287–298

100.

Ridgway HB, Lowe NJ. Staphylococcal scalded skin syndrome in an adult with Hodgkin’s disease. Arch Dermatol 1979; 115: 589–590PubMedCrossRef

101.

Melish ME, Glasgow LA. The staphylococcal scalded skin syndrome: the expanded clinical syndrome. J Paediatr 1971; 78: 958–967CrossRef

102.

Rassmussen JE. Toxic epidermal necrolysis: a review of 75 cases in children. Arch Dermatol 1975; 111: 1135–1139CrossRef

103.

Rudolph RI, Shwartz W, Leyden JJ. Treatment of staphylococcal toxic epidermal necrolysis. Arch Dermatol 1974; 110: 559–562PubMedCrossRef

104.

Yokota S, Imagawa T, Katakura S, et al. Staphylococcal scalded skin syndrome caused by exfoliative toxin B-producing methicillin-resistant Staphylococcus aureus [letter]. Eur J Paediatr 1996; 155: 722

105.

Mackenzie A, Johnson W, Heyes B, et al. A prolonged outbreak of exfoliative toxin A-producing Staphylococcus aureus in a newborn nursery. Diagn Microbiol Infect Dis 1995; 21: 69–75PubMedCrossRef

Title: Staphylococcal Scalded Skin Syndrome
Diagnosis and Management
Authors: Dr Girish K. Patel
Andrew Y. Finlay
Publication date: 01-03-2003
Publisher: Springer International Publishing
Published in: American Journal of Clinical Dermatology / Issue 3/2003
Print ISSN: 1175-0561
Electronic ISSN: 1179-1888
DOI: https://doi.org/10.2165/00128071-200304030-00003

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Staphylococcal Scalded Skin Syndrome

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