Basics
Description
- A spectrum of generalized exfoliative skin disease with blistering of the upper layer of skin caused by an epidermolytic toxin produced by certain strains of Staphylococcus aureus
- In neonates and young infants, also known as Ritter disease or pemphigus neonatorum
- Classically described as skin tenderness and erythema, with bullae formation and desquamation
- Severity of the disease ranges from
- Few blisters localized to site of infection
- Mild illness with desquamation of skinfolds following impetigo
- Generalized severe exfoliation involving much of the body (typically seen in neonates)
- Classic staphylococcal scalded skin syndrome (SSSS): tenderness, erythema, desquamation, or bullae formation. May resemble scalding injury
- Pitfalls
- Failure to differentiate from streptococcal disease, as SSSS requires treatment with penicillinase-resistant antibiotic therapy (e.g., nafcillin)
- Late recognition leading to delayed therapy and shock
- Not appreciating increased fluid losses through affected skin
- Differentiation from toxic epidermal necrolysis (TEN) is critical, as therapy is very different.
Epidemiology
- Most cases occur in neonates and children.
- 62% of affected children are <2 years of age.
- 98% of affected children are <6 years of age.
- Rare in adults due to increased circulating antibodies and adult kidney excretion of the toxin
Incidence
- No differences in incidence based on gender in children; however, in adults, the male-to-female ratio is 2:1.
Risk Factors
- Immunocompromised state (in children or adults)
- Maternal antibodies transferred via breast milk are partially protective, but neonatal cases can still occur.
- Increased S. aureus carriage and susceptibility to toxin (usually in adults)
- Renal impairment either due to immature renal clearance of toxin in children or underlying renal disease
General Prevention
- Good hand hygiene practices, including adherence to contact precautions in hospitalized patients, to prevent spread from asymptomatic carriers
- Prevent skin from becoming overly moist or macerated.
- Isolation of hospitalized patient
- Suspected or documented cases should be placed in contact isolation.
Pathophysiology
- Exfoliative toxins circulate throughout the body, causing blisters at sites distant from the infection.
- Destruction of protein desmoglein 1 (attachment protein found only in the superficial epidermis) by exfoliative toxin A (ETA) and exfoliative toxin B (ETB) cause intraepidermal splitting leading to bullae development and skin desquamation.
Etiology
- Exfoliative toxin released by S. aureus:
- 2 major serotypes of the toxin: ETA and ETB
- Mostly caused by S. aureus belonging to phage group II, types 71 and 55
Commonly Associated Conditions
- Skin and soft tissue infections or abscesses
- Bullous impetigo
Diagnosis
Alert
- Diagnosis is primarily clinical; do not delay treatment. Cultures and other diagnostic tests are largely confirmatory.
- Confusion with TEN may lead to use of corticosteroids or discontinuation of antibiotics, resulting in worsening infection from prolonged toxin production.
History
- Nonspecific virus-like prodrome with irritability, sore throat, conjunctivitis, and upper respiratory infection is typical.
- Usual onset of fever within 48 hours after prodrome
- Rash typically begins periorally, then extends to the trunk and extremities, and finally desquamates.
- Recent localized extracutaneous infection is common.
- Infections involving the nasopharynx, middle ear, conjunctivae, pharynx, tonsils, umbilicus, or urinary tract are frequently recalled.
- A history of recent drug use suggests other etiology such as TEN.
Physical Exam
- Erythroderma: red, painful skin
- Large flaccid bullae that leave behind denuded skin resembling a burn after rupturing appear within 1 " “2 days
- Bullae often appear in areas of trauma or in areas that are rubbed or touched, including intertriginous zones.
- Nikolsky sign (gentle friction applied to apparently healthy skin will cause blistering and sloughing) appears within 1 " “2 days.
- Distribution of lesions: most commonly face, neck, axilla, perineum
- Facial edema with perioral and periocular crustiness is typical and may be the primary clinical features.
- Conjunctivitis with or without periorbital edema may also be present.
Alert
- Nikolsky sign can be seen in both TEN and SSSS, but in SSSS, it is often noted over areas of unaffected skin as well.
- SSSS does NOT involve the mucous membranes, but TEN does.
Diagnostic Tests & Interpretation
Lab
- General: CBC may be normal; erythrocyte sedimentation rate (ESR) typically elevated; electrolytes and renal function should be followed closely in severe cases and cases with dehydration.
- Microbiologic diagnosis: Culture of the original infected site, other involved sites/abnormal skin, blood, urine, nasopharynx, and umbilicus should be performed to determine the organism and antibiotic susceptibility.
- Typically isolate phage group II S. aureus
- Some immunologic methods exists to specifically identify the exfoliative exotoxins.
- Intact bullae are sterile.
- Blood cultures are typically negative.
- Histologic diagnosis: Skin biopsy can be used to differentiate SSSS from TEN; SSSS demonstrates separation of the epidermis at the granular layer, whereas with TEN, there is necrosis of the entire epidermis with a deeper plane of separation at the basement membrane.
Differential Diagnosis
- TEN
- Kawasaki disease
- Bullous impetigo
- Erythema multiforme bullosum
- Erythema multiforme major (Stevens-Johnson syndrome)
- Streptococcal scarlet fever
- Streptococcal or staphylococcal toxic shock syndrome (TSS)
- Bullous varicella
- Burns, including inflicted burns in suspected child abuse
- Primary bullous disorders (e.g., bullous mastocytosis)
- Chronic bullous disease of childhood
- Pemphigus vulgaris or foliaceus
- Epidermolysis bullosa
Treatment
General Measures
- Hospitalization is necessary for antibiotic therapy and supportive care.
- Consider consultation with infectious disease and/or dermatology.
- Apply principles of good burn care in severe cases, including the following:
- Consideration of management in a critical care setting
- Aggressive and early fluid and electrolyte management including daily maintenance requirements in addition to replacement of increased insensible skin losses
- Petrolatum gauze should cover eroded areas to prevent further skin trauma.
- Blisters should be left intact.
- Children should be allowed to rest unclothed on clean linens, and handling of the child should be kept to a minimum.
- Use of pressure-relieving mattresses
Medication
- 1st-line agent: parenteral antistaphylococcal antibiotics: nafcillin, oxacillin, or 1st-generation cephalosporin (e.g., cefazolin)
- Some experts add clindamycin to inhibit exotoxin production.
- Clindamycin or vancomycin can be used for penicillin-allergic patients (severe allergies).
- 2nd-line agent: vancomycin for severe cases with toxic-appearing patient or concern for methicillin-resistant Staphylococcus aureus (MRSA)
- MRSA is rare but can occur.
- Antibiotic therapy can be tailored once sensitivities are known.
- Topical antibiotics are of no benefit.
- Oral antibiotics are not effective initially, but once a clear response has been noted with parenteral antibiotics, an oral antibiotic active against S. aureus can be used to complete the course of therapy.
- Corticosteroids have been shown to be detrimental both in experimental animal models as well as clinical trials.
- Adequate pain management is essential.
Alert
Avoid nonsteroidal anti-inflammatories due to potential for renal impairment, ‚
Ongoing Care
Prognosis
- Usually complete recovery within 10 " “14 days without scarring if treated
- Prognosis is more guarded in infants and those with underlying illness.
- Childhood mortality approximately 4%, whereas adult mortality reportedly >60%
- Does not tend to recur
Complications
- Occasional shedding of hair and nails
- Fungal or bacterial superinfection following desquamation
- Serious fluid and electrolyte disturbances may occur in cases involving large surface areas, which may lead to poor temperature control, sepsis, shock, and death.
- Neonates are particularly susceptible.
Additional Reading
- Braunstein ‚ I, Wanat ‚ KA, Abuabara ‚ K, et al. Antibiotic sensitivity and resistance patterns in pediatric staphylococcal scalded skin syndrome. Pediatr Dermatol. 2014;31(3):305 " “308. doi:10.1111/pde.12195. ‚ [View Abstract]
- Li ‚ MY, Hua ‚ Y, Wei ‚ GH, et al. Staphylococcal scalded skin syndrome in neonates: an 8-year retrospective study in a single institution. Pediatr Dermatol. 2013;31(1):43 " “47. doi:10.1111/pde.12114. ‚ [View Abstract]
- Patel ‚ GK, Finlay ‚ AY. Staphylococcal scalded skin syndrome: diagnosis and management. Am J Clin Dermatol. 2003;4(3):165 " “175. ‚ [View Abstract]
- Schenfeld ‚ LA. Images in clinical medicine. Staphylococcal scalded skin syndrome. N Engl J Med. 2000;342(16):1178. ‚ [View Abstract]
- Stanley ‚ JR, Amagai ‚ M. Pemphigus, bullous impetigo, and the staphylococcal scalded-skin syndrome. N Engl J Med. 2006;355(17):1800 " “1810. ‚ [View Abstract]
Codes
ICD09
ICD10
- L00 Staphylococcal scalded skin syndrome
SNOMED
- 200946001 Staphylococcal scalded skin syndrome (disorder)
- 402967005 Neonatal staphylococcal scalded skin syndrome (disorder)
FAQ
- Q: Can SSSS recur?
- A: Yes, although it is uncommon.
- Q: Is SSSS contagious?
- A: Yes. The staphylococci are spread primarily from person to person (familial clusters have been reported), even from mother to fetus, most efficiently by someone with lesions, but asymptomatic carriers may also spread infection. Spread of organisms does not necessarily lead to signs of toxin production in those acquiring infection.
- Q: How can one distinguish TEN from SSSS?
- A: TEN is frequently confused with SSSS and may be differentiated by skin biopsy showing cleavage plane at the dermal " “epidermal junction. TEN is more common in older children and adults and is usually secondary to drug hypersensitivity (e.g., sulfonamides, barbiturates, pyrazolone derivatives).
- Q: Can Staphylococcus be isolated from the bullae?
- A: SSSS bullae are sterile, although organisms may be found in a distant focus, such as the nares or conjunctivae. In bullous impetigo, however, staphylococci may be isolated from the bullae.