Core Dermatology Diagnoses / CategoriesInfectious

Staphylococcal scalded skin syndrome

Author: Faculty Reviewer:

Publish date: Posted on
Last updated: April 1, 2021

Keywords #

staphylococcal scalded skin syndrome
staphylococcus aureus
exfoliative toxin
MSSA
MRSA
blistering disease

Diagnosis #

Staphylococcal scalded skin syndrome (SSSS) is a superficial blistering disease caused by toxogenic strains of Staphylococcus aureus.[1,2] Exfoliative toxins A and B (ETA, ETB) cleave desmoglein 1, a cadherin in the stratum granulosum, leading to superficial, flaccid bulla formation and epidermal detachment.[1,2] SSSS occurs most commonly in children under the age of 5; however, adults—particularly those with risk factors including immune suppression and kidney dysfunction—can be affected.[2] Children generally make a full recovery with prompt treatment; however, adults typically have worse outcomes with reported mortality rates approaching 60-65%.[2]

Key Concepts #
  • SSSS is characterized by superficial, flaccid bullae with a positive Nikolsky sign.
  • Radial fissuring and crusting are characteristic around the mouth, but intraoral involvement is absent as SSSS does not affect the mucosae.
  • Infants and young children are most frequently affected. Adults with certain host factors—such as immunocompromise and renal dysfunction—are also at risk.
  • aureus infection originates locally with subsequent hematogenous spread of toxin. As such, cultures from the primary site of infection are positive, while blister fluid from distant sites are not.
  • Methicillin-sensitive strains of aureus (MSSA) are more common than methicillin-resistant (MRSA).
  • Treatment centers around supportive care and intravenous anti-staphylococcal antibiotics.
Epidemiology #

SSSS is more common in infants and young children below the age of 6 given a lack of protective antibodies against ETA and ETB.[1,2] The estimated annual incidence is approximately 8 cases per million in often otherwise healthy children.[3] This is in contrast to adults where the annual incidence is approximately 1 case per million with a predilection for those with poor immunity, impaired renal clearance and malignancy.[3] Additional comorbidities observed in the adult population were diabetes mellitus, substance abuse and other bacterial and fungal infections.[2,3]

Clinical Features #
  • There is often a prodromal period of fever, malaise, and irritability followed by skin tenderness and widespread erythema typically beginning on the head and within skin folds.
  • Wrinkled skin texture is noted with development of fluid-filled, fragile blisters sparing the mucous membranes.
  • Gentle tangential pressure on the skin results in separation of the superficial epidermis (Nikolsky sign).[1,2]
  • Large sheets of skin desquamate, leaving a red, moist surface behind.
  • Crusting and radial fissuring, especially around the periorificial surfaces, are common.[2,4]
  • Complications of SSSS are a result of epidermal detachment and barrier compromise.
  • Dehydration, electrolyte imbalances, and hypothermia may occur.[1,2]
  • Exposed skin is also susceptible to secondary infection.[1,2,5]
Differential Diagnoses #
  • Stevens-Johnson syndrome
  • Staphylococcal toxic shock syndrome
  • Bullous impetigo
  • Pemphigus
  • Scalding thermal burn
  • Kawasaki disease
  • Scarlet fever

    • Lack of mucosal involvement and the level of cleavage assist in the exclusion of SJS and TEN, which may also have a positive Nikolsky sign.  In bullous impetigo, S. aureus can be cultured from skin lesions and there is an absence of generalized erythema and skin pain.[1] While pemphigus foliaceous shares a target in desmoglein 1, the clinical context and host factors help to distinguish this entity. Direct immunofluorescence microscopy can be utilized in cases of uncertainty.  While also common in children, scarlet fever lacks skin fragility and blistering.[1,5]

Diagnostic Workup #

The diagnosis is made clinically based on history and physical examination. Bacterial cultures may be obtained from the primary site of infection, but do not yield organisms from distant intact bullae.  Children generally do not have positive blood cultures for S. aureus, though this may be seen in adult patients.[1,2] Histologic examination is not always necessary, but an urgent frozen section can be helpful in identifying the level of separation in cases of uncertainty.[1,2]

Treatment #

Prompt treatment is necessary with an intravenous anti-staphylococcal antibiotic, such as nafcillin or oxacillin.[1,4] If there is poor response to treatment or MRSA is suspected, vancomycin should be considered.[1,2] Clindamycin may reduce production of exfoliative toxins, though resistance precludes use as monotherapy.[1,4] Supportive care with sufficient IV fluids, temperature control, and wound care is also necessary.  In children, the skin is expected to heal without scarring in approximately 2 weeks.[1,2]

References #
  1. Leung AKC, Barankin B, Leong KF. Staphylococcal-scalded skin syndrome: evaluation, diagnosis, and management. World J Pediatr. 2018;14(2):116-120.
  2. Handler MZ, Schwartz RA. Staphylococcal scalded skin syndrome: diagnosis and management in children and adults. J Eur Acad Dermatol Venereol. 2014;28(11):1418-1423.
  3. Staiman A, Hsu DY, Silverberg JI. Epidemiology of staphylococcal scalded skin syndrome in US adults. J Am Acad Dermatol. 2018;79(4):774-776.
  4. Conway DG, Lyon RF, Heiner JD. A desquamating rash; staphylococcal scalded skin syndrome. Ann Emerg Med. 2013;61(1):118, 129.
  5. Mishra AK, Yadav P, Mishra A. A Systemic Review on Staphylococcal Scalded Skin Syndrome (SSSS): A Rare and Critical Disease of Neonates. Open Microbiol J. 2016;10:150-159.
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