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Staphylococcal scalded skin syndrome in Adult
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Staphylococcal scalded skin syndrome in Adult

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Contributors: Lauren Ko, Susan Burgin MD
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Synopsis

Staphylococcal scalded skin syndrome (SSSS), or Ritter disease, is a toxin-mediated infection characterized by skin tenderness, flaccid bullae, and skin detachment. A prodrome of fever, sore throat, and malaise, accompanied by purulent rhinorrhea and/or conjunctivitis, often occurs prior to the onset of bullae and desquamation.

SSSS is mainly a disease of infants and children younger than 6, with a higher incidence seen in males. With appropriate antibiotics, SSSS often resolves entirely in 1-2 weeks. However, many adults who suffer from SSSS have underlying medical problems such as renal insufficiency, and mortality rates are estimated at 60% or higher in immunocompromised patients.

In the United States, phage group II staphylococci are the most common toxin-producing strains. These phage group II strains can be methicillin-sensitive and resistant and produce exotoxins (epidermolytic toxins A and B or ETA and ETB, respectively) that cause intraepidermal cleavage with subsequent superficial sloughing. Specifically, ETA and ETB are serine proteases that cleave the extracellular domain of desmoglein 1, a molecule that facilitates keratinocyte adhesion. Note that bullous impetigo and toxic shock syndrome are also toxin-mediated diseases considered within the same spectrum of SSSS.

The natural history of SSSS is characterized by the following:
  • Prodromal symptoms and/or purulent rhinorrhea and/or conjunctivitis
  • Facial erythema that generalizes to the body in less than 48 hours
  • Bullae development, positive Nikolsky's sign, and very tender skin
  • Skin wrinkling and epidermal sloughing within 48 hours after bullae develop
  • Desquamation continues for up to 5 days
  • Re-epithelialization without scarring in approximately 2 weeks

Codes

ICD10CM:
L00 – Staphylococcal scalded skin syndrome

SNOMEDCT:
200946001 – Staphylococcal scalded skin syndrome

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Last Reviewed: 06/09/2017
Last Updated: 01/11/2018
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Staphylococcal scalded skin syndrome in Adult
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Staphylococcal scalded skin syndrome : Bullae, Desquamation, Erythroderma, Painful skin lesions, Rhinorrhea, Widespread, Conjunctival injection
Clinical image of Staphylococcal scalded skin syndrome
Diffuse bright red erythema with large areas of overlying peeling on the trunk and legs.
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