Staphylococcal scalded skin syndrome (SSSS), or Ritter disease, is an acute disease caused by epidermolytic toxins released by strains of Staphylococcus aureus. Newborns are particularly susceptible to these exfoliative toxins. Oftentimes, focal infection of the nasopharynx, conjunctivae, perineum, or umbilicus produces toxins that lead to diffuse fragile bullae, which are often no longer intact at the time of presentation. However, any systemic or cutaneous infection with epidermolytic toxin-producing S. aureus may induce SSSS. Infants are believed to have increased susceptibility to SSSS due to lack of neutralizing antibodies and decreased renal capacity for toxin excretion.
In the newborn, presentation generally occurs between 3-7 days of life, with only one reported case of congenital SSSS. Symptoms occur acutely, with sudden onset of fever, irritability, cutaneous tenderness, and characteristic cutaneous eruption. Symptoms of SSSS can occur in conjunction with bullous impetigo. Full recovery occurs in most cases. Sepsis (with multisystemic involvement) and fluid and electrolyte abnormalities are possible.
Staphylococcal scalded skin syndrome in Infant/Neonate
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Synopsis
Codes
ICD10CM:
L00 – Staphylococcal scalded skin syndrome
SNOMEDCT:
200946001 – Staphylococcal scalded skin syndrome
L00 – Staphylococcal scalded skin syndrome
SNOMEDCT:
200946001 – Staphylococcal scalded skin syndrome
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Differential Diagnosis & Pitfalls
- Bullous impetigo – Localized with honey-colored crusted plaques, bacteria present in bullae.
- Neonatal candidiasis
- Exanthematous drug eruption
- Drug-induced erythroderma
- Collodion baby
- Conradi disease
- Leiner disease
- Lamellar ichthyosis
- Keratitis-ichthyosis-deafness syndrome (KID syndrome)
- Netherton syndrome
- Epidermolytic ichthyosis – Present at birth and persists despite appropriate antibiotics.
- Toxic epidermal necrolysis (TEN) – Nikolsky's sign only in erythematous areas and mucous membrane involvement, drug induced, high fevers, skin tenderness, mucosal erosions, and skin detachment about 1-3 weeks after the inciting medication is started.
- Ichthyosis bullosa of Siemens (see epidermolytic ichthyosis) – Present at birth and persists despite appropriate antibiotics.
- Epidermolysis bullosa simplex
- Congenital syphilis
- Boric acid poisoning
- Methylmalonic acidemia
- Toxic shock syndrome – High fevers and severe systemic symptoms including vomiting and diarrhea; hypotension quickly ensues; diffuse scarlatiniform exanthem that starts on the trunk (in contrast to face in SSSS).
- Drug hypersensitivity syndrome (DRESS)
- Stevens-Johnson syndrome – Drug induced, high fevers, skin tenderness, mucosal erosions, and skin detachment about 1-3 weeks after the inciting medication is started.
- Scarlet fever – 1 mm erythematous papules, always elevated WBC with left shift, eosinophilia in up to 20% of patients.
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Last Reviewed:06/08/2017
Last Updated:05/05/2021
Last Updated:05/05/2021
Staphylococcal scalded skin syndrome in Infant/Neonate