Staphylococcal scalded skin syndrome in Adult
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Synopsis
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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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Bullous impetigo – Localized with honey-colored crusted plaques; bacteria present in bullae.
- 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)
- Exanthematous drug eruption
- Drug-induced erythroderma
- Toxic epidermal necrolysis (TEN) – Drug induced, high fevers, skin tenderness, mucosal erosions, and skin detachment about 1-3 weeks after the inciting medication is started.
- 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.
- Erythrodermic psoriasis
- Atopic dermatitis
- Contact dermatitis
- Lupus erythematosus
- Pemphigus erythematosus
- Pityriasis rubra pilaris
- Scabies
- Seborrheic dermatitis
- Lymphomas
- Reactive arthritis (Reiter syndrome)
- Sézary syndrome (see cutaneous T-cell lymphoma)
- Urticaria
- Erysipelas
- Necrotizing fasciitis – Rapidly progressing necrosis of fascia and subcutaneous fat.
- Methotrexate-induced mucocutaneous toxicity
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References
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Last Reviewed:06/08/2017
Last Updated:10/10/2019
Last Updated:10/10/2019
Staphylococcal scalded skin syndrome in Adult