Bacteria
Staphylococcal Scalded Skin Syndrome
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Staphylococcal Scalded Skin Syndrome
, Staphylococcus Scalded Skin Syndrome, Ritters Disease
Epidemiology
Typically affects age <5 years old (and especially <2 years)
Incidence
: <0.56 per Million (rare)
Pathophysiology
Group 2 Coagulase Positive
Staphylococcus aureus
strains
Strains that carry exfoliative toxins A and B (only 5% of S. aureus strains)
Breaks down desmoglein-1 resulting in epidermolysis or
Acantholysis
(breakage of cell to cell adhesions)
Similar to
Pemphigus Vulgaris
(which in contrast is immune mediated)
Sources
Initial localized infection
Skin sites (e.g.
Diaper Dermatitis
, ubilicus, face)
Nn-skin sites (e.g.
Otitis Media
,
Upper Respiratory Infection
Later, hematologic dissemination of
Staphylococcus aureus
from an infectious source
Contrast with
Impetigo
which spreads contiguously
Symptoms
Prodrome (initial
Upper Respiratory Infection
)
Otitis Media
Pharyngitis
Conjunctivitis
Acute phase (initial, prior to
Desquamation
)
Fever
Malaise
Irritability
Red, painful skin
Signs
Red, scarlatiniform, sandpaper-like rash
Paper-thin skin that desquamates (
Exfoliative Dermatitis
)
Distribution especially in the flexor creases, perioral area, neck and groin
Large, flaccid
Blister
s
Positive
Nikolsky Sign
(on affected skin)
No mucous membrane involvement
Labs
Blood Culture
s
Bacteremia is often present in Staphylococcal Scalded Skin Syndrome
Skin biopsy
Exotoxin assay
Avoid skin lesion cultures
Lesions are sterile and will be non-diagnostic
Management
Admit patients with diffuse involvement to ICU or burn unit
Intravenous antibiotics covering
Staphylococcus aureus
(consider
MRSA
coverage depending on risk)
Methicillin
Sensitive
Staphylococcus aureus
infections account for 95% of cases (as of 2018)
Systemic Corticosteroid
s may be used in non-toxic appearing patients
Adjunctive measures in severe cases
Intravenous
Immunoglobulin
Plasmapheresis
Course
Resolution in 5-7 days after antibiotics are initiated
Differential Diagnosis
See
Bullous Condition
s
Steven Johnson Syndrome
Toxic Epidermal Necrolysis
Multisystem Inflammatory Syndrome in Children
(
MIS-C
,
Kawasaki Disease
)
Drug Reaction with Eosinophilia and Systemic Symptoms
(
DRESS Syndrome
)
References
Long (2016) Crit Dec Emerg Med 30(7):3-10
Stewart (2022) Crit Dec Emerg Med 36(1): 16-7
Rare Disease Database, accessed 1/13/2022
https://rarediseases.org/rare-diseases/staphylococcal-scalded-skin-syndrome/
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