Basics
Description
- Results from the actions of a soluble epidermolytic exotoxin produced by Staphylococcus aureus:
- Produced at a distant site of infection or colonization
- Disseminates hematogenously
- Lyses desmosomes of granular cells in the superficial epidermis
- Results in generalized intradermal exfoliation
- Typically affects infants and children <6 yr of age:
- Adults have specific staph antibodies allowing them to localize, metabolize, and excrete the staph toxins.
- Infants and children are unable to metabolize and excrete toxin efficiently.
- Immunocompromised adults and those with severe renal dysfunction are also susceptible
- Presentation determined by age and extent of rash:
- Classic staphylococcal scalded skin syndrome
- Pemphigus neonatorum
- Bullous impetigo
- Generalized in the newborn: Ritter disease
- Typically, coagulase-positive phage group II Staphylococcus:
- Phage groups I and III also implicated
Etiology
- Colonization often without overt infection
- Concurrent infection or break of skin barrier:
- Nasopharynx
- Urinary tract
- Minor skin abrasions
- Circumcision site
- Conjunctivitis
- Umbilicus/omphalitis
- Impetigo
- Endocarditis and septicemia
- Often no focus identified
Diagnosis
Signs and Symptoms
- Constitutional symptoms:
- Malaise
- Fever
- Irritability
- Child may appear well, ill, or overtly toxic
- Abrupt onset
- Scarlatiniform erythematous rash (sandpaper like) resembling a "sunburn " ¯ " ”erythroderma
- Exquisitely tender skin
- Areas of prominence:
- Around the flexor areas of the neck
- Intertriginous areas, especially axilla and groin
- Near the eyes and mouth
- Increased erythema in skin creases
- Facial edema with radial crusting fissures around the eyes, nose, and mouth
- Flaccid bullae:
- Within 1 " “3 days after onset of rash
- Initially over flexures (axillae, groin, body orifices)
- Bullae migrate through epidermis with light lateral pressure; epidermis separates with minor pressure (Nikolsky sign).
- Rupture within hours
- Epidermis separates with minor trauma.
- Epidermis is shed in sheets.
- Denuded areas are moist, sensitive, and painful.
- Complete healing within 2 wk, no scarring
- Purulent conjunctivitis
- Mucous membranes not affected
- Complications rare:
- Hypothermia
- Fluid and electrolyte imbalance
- Secondary infection
- Pneumonia
- Septicemia
- Cellulitis
- Osteomyelitis
Essential Workup
- Clinical presentation is diagnostic.
- Determine location/source of toxin producing Staphylococcus.
- Assess systemic nature of infection.
Diagnosis Tests & Interpretation
Lab
- CBC and urinalysis:
- Assess for sepsis if source not obvious.
- Electrolytes:
- Indicated if signs of dehydration or extensive rash
- Blood cultures (rarely positive)
Imaging
Indicated as need to determine location/source of infection ‚
Diagnostic Procedures/Surgery
- Fluid aspirated from bullae:
- Sterile in staphylococcal scalded skin syndrome
- Consistent with hematogenous dissemination of the toxin
- Isolation of staphylococci from a site other than the blisters:
- Commonly conjunctivae, nasopharynx, or blood
- Skin biopsy or frozen histologic section:
- Determine level of epidermal/dermal separation (cleavage is in granular layer of dermis).
- Indicated for children on medications, those >6 yr, and in cases of mixed presentation
Differential Diagnosis
- Infection:
- Scarlet fever:
- Involves the mucous membranes
- Strawberry tongue
- Painful desquamation does not occur
- Bullous impetigo:
- Turbid or cloudy bullous fluid
- Bullous varicella:
- Tzanck prep or viral base reveals giant cells.
- 5 days after the onset of varicella
- Toxic shock syndrome:
- Rapid development of clinical signs and symptoms
- Mucous membrane and multiorgan involvement
- Toxic epidermal necrolysis or drug eruption:
- Much more common in adults
- Severely afflicted mucous membranes
- Full-thickness epidermal necrosis
- Dermatologic:
- Erythema multiforme
- Epidermolysis hyperkeratosis
- Epidermolysis bullosa
- Pemphigus vulgaris
- Scald injury
- Secondary rash of an underlying disorder:
- Lymphoma
- Aspergillosis
- Irradiation
- Graft-versus-host reaction
- Kawasaki disease
Treatment
Pre-Hospital
- 9% NS fluid bolus if dehydration present
- Initial burn treatment
Initial Stabilization/Therapy
- Management is similar to an extensive 2nd-degree burn:
- Involvement of large body surface area will require IV fluids.
- Provide adequate analgesia.
- Undress and place child on sterile linen.
- Limit handling of child.
- Apply moist sterile dressings.
- Avoid excess heat loss.
Ed Treatment/Procedures
- Topical burn creams are of no proven benefit.
- Steroids are contraindicated.
- IV antibiotics effective against penicillinase-resistant S. aureus:
- Cefazolin
- Nafcillin
- Vancomycin if methicillin-resistant S. aureus (MRSA) suspected
- Oral antibiotics for mild involvement:
- Dicloxacillin
- Erythromycin
- Cephalexin
Medication
- Cefazolin: 50 " “100 mg/kg/24 h IV div. QID
- Cephalexin: 25 " “100 mg/kg/24 h PO div. QID
- Dicloxacillin: 12 " “25 mg/kg/24 h PO div. QID
- Erythromycin: 30 " “50 mg/kg/24 h PO div. QID
- Nafcillin: 1 " “2 g IV q6h (peds: Newborns, 50 " “100 mg/kg/24 h IV div. q6h; children, 100 " “200 mg/kg/24 h IV div. q6h)
- Vancomycin: 40 mg/kg/24h IV q 6 hrs
Follow-Up
Disposition
Admission Criteria
- Children <1 yr
- All toxic-appearing children
- Widespread skin involvement
- Dehydration and/or electrolyte derangement
Discharge Criteria
- Older, well-appearing children with mild involvement
- Oral antibiotics for 7 days
- Follow-up within 48 hr
Issues for Referral
- Infectious disease consultant
- Surgeon if source needs excision/drainage
Additional Reading
- Blyth ‚ M, Estela ‚ C, Young ‚ AE. Severe staphylococcal scalded skin syndrome in children. Burns. 2008;34:98 " “103.
- Freedberg ‚ IM, Eisen ‚ AZ, Wolff ‚ K, et al. Fitzpatricks Dermatology in General Medicine. 6th ed. New York, NY: McGraw-Hill; 2003:195.
- Ladhani ‚ S. Recent developments in staphylococcal scalded skin syndrome. Clin Microbiol Infect. 2001;7(6):301 " “307.
- Patel ‚ GK, Finlay ‚ AY. Staphylococcal scalded skin-syndrome: Diagnosis and management. Am J Clin Dermatol. 2003;4:165 " “175.
- Stanley ‚ JR, Amagai ‚ M. Pemphigus, bullous impetigo, and the staphylococcal scalded-skin syndrome. N Engl J Med. 2006;355(17):1800 " “1810.
Codes
ICD9
695.81 Ritters disease ‚
ICD10
L00 Staphylococcal scalded skin syndrome ‚
SNOMED
- 200946001 Staphylococcal scalded skin syndrome (disorder)
- 402967005 Neonatal staphylococcal scalded skin syndrome (disorder)