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Staphylococcal Scalded Skin Syndrome, Emergency Medicine

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

- 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

- Infection: - Scarlet fever: - Involves the mucous membranes - Strawberry tongue - Painful desquamation does not occur

- 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

  • Results from the actions of a soluble epidermolytic exotoxin produced by Staphylococcus aureus:Produced at a distant site of infection or colonizationDisseminates hematogenouslyLyses desmosomes of granular cells in the superficial epidermisResults 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 syndromePemphigus neonatorumBullous impetigoGeneralized 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:NasopharynxUrinary tractMinor skin abrasionsCircumcision siteConjunctivitisUmbilicus/omphalitisImpetigoEndocarditis and septicemia
  • Often no focus identified

Diagnosis

Signs and Symptoms

  • Constitutional symptoms:MalaiseFeverIrritabilityChild may appear well, ill, or overtly toxicAbrupt onset
  • Scarlatiniform erythematous rash (sandpaper like) resembling a "sunburn " ¯ " ”erythroderma
  • Exquisitely tender skin
  • Areas of prominence:Around the flexor areas of the neckIntertriginous areas, especially axilla and groinNear the eyes and mouthIncreased 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 rashInitially over flexures (axillae, groin, body orifices)Bullae migrate through epidermis with light lateral pressure; epidermis separates with minor pressure (Nikolsky sign).Rupture within hoursEpidermis 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:HypothermiaFluid and electrolyte imbalanceSecondary infectionPneumoniaSepticemiaCellulitisOsteomyelitis

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 syndromeConsistent 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 membranesStrawberry tonguePainful 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 symptomsMucous membrane and multiorgan involvement
  • Toxic epidermal necrolysis or drug eruption:Much more common in adultsSeverely afflicted mucous membranesFull-thickness epidermal necrosis
  • Dermatologic:Erythema multiformeEpidermolysis hyperkeratosisEpidermolysis bullosaPemphigus vulgaris
  • Scald injury
  • Secondary rash of an underlying disorder:LymphomaAspergillosisIrradiationGraft-versus-host reactionKawasaki 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:CefazolinNafcillinVancomycin if methicillin-resistant S. aureus (MRSA) suspected
  • Oral antibiotics for mild involvement:DicloxacillinErythromycinCephalexin

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)