What is the difference in management between Staphylococcal Scalded Skin Syndrome (SSSS) and Stevens-Johnson Syndrome (SJS)?

Distinguishing SSSS from SJS: Critical Differences in Management

The most critical management difference is that SSSS requires immediate anti-staphylococcal antibiotics while SJS requires immediate drug discontinuation and specialized supportive care—making accurate differentiation essential as these conditions demand fundamentally opposite therapeutic approaches. 1, 2

Key Clinical Distinctions

Mucosal Involvement: The Primary Differentiator

• SSSS characteristically spares mucous membranes, while SJS presents with prominent erosive and hemorrhagic mucositis affecting the eyes, mouth, nose, and genitalia 1
• Absence of mucosal involvement clinically distinguishes SSSS from SJS/TEN in the vast majority of cases 1

Histopathologic Plane of Cleavage

• SSSS shows intraepidermal cleavage within the stratum granulosum due to exfoliative toxins targeting desmoglein 1 2
• SJS demonstrates subepidermal cleavage with full-thickness epidermal necrolysis and extensive keratinocyte apoptosis 1
• When diagnostic uncertainty exists, skin biopsy or frozen section of blister roof definitively identifies the plane of cleavage 1

Age and Etiology

• SSSS predominantly affects children under 5 years and is caused by toxigenic Staphylococcus aureus strains producing exfoliative toxins 2
• SJS occurs across all ages but has different triggers: drugs in adults (allopurinol, sulfonamides, anticonvulsants, NSAIDs) and infections (Mycoplasma pneumoniae) more commonly in children 1, 3

Management Algorithms

SSSS Management Protocol

Immediate antibiotic therapy is the cornerstone of SSSS treatment:

• Start empiric IV anti-staphylococcal antibiotics immediately: nafcillin, oxacillin, or flucloxacillin as first-line agents 2
• For penicillin allergy: use clarithromycin or cefuroxime 2
• In critically ill patients or high MRSA prevalence areas: initiate vancomycin 2
• Obtain cultures from potential infection sources (skin, nasopharynx, conjunctiva) to guide definitive therapy 2
• Supportive care includes fluid management, pain control, and gentle wound care 2
• No need for specialized burn unit transfer in most cases unless complications develop 2

SJS/TEN Management Protocol

Drug discontinuation and specialized supportive care are paramount:

Immediate Actions

• Discontinue ALL suspected culprit drugs immediately—this is the single most critical intervention 1, 4
• Calculate SCORTEN within 24 hours to predict mortality risk (scores 0-7 correlate with 1-99% mortality) 4, 5
• Transfer patients with >10% body surface area epidermal detachment to specialized burn unit or ICU with multidisciplinary expertise within hours 1, 4
• Delayed transfer significantly increases mortality 1, 4

Specialized Environment Requirements

• Barrier nursing in temperature-controlled isolation room (25-28°C) on pressure-relieving mattress 4, 6
• Multidisciplinary team including dermatology, intensive care, ophthalmology, and specialist skincare nursing 1, 4

Supportive Care Framework

• Fluid resuscitation: careful balance to prevent end-organ hypoperfusion without causing pulmonary/cutaneous edema 4
• Wound management: minimize shearing forces, leave detached epidermis in situ as biological dressing, apply greasy emollients every few hours 4
• Infection prevention: DO NOT use prophylactic antibiotics—this increases colonization with resistant organisms, particularly Candida albicans 4, 6
• Use targeted antimicrobial therapy only when clinical infection signs appear 4, 6
• Ophthalmology consultation within 24 hours with daily examinations—apply preservative-free lubricant drops every 2 hours 4
• Continuous enteral nutrition: 20-25 kcal/kg daily during acute phase, increasing to 25-30 kcal/kg during recovery 4

Immunomodulatory Therapy Considerations

• Cyclosporine (3 mg/kg daily for 10 days) has shown mortality benefit in multiple studies 4
• Early methylprednisolone pulse therapy may be beneficial if started within 72 hours of onset 4
• IVIg evidence is equivocal—pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75) 4
• Avoid thalidomide—associated with excess deaths in randomized trials 4

Common Diagnostic Pitfalls

When to Perform Skin Biopsy

• Always biopsy when diagnostic uncertainty exists, particularly in children where SSSS is a common mimicker 1
• Send one specimen for routine histopathology and a second unfixed specimen for direct immunofluorescence to exclude immunobullous disorders 1
• Frozen section can provide rapid differentiation in urgent cases 1

Critical Red Flags for SJS (Not SSSS)

• Mucosal involvement (oral, ocular, genital erosions) 1
• Purpuric macules or flat atypical target lesions 4
• Positive Nikolsky sign with painful cutaneous tenderness 1
• Recent drug exposure (5-28 days prior to onset) 1
• Systemic symptoms including fever, malaise, respiratory symptoms 1

Critical Red Flags for SSSS (Not SJS)

• Young age (typically <5 years) 2
• Complete absence of mucosal involvement 1, 2
• Periorificial crusting and scabs 2
• Tender erythroderma with desquamation in friction zones 2
• Scalded appearance of skin 2

Prognostic Differences

SSSS Prognosis

• Generally excellent with prompt antibiotic therapy 2
• Mortality rare in children with appropriate treatment 2
• Complete resolution expected without long-term sequelae 2

SJS/TEN Prognosis

• SJS mortality: 1-5%; TEN mortality: 25-35% 5, 3
• Higher mortality in elderly patients and those with extensive epidermal detachment 5, 3
• More than 50% of TEN survivors experience long-term sequelae affecting quality of life, particularly ophthalmic, mucocutaneous, and psychological complications 1, 3
• Recurrence occurs in up to 18% of pediatric cases, often related to recurrent infections 1