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Staphylococcal Scalded Skin Syndrome

By

Wingfield E. Rehmus

, MD, MPH, University of British Columbia

Reviewed/Revised Jun 2023
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Topic Resources

Staphylococcal scalded skin syndrome is an acute epidermolysis caused by a staphylococcal toxin. Infants and children are most susceptible. Symptoms are widespread bullae with epidermal sloughing. Diagnosis is by examination and sometimes biopsy. Treatment is antistaphylococcal antibiotics and local care. Prognosis is excellent with timely treatment.

Staphylococcal scalded skin syndrome almost always affects children < 6 years (especially infants); it rarely occurs in older adults unless they have renal failure or are immunocompromised. Epidemics may occur in nurseries, presumably transmitted by the hands of personnel who are in contact with an infected infant or who are nasal carriers of Staphylococcus aureus. Sporadic cases also occur.

Staphylococcal scalded skin syndrome is caused by group II coagulase-positive staphylococci, usually phage types 55 and 71, which elaborate exfoliatin (also called epidermolysin), a toxin that splits the upper part of the epidermis just beneath the granular cell layer by targeting desmoglein-1 (see Staphylococcal Infections Staphylococcal Infections Staphylococci are gram-positive aerobic organisms. Staphylococcus aureus is the most pathogenic; it typically causes skin infections and sometimes pneumonia, endocarditis, and osteomyelitis... read more Staphylococcal Infections ).

The primary infection often begins during the first few days of life in the umbilical stump or diaper area; in older children, the face is the typical site. Toxin produced in these areas enters the circulation and affects the entire skin.

Symptoms and Signs of Staphylococcal Scalded Skin Syndrome

When a localized area of infection can be found (eg, impetigo), the initial lesion is usually superficial and crusted. Within 24 hours, the surrounding skin becomes painful and erythematous, changes that quickly spread to other areas. The skin may be exquisitely tender and have a wrinkled tissue paper–like consistency.

Large, flaccid blisters arise on the erythematous skin and quickly break to produce erosions. Blisters are frequently present in areas of friction, such as intertriginous areas, buttocks, hands, and feet. Intact blisters extend laterally with gentle pressure (Nikolsky sign).

The epidermis may peel easily, often in large sheets. Widespread desquamation occurs within 36 to 72 hours, and patients may become very ill with systemic manifestations (eg, malaise, chills, fever). Desquamated areas appear scalded. Loss of the protective skin barrier can lead to sepsis Sepsis and Septic Shock Sepsis is a clinical syndrome of life-threatening organ dysfunction caused by a dysregulated response to infection. In septic shock, there is critical reduction in tissue perfusion; acute failure... read more and to fluid and electrolyte imbalance.

Manifestations of Staphylococcal Scalded Skin Syndrome (SSSS)

Diagnosis of Staphylococcal Scalded Skin Syndrome

  • Clinical examination

  • Cultures from areas of suspected primary infection

Diagnosis of staphylococcal scalded skin syndrome is suspected clinically, but confirmation may require biopsy (frozen section may give earlier results). Specimens show noninflammatory superficial splitting of the epidermis.

Cultures should be taken from the conjunctiva, nasopharynx, blood, urine, and areas of possible primary infection, such as the umbilicus in a neonate or suspect skin lesions. Cultures should not be taken from bullae because they are sterile, unlike in bullous impetigo, where cultures of the blister fluid yield a pathogen.

Differential diagnosis

Differential diagnosis includes drug hypersensitivity Drug Hypersensitivity Drug hypersensitivity is an immune-mediated reaction to a drug. Symptoms range from mild to severe and include rash, anaphylaxis, and serum sickness. Diagnosis is clinical; skin testing is occasionally... read more , viral exanthemas, scarlet fever Scarlet fever Streptococci are gram-positive aerobic organisms that cause many disorders, including pharyngitis, pneumonia, wound and skin infections, sepsis, and endocarditis. Symptoms vary with the organ... read more Scarlet fever , thermal burns, genetic bullous diseases (eg, some types of epidermolysis bullosa Types of epidermolysis bullosa Epidermolysis bullosa is a group of 4 very rare genetic diseases and their subtypes. Epithelial fragility and easy blistering of skin and mucous membranes usually manifest at birth or in infancy... read more Types of epidermolysis bullosa ), acquired bullous diseases (eg, pemphigus vulgaris Pemphigus Vulgaris Pemphigus vulgaris is an uncommon, potentially fatal, autoimmune disorder characterized by intraepidermal blisters and extensive erosions on apparently healthy skin and mucous membranes. Diagnosis... read more Pemphigus Vulgaris , bullous pemphigoid Bullous Pemphigoid Bullous pemphigoid is a chronic autoimmune skin disorder resulting in generalized, pruritic, bullous lesions in older adults. Mucous membrane involvement is rare. Diagnosis is by skin biopsy... read more Bullous Pemphigoid ), and toxic epidermal necrolysis Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) Stevens-Johnson syndrome and toxic epidermal necrolysis are severe cutaneous hypersensitivity reactions. Drugs, especially sulfa drugs, antiseizure drugs, and antibiotics, are the most common... read more Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) (see table and see Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis (TEN) Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) Stevens-Johnson syndrome and toxic epidermal necrolysis are severe cutaneous hypersensitivity reactions. Drugs, especially sulfa drugs, antiseizure drugs, and antibiotics, are the most common... read more Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) ).

Table

Differentiating Staphylococcal Scalded Skin Syndrome (SSSS) and Toxic Epidermal Necrolysis (TEN)

Feature

SSSS

TEN

Patients affected

Infants, young children, and adults who are immunocompromised

Older adults

Patient history

Recent staphylococcal infection

Medication use

Level of epidermal cleavage (blister formation)*

Within the granular cell (outermost) layer of the epidermis

Between the epidermis and dermis or at the level of the basal cell

* Determined by Tzanck test or by a frozen section of a fresh specimen.

Treatment of Staphylococcal Scalded Skin Syndrome

  • Antibiotics

  • Gel dressings for weeping lesions

Penicillinase-resistant antistaphylococcal antibiotics given IV must be started immediately. Typically nafcillin is given until improvement is noted, followed by oral cloxacillin. Oxacillin and cephalosporins (eg, cefazolin) are alternatives. Most cases are caused by methicillin-susceptible S. aureus (MSSA), but vancomycin, linezolid, or other antibiotics effective against MRSA MRSA and purulent or complicated cellulitis Cellulitis is acute bacterial infection of the skin and subcutaneous tissue most often caused by streptococci or staphylococci. Symptoms and signs are pain, warmth, rapidly spreading erythema... read more MRSA and purulent or complicated cellulitis should be considered in areas with a high prevalence of methicillin-resistant S. aureus (MRSA) or in patients whose initial therapy failed. Corticosteroids are contraindicated.

Emollients (eg, white petrolatum) are sometimes used to prevent further insensible water loss from ulcerated skin. However, topical therapy and patient handling must be minimized.

If disease is widespread and lesions are weeping, the skin should be treated as for burns Treatment Burns are injuries of skin or other tissue caused by thermal, radiation, chemical, or electrical contact. Burns are classified by depth (superficial and deep partial-thickness, and full-thickness)... read more Treatment . Hydrolyzed polymer gel dressings may be very useful; the number of dressing changes should be minimized. Patients should be monitored and treated for complications similar to those that occur with burns (eg, fluid and electrolyte imbalance, sepsis).

Prognosis for Staphylococcal Scalded Skin Syndrome

With prompt diagnosis and therapy, death rarely occurs; the stratum corneum is quickly replaced, and healing usually occurs within 5 to 7 days after start of treatment.

Key Points

  • Generalized desquamation and systemic illness most often indicate staphylococcal scalded skin syndrome (SSSS) in infants and young children (and occasionally in adults who are immunocompromised), whereas in older adults, they most often indicate toxic epidermal necrolysis.

  • Culture the conjunctiva, nasopharynx, blood, urine, and areas of possible primary infection, such as the umbilicus and suspect skin lesions.

  • Treat patients with antistaphylococcal antibiotics and, if disease is widespread, in a burn unit if possible.

  • Monitor and treat for complications similar to those that occur with burns (eg, fluid and electrolyte imbalance, sepsis).

Drugs Mentioned In This Article

Drug Name Select Trade
Nallpen
No brand name available
Ancef, Kefzol
FIRVANQ, Vancocin, Vancocin Powder, VANCOSOL
Zyvox, Zyvox Powder, Zyvox Solution
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NOTE: This is the Professional Version. CONSUMERS: View Consumer Version
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