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Staphylococcal Scalded Skin Syndrome

(Ritter Disease)

By

Wingfield E. Rehmus

, MD, MPH, University of British Columbia

Last review/revision Feb 2021 | Modified Sep 2022
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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 patients 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 type 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 also 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

The initial lesion is usually superficial and crusted. Within 24 hours, the surrounding skin becomes painful and scarlet, 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 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

  • Biopsy

  • 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 will yield a pathogen.

Differential diagnosis

Differential diagnosis includes drug hypersensitivity, viral exanthemas, scarlet fever, thermal burns, genetic bullous diseases (eg, some types of epidermolysis bullosa), acquired bullous diseases (eg, pemphigus vulgaris, bullous pemphigoid), and toxic epidermal necrolysis ( see Table: Differentiating Staphylococcal Scalded Skin Syndrome (SSSS) and Toxic Epidermal Necrolysis (TEN) Differentiating Staphylococcal Scalded Skin Syndrome (SSSS) and Toxic Epidermal Necrolysis (TEN) Differentiating Staphylococcal Scalded Skin Syndrome (SSSS) and Toxic Epidermal Necrolysis (TEN) 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) ). The mucosal surfaces are spared in staphylococcal scalded skin syndrome; however, they are affected in Stevens-Johnson syndrome and toxic epidermal necrolysis.

Table

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

Feature

SSSS

TEN

Patients affected

Infants, young children, immunocompromised adults

Older patients

Patient history

Recent staphylococcal infection

Drug 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

  • Antibiotics

  • Gel dressings for weeping lesions

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.

Penicillinase-resistant antistaphylococcal antibiotics given IV must be started immediately. Nafcillin 25 mg/kg/dose every 12 hours for neonates < 2 kg, 25 mg/kg/dose every 8 hours for neonates > 2 kg, and 25 to 50 mg/kg/dose every 6 hours (maximum daily dose 12 g per day) for older children is given until improvement is noted, followed by oral cloxacillin 12.5 mg/kg every 6 hours (for infants and children weighing 20 kg) and 250 to 500 mg every 6 hours (for older children). Oxacillin and cephalosporins (eg, cefazolin) are alternatives. Most cases are caused by methicillin-susceptible S. aureus (MSSA), but vancomycin, linezolid, or other drugs 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 failing to respond to initial therapy. 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, and the number of dressing changes should be minimized.

Key Points

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

  • Do a biopsy and 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
Vancocin, Vancocin Powder, VANCOSOL
Zyvox, Zyvox Powder, Zyvox Solution
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