Staphylococcal scalded skin syndrome

OVERVIEW: What every practitioner needs to know

Are you sure your patient has Staphylococcal Scalded Skin Syndrome? What are the typical findings for this disease?

Diffuse erythema with accentuation in flexures and perioral region

Crusting and radial fissuring of perioral, perinasal, and/or periocular regions (but absence of true mucous membrane lesions)
Superficial desquamation
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Skin tenderness
Fever
Irritability
Positive Nikolsky sign (slight rubbing of the skin results in desquamation)
Rapid progression
Bullae so flaccid that it is more common to see erosions than intact bullae
Conjunctivitis, sore throat, cellulitis, depending on focus of infection

What is another name for Staphylococcal Scalded Skin Syndrome?

Ritter disease

How can Staphylococcal Scalded Skin Syndrome (SSSS) be distinguished from Toxic Epidermal Necrolysis?

The level of skin cleavage is more superficial in SSSS (at the level of the stratum granulosum) than in toxic epidermal necrolysis, which demonstrates cleavage of the entire epidermis. This difference can be seen on skin biopsy, or oftentimes just by clinical examination.

On physical examination, SSSS demonstrates perioral involvement, while toxic epidermal necrolysis affects the lips themselves.

Mortality is much higher in toxic epidermal necrolysis than SSSS (30% vs. less than 5%).

While SSSS has a predilection for infants and young children, toxic epidermal necrolysis typically affects adults or older children.

Recovery is very quick in SSSS (days to weeks), but quite slow in toxic epidermal necrolysis (months; more time is needed for the much more significant re-epithelialization that must occur).

What other disease/condition shares some of these symptoms?

Toxic epidermal necrolysis

Stevens-Johnson syndrome

Scarlet fever, staphylococcal scarlet fever

Toxic shock syndrome

Epidermolysis bullosa

Kawasaki disease

Recurrent toxin-mediated perineal erythema

Burns, traumatic

Child abuse

Nutritional deficiencies, especially zinc deficiency

Epidermolytic hyperkeratosis

Erythema multiforme

Pemphigus foliaceus and other immunobullous disorders

Bullous mastocytosis

Porphyrias

Bullous impetigo

What caused this disease to develop at this time?

SSSS usually occurs in children < 5 years old, most often children < 2 years old. This may be due to decreased renal clearance of epidermolytic toxins, lack of antibodies to these toxins, or increased amounts of desmoglein-1 in the skin (the target of epidermolytic toxins) at this age.

The primary source of infection is commonly the head and neck region, the umbilical area, or the circumcision site.

Outbreaks in nurseries or intensive care units may occur.

SSSS may occur with infection with particular strains of
S. aureus, which produce the epidermolytic toxin(s), e.g., phage groups 1, 2, or 3, especially group 2 strains 71 and 55.

Renal failure and immunosuppression may predispose a person to SSSS.

Parents and healthcare workers may be asymptomatic carriers of causative strains.

No gender predilection has been seen in children, but there is a slight male predominance among affected adults.

There may be a predominance among Caucasians relative to African Americans.

The incidence appears to be higher in developing countries.

What laboratory studies should you request to help confirm the diagnosis? How should you interpret the results?

The diagnosis may be confirmed by culturing S. aureus from foci of infection, e.g., umbilicus, nostrils, conjunctivae.

Sometimes a skin biopsy may help confirm the superficial cleavage plane (at the stratum granulosum) typical of SSSS.

Culturing blisters is not helpful – the blisters are due to circulating toxins, not local infections.

Bacteremia is uncommon.

Would imaging studies be helpful? If so, which ones?

None, except chest radiography if there is concern for pneumonia as a complication.

If you are able to confirm that the patient has Staphylococcal Scalded Skin Syndrome, what treatment should be initiated?

Most young children with SSSS should be hospitalized to treat the skin and to provide antibiotic therapy.
Classically, empiric IV antibiotics such as a penicillinase-resistant penicillin, first or second generation cephalosporin, and clindamycin (may limit toxin production) have been recommended.
Consider including an antibiotic effective against methicillin-resistant
S. aureus(MRSA).
Adjust antibiotics based on local resistance patterns and culture results.
Patients can usually transition to oral antibiotics within a few days.
Closely monitor electrolytes and hemodynamics.
Bland emollients.
Minimize handling of patients.
Mupirocin ointment twice daily at focus of infection.
Contact isolation.
Pain management, e.g., sedatives and analgesics.
Avoid adhesive wound dressings (as often used in burn patients).
Non-sticky, sterile undersheets.
Daily dressing changes.
In outbreaks, treating parents and healthcare workers with intranasal mupirocin is important.
Do not apply antiseptics or antibiotics over large areas of erosions to avoid systemic absorption and toxicity.
Do not give systemic corticosteroids.
For newborns, use incubators with high humidity.
Enteral feedings if needed.
Consider whether a close contact is a carrier, e.g., in a nursery outbreak.
Avoid non-steroidal anti-inflammatory agents (NSAIDs).
Toxic patients may benefit from fresh frozen plasma or intravenous immunoglobulins because these products may contain antibodies against the causative epidermolytic toxins.

What are the adverse effects associated with each treatment option?

Drug rashes and GI upset may occur with antibiotics.

Fluid overload.

Hyponatremia.

What are the possible outcomes of Staphylocococcal Scalded Skin Syndrome?

The mortality rate is less than 5%.

The prognosis is worse in infants than older children.

Many children respond rapidly and completely recover in 2-3 weeks.

Recurrence is very rare because of the development of immunity.

What causes this disease and how frequent is it?

SSSS is due to infection with certain S. aureus strains which produce epidermolytic toxin(s) (also known as exfoliative toxin)

Epidemiological studies are lacking.

Approximately 5% of S. aureus strains produce the epidermolytic toxin(s) responsible for SSSS.

SSSS typically affects young children, especially less than 2 years old.

Genetic risk factors are not yet defined.

Seasonal variation has not been described.

How do these pathogens/genes/exposures cause the disease?

Most of the signs and symptoms of SSSS are due to hematogenous spread of two particular epidermolytic toxins, ET-A (chromosomally encoded) and ET-B (plasmid derived). ET-B is most frequently implicated in SSSS, whereas ET-A is more frequently implicated in bullous impetigo. This may be related to the finding of higher titers of neutralizing antibodies to ET-A than ET-B in healthy populations.

ET-A and ET-B are serine proteases that target and cleave desmoglein-1, a cell adhesion molecule in the superficial epidermis. Locally, this cleavage allows S. aureus to penetrate the skin barrier. ET-A and ET-B may also act as superantigens, although only at very high concentrations. Moreover, the superantigen properties of the epidermolytic toxins are probably not important factors in the disease manifestations of SSSS.

There are two other epidermolytic toxins, ET-C and ET-D, but they do not seem to be important in the pathogenesis of SSSS.

Other clinical manifestations that might help with diagnosis and management?

Sandpaper/scarlatiniform rash

Wrinkled appearance of skin

Edematous appearance

Focus of infection may be purulent

What complications might you expect from the disease or treatment of the disease?

Secondary infections, sepsis, hypothermia, electrolyte imbalances, dehydration, pneumonia.

Scarring does not occur in SSSS since the cleavage plane is so superficial.

Are additional laboratory studies available; even some that are not widely available?

A frozen biopsy specimen of desquamating epidermis may demonstrate only the corneal layer of epidermis, rapidly supporting the diagnosis.

A Tzanck smear may demonstrate acantholysis suggestive of SSSS.

Typing the epidermolytic toxin may be helpful in epidemiological studies of outbreaks (e.g., via polymerase chain reaction (PCR), enzyme-linked immunosorbent assay (ELISA),or slide latex agglutination).

White blood count and erythrocyte sedimentation rate may be elevated.

How can Staphylococcal Scalded Skin Syndrome be prevented?

Prompt evaluation and treatment of staphylococcal infections, as well as treatment of asymptomatic carriers, may prevent spread to other individuals.

Infection control measures should be employed to avoid outbreaks, e.g., barrier protocols, hand washing; treatment of infected healthcare workers or family; mupirocin ointment for nasal carriers.

What is the evidence?

Berk, DR, Bayliss, SJ. “MRSA, staphylococcal scalded skin syndrome, and other cutaneous bacterial emergencies”. Pediatr Ann. vol. 39. 2010. pp. 627-33. (This article reviews dermatological bacterial emergencies including SSSS, cutaneous MRSA infection, and toxic shock syndromes.)

Nishifuji, K, Sugai, M, Amagai, M. “Staphylococcal exfoliative toxins: "molecular scissors" of bacteria that attack the cutaneous defense barrier in mammals”. J Dermatol Sci. vol. 49. 2008. pp. 21-31. (This recent article reviews the significance and targets for the various staphylococcal exfoliative toxin isoforms, with focus on the molecular mechanism and animal models.)

Hubiche, T, Bes, M, Roudiere, L. “Mild staphylococcal scalded skin syndrome: an underdiagnosed clinical disorder”. Br J Dermatol. vol. 166. 2012. pp. 213-5. (This article proposes that SSSS may present in a mild form that may actually be more common than the classical, severe form of SSSS.)

Ongoing controversies regarding etiology, diagnosis, treatment

SSSS likely exists along a spectrum.

The localized version of SSSS is bullous impetigo, which occurs when there is no significant hematogenous spread of the epidermolytic toxins.

Classical SSSS occurs when there is widespread hematogenous spread and skin accumulation of the epidermolytic toxins.

Recently, the existence of a mild version of SSSS has been proposed as an underdiagnosed disorder, which may be even more common than classical SSSS. It is hypothesized that the occurrence of mild vs. severe disease may be related to the particular S. aureus clone, or host factors such as age or immune status.

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