Staphylococcal scalded skin syndrome (SSSS), also known as Ritter’s disease, is a serious skin condition caused by certain strains of Staphylococcus aureus (S. aureus) bacteria. It causes large, peeling blisters that look burnt or scalded. SSSS mostly affects infants and toddlers but may also affect adults with severely weakened immune systems.

SSSS is mainly diagnosed by its appearance, although lab tests may be ordered to rule out other causes. While children treated with intravenous (IV) antibiotics usually recover within two weeks, adults may be harder to treat and are far more likely to develop serious complications, including death.

Causes of SSSS

SSSS is a complication of a staph infection. It is caused by certain strains of Staphylococcus aureus, a relatively common bacterium that frequently inhabits the eyes, ears, navel, and groin.

While all strains of S. aureus produce toxins, only around 5% secrete specific types known as exfoliative toxins A and B (ETA and ETB). These are especially damaging to the skin and can lead to SSSS and a related, milder condition known as bullous impetigo.

ETA and ETB act like scissors, cutting away proteins called desmoglein-3 that hold together the outermost layers of skin. The separation of these layers leads to the formation of large, thin blisters that erupt and peel away in sheets (desquamate).

In Children

SSSS mainly affects children under age 5 who, because of their age, have immature immune systems. Newborns are usually safe as they have naturally high levels of desmoglein-3 in their skin. But, within the first few weeks of birth, desmoglein-3 levels rapidly drop, increasing the risk of SSSS in infants and toddlers.

SSSS outbreaks are common in nurseries and day care centers due to the frequent skin-to-skin contact that can transmit the bacterium. These outbreaks tend to occur during cold and flu season, and are most common in autumn.

In Adults

SSSS is extremely rare in adults as most will have built up natural immune defenses to ETA and ETB. When SSSS does occur, it is almost always in immunocompromised people who lack these defenses, most especially those with:

According to research, only around 1 in every 1 million adults in the United States gets SSSS each year. Unlike children, SSSS in adults is not influenced by season and can occur at any time.

Symptoms of SSSS 

SSSS symptoms can differ in children and adults, with adults being at greater risk of severe and potentially life-threatening complications.

In Children

SSSS in children typically starts with prodromal (preliminary) symptoms such as irritability, fatigue, and fever 24 to 48 hours before the outbreak of rash. The outbreak is most common in and around mucous membranes as well as disrupted skin, such as:

• Around the eyes, nose, or mouth
• Buttocks
• Circumcision site and other surgical sites in babies
• Diaper area in babies
• Groin
• Umbilicus stump in babies

It starts with a painful, widespread rash, followed within hours by the appearance of large, fragile blisters, called bullae. As the blisters burst, they give the skin a characteristic wrinkled appearance. SSSS is recognized by what’s known as the Nikolsky sign, in which the skin easily peels off with gentle rubbing.

SSSS usually has a second period of desquamation 10 days after the first, followed by rapid healing.

In Adults

SSSS in adults usually starts with a localized bacterial infection. Without the immune defenses to fight the infection, S. aureus can become systemic, meaning it can enter the bloodstream and affect the entire body. For this reason, SSSS can sometimes involve most or all of the body of immunocompromised adults.

The initial, localized infection can be caused by many different things, including:

As with children, SSSS in adults often starts with flu-like prodromal symptoms. However, when the blistering and peeling occur, it is often more severe. This is because desmoglein-3 lies in deep layers of the skin in adults, leading to greater tissue and fluid loss.

SSSS in adults can be extremely painful, forming thick crusts with fissures (small tears) around the mouth, nose, or eyes. The torso and limbs are also commonly affected, especially flexural surfaces like the elbows, knees, and ankles.

With appropriate treatment, SSSS in adults can also heal with little, if any, scarring.

Complications

If not treated appropriately, SSSS can lead to serious complications. This is mainly due to the loss of fluids through disrupted skin and the increased vulnerability of exposed tissues to systemic infections and disease.

Possible complications of SSSS include:

• Bacteremia (the spread of bacteria in the bloodstream)
• Cellulitis (caused when a local infection spreads to deeper tissues)
• Dehydration (depletion of fluid in the body)
• Hypothermia (an abnormal and potentially dangerous drop in body temperature)
• Hypovolemic shock (a form of shock caused by the loss of fluid volumes)
• Post-staphylococcal glomerulonephritis (kidney damage caused by a systemic S. aureus infection)
• Scarring
• Secondary infection (caused when another bacteria passes through disrupted skin)
• Sepsis (a potentially deadly immune overreaction to a systemic infection)

Importance of Diagnosis

SSSS is mainly diagnosed by the clinical signs of the disease along with a review of your symptoms and medical history.

Blood cultures may be ordered to isolate S. aureus, but doing so is usually only helpful in adults, who are more likely to have bacteremia. In children with SSSS, blood cultures are almost always negative.

A nasal, oral, anal, or periocular (eye) swab can usually isolate S. aureus in children and adults. Although uncommon, a skin biopsy (removing a sample of skin for analysis in the lab) may be ordered if there is any doubt about the cause.

Additional tests may be ordered to exclude other possible causes of symptoms, including:

Treatment

The treatment of SSSS usually requires a hospital stay during which antibiotic drugs are administered intravenously. The choice of antibiotic can vary by the person’s age, such as:

• Children with SSSS are commonly treated with nafcillin or oxacillin six times daily or cephazolin three times daily.
• Adults with SSSS are commonly treated with vancomycin twice daily.

Intravenous fluids may also be used to treat dehydration caused by fluid loss. Severe dehydration in children can also be treated with a plasma transfusion (transferring the liquid portion of blood into a person).

Gentle wound care with topical antibiotics may be recommended to aid with healing. Pain control may be achieved with Tylenol (acetaminophen) or low-dose opioid painkillers. Nonsteroidal anti-inflammatory drugs (NSAIDs) like Advil and Motrin (ibuprofen) or Aleve (naproxen) are generally avoided as they may promote bleeding of already injured skin.

Prognosis

If treated appropriately, full recovery from SSSS in children takes around two weeks. Adults with SSSS may take longer given their weakened immune state. Even so, most who recover do so with minimal or no scarring.

Although SSSS is more common in children, complications of SSSS are far more common in adults. This is because adults are almost certain to have comorbidities (co-occurring medical conditions) that placed them at risk of SSSS in the first place. Children with SSSS rarely have any such comorbidity.

Because of this, the risk of death is relatively low in children with SSSS, hovering at around 3%. By contrast, the mortality (death) rate in adults is thought to be greater than 60%, most often due to sepsis or complications of dehydration (including shock).

How to Prevent Exposure

The prevention of SSSS is focused on good hygiene to avoid exposure to Staphylococcus aureus. Most staph infections are caused by skin-to-skin contact with someone with S. aureus or objects they touched. This includes other kids in day care or caregivers in nurseries who handle babies.

To prevent getting and transmitting S. aureus:

• Wash your hands frequently with soap and warm water, including under your fingernails.
• Don’t share towels, sheets, or personal care items if someone in your household has a staph infection. 
• Keep cuts, abrasions, or rashes clean and covered until they heal.
• Shower or bathe daily, including smaller children who return from day care.

Summary

Staphylococcal scalded skin syndrome (SSSS) is a serious skin condition caused when toxins from certain strains of Staphylococcus aureus cause the skin to blister and peel in sheets. It is most common in children under 5 but can also affect adults with compromised immune systems.

SSSS is almost invariably treated in the hospital with antibiotics, IV fluids, wound care, and pain medications. Most treated individuals will recover without scarring. With that said, adults with SSSS are vulnerable to severe complications and are at far greater risk of death.