NORD gratefully acknowledges Bernard A. Cohen, MD, Division of Pediatric Dermatology, Johns Hopkins Children's Center, for assistance in the preparation of this report.
Staphylococcal scalded skin syndrome (SSSS) is a disorder that develops because of a toxin produced by a staphylococcal infection. A toxin is a harmful substance that causes disease when it enters tissues of the body. In SSSS the toxin spreads to the skin through the blood stream and specifically binds to a target protein very high in the epidermis (outer layer of the skin) producing total body reddening of the skin and blistering and sloughing of the skin resembling a hot water burn or scalding of the skin. The top layer of the skin may peel off and shed. Affected individuals may also experience nonspecific symptoms such as fever (usually low grade), chills, and weakness. Unlike similar disorders that cause a scalded skin appearance, the mucous membranes are not affected. Infants and younger children are most susceptible, because they lack antibodies to the toxin and may be slow to clear the toxin-antibody complex in their immature kidneys, but the disorder can also occur in certain older children or adults such as people who have compromised immune systems or insufficient kidney (renal) function. Staphylococcal scalded skin syndrome is caused by toxins produced by certain strains (most commonly phage group 2 strains 55 and 71) of the bacterial germ Staphylococcus aureus.
Initial symptoms can include fever (usually low grade), generalized redness, and tenderness of the skin. Onset of symptoms is usually rapid. Some individuals may experience nonspecific symptoms that develop before the skin symptoms including a sore throat and inflammation of the eyelids known as conjunctivitis.
Initially the affected skin may have a sandpaper-like feel before becoming red and wrinkled. Areas prone to movement are most commonly initially involved. In children, the area around the mouth, eyes and ears is often affected. In infants, the diaper area and the area around the bellybutton are most often affected. The rash spreads rapidly with a propensity to affect the area around the mouth (perioral area) and areas where the skin creases, especially on the legs, arms, groin and neck. The top layer of the epidermis, which is the top layer of the skin, may separate (detach) from the underlying layers resulting in loose blisters and shallow erosions (sores). Affected skin may slough off in sheets. Sloughing results in the exposure of moist, reddish tissue very close to the top of the epidermis and gives the skin a scalded or burned appearance. The application of gentle pressure to the skin will also cause sloughing which is known as a Nikolsky sign.
Affected individuals may also experience additional symptoms including chills, weakness, aches and pain of the joints and muscles, fluid loss through the damaged skin, and a general feeling of poor health (malaise). The loss of the top of the epidermis, which serves as a protective barrier, carries a risk of developing sepsis, a serious, potentially life-threatening infection of the blood and tissues of the body. However, in otherwise healthy children the risk of sepsis is rare. Pneumonia can also potentially occur.
The severity of the disorder is highly variable. Staphylococcal scalded skin syndrome can cause mild disease or potentially it can progress to cause life-threatening complications. Such severe complications are rare in children, with the mortality rate below 5%. However, the mortality rate is higher in adults, due primarily to additional factors including the presence of other health issues (e.g. weakened immune system, poor kidney health).
Staphylococcal scalded skin syndrome is caused by a Staphylococcus or “Staph” infection. Staphylococcus is a type of bacterium of which there are more than 30 different varieties. Staphylococcus aureus is the most common form associated with disease. Staphylococcus aureus is commonly found on human skin and begins colonization immediately after birth. Usually, this bacterium resides on the skin and mucous membranes of humans but does no harm. However, it does predispose an individual to infection, especially when given the opportunity to break through the skin. Staphylococcus aureus is the underlying infection in individuals with staphylococcal scalded skin syndrome. However, in many healthy children no underlying bacterial infection can be detected clinically.
Symptoms develops because a Staphylococcus aureus infection (or often only colonization when the Staph germ does not cause infection but makes toxin) releases toxins into the blood at the primary site of infection or colonization. These toxins spread to the skin and damage the upper part of the epidermis (outer part of the outer layer of the skin). Specifically, the toxins damage desmoglein 1, a molecule essential for epidermal cells to stick together (adhere) and form a protective barrier. Damaged desmoglein 1 prevents epidermal cells from sticking together causing the upper level of the epidermis to break apart and eventually pull away (detach) from the rest of the epidermis and the dermis (the layer of the skin beneath the epidermis which attaches to the underlying fat). Local release of the toxin into the skin results in bullous impetigo (see Related Disorders below) at the site of primary infection or colonization. When these toxins enter the bloodstream and spread to affect the skin in other areas of the body, staphylococcal scalded skin syndrome develops.
In newborns, the initial lesions are often in the diaper area or around the umbilical cord. In older children, the face is often the initial site of the rash.
Staphylococcal scalded skin syndrome affects males and females in equal numbers. The incidence, which has doubled in the last decade (based on national hospitalization data), is estimated to be between .09 and .56 per 1,000,000 individuals in the general population. However, these estimates may reflect cases reported in the medical literature and the disorder most likely is more common in the United States than estimated, particularly in infants and young children. The majority of cases are in children under the age of 6. Newborns (neonates) are at particular risk because they do not have fully developed immune systems, do not have neutralizing antibodies for the toxin, and their kidneys cannot fully clear toxins from the body yet. For similar reasons certain adults, specifically adults with a compromised immune system or poor kidney function, are at a greater risk than the general population of developing the disorder.
A diagnosis of staphylococcal scalded skin syndrome is based upon identification of characteristic symptoms, a thorough clinical evaluation, and a detailed patient history. Although not usually necessary, in some cases, a skin biopsy, in which a tiny piece of affected skin is removed and studied under a microscope, may be performed. A biopsy can reveal non-inflammatory superficial splitting of the epidermis, which is indicative of the disorder and can differentiate it from similar disorders.
Cultures can be taken from areas that harbor the bacterial germ including the conjunctiva (corners of the eyes), nasal passages, umbilicus, the upper area of the throat that connects with the nasal passages (nasopharynx area. Rarely underling serious infections such as pneumonia, meningitis, arthritis, and deep skin infection can trigger SSSS, and cultures may need to be taken from these sites. Cultures from blisters (bullae) and skin erosions are usually sterile, because there are triggered by toxin and not direct bacterial infection.
A complete blood count (CBC) can reveal elevated levels of white blood cells or an elevated erythrocyte sedimentation rate, which measures how long it takes red blood cells (erythrocytes) to settle in a test tube over a given period. However, neither the white blood cell count nor the erythrocyte sedimentation rate are elevated in all individuals.
Treatment is directed toward the specific symptoms that are apparent in each individual and may require the coordinated efforts of a team of specialists. Hospitalization may be required. However, older infants and children who are still eating and drinking well can often be treated as outpatient with local skin care and oral antibiotics. Rarely, severe cases may require treatment in a burn center, but aggressive debridement of blisters and erosions should be avoided, since in healthy individuals the superficial sloughing of the skin heals quickly and trauma to the skin may prolong healing.
Most individuals respond to oral or intravenous antibiotic therapy, specifically penicillase-resistance antibiotics with activity against Staphylococcal infections. Initial antibiotics therapy may include nafcillin, oxacillin or cephalosporin, and oral antibiotics should be considered in healthy patients who are still taking fluids well. In areas with a high prevalence of methicillin-resistant Staphylococcal aureus (MRSA) infection, vancomycin may be considered. Vancomycin can also be used for individuals who do not respond to initial therapy. Interestingly, most cases (over 95%) are caused by methicillin sensitive Staphylococcus aureus bacteria, and resistance to clindamycin has been increasing and may be over 20% in many areas. Topical agents such as mupirocin can be used as adjunct therapy at the site of colonization in an attempt to eradicate colonization.
Exposed, damaged areas should be treated with gentle ointments, such as petroleum jelly, that soothe and moisturize the skin (emollients). Emollients should be applied as often as possible to the lips to facilitate oral intake. Topical wound care should be conservative in general and dressings should be changed as infrequently as possible and in many areas only emollients may be required. In otherwise healthy children healing usually begins in 24-48 hours, and is often complete in a few more days. Seven to 10 days later many patients will develop innocent dry peeling.
Fluid replacement with electrolytes may be necessary in patients who are unable to eat or drink.
Pain management may be required for a few days with specific medications such as paracetamol and acetominophen. Nonsteroidal anti-inflammatories (NSAIDs) should not be given for pain because they may reduce kidney function and complicate the disorder. Steroids should not be given because they can worse immune system function.
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