What is Staphylococcal Scalded Skin Syndrome (Ritter disease)?
Staphylococcal Scalded Skin Syndrome, also known as Ritter disease, is a condition that causes skin peeling due to toxins produced by a specific type of bacteria, called Staphylococcus. These bacteria usually enter the body from somewhere else and cause the condition. This typically appears about two days after a baby is born, and it’s unlikely to develop in children over six years old. However, it may also affect adults with weak immune systems or severe kidney disease.
This disorder is marked by serious skin peeling following a skin infection. The intensity of this condition can range from just a few blisters to extreme skin peeling causing significant body temperature drops and instability in blood circulation.
What Causes Staphylococcal Scalded Skin Syndrome (Ritter disease)?
Staphylococcal Scalded Skin Syndrome, or SSSS for short, is caused by toxins from certain types of Staphylococcus bacteria. Although at least two toxins have been identified as causing the condition, testing for the specific type of bacteria is not usually helpful or possible. The condition might start from an infection in the upper respiratory tract, ears, eyes or naval cord. In adults, it could also come from an abscess, an infected connection between an artery and a vein, or infection in the joints, among other causes. However, oftentimes, it’s not possible to identify where the infection came from.
Kids are more susceptible to SSSS due to an immature kidney system, lack of immunity and the absence of maternal antibodies, particularly in children who are not breastfed. SSSS can sometimes occur as a breakout in places like daycares or nurseries. Most of the time, the kids affected by this condition are under the age of 2.
Risk Factors and Frequency for Staphylococcal Scalded Skin Syndrome (Ritter disease)
Staphylococcal Scalded Skin Syndrome is not very common. Research in the Czech Republic found about 25 cases per 100,000 babies under the age of one. Most of the cases happen in children under two years old. However, the syndrome could strike in groups, particularly in places like nurseries. It’s rare in adults but when it does happen, it’s generally in those with a weakened immune system, such as people with HIV/AIDS, severe kidney problems or kidney failure, or cancer.
- Staphylococcal Scalded Skin Syndrome is fairly uncommon.
- In the Czech Republic, about 25 cases are seen per 100,000 babies under the age of one.
- Most cases occur in children under two years old.
- It can occur in outbreaks, often in nurseries.
- It is rare in adults, but can affect those with a weak immune system, severe kidney problems, kidney failure, or cancer.
Signs and Symptoms of Staphylococcal Scalded Skin Syndrome (Ritter disease)
Staphylococcal Scalded Skin Syndrome, or SSSS, is a skin condition that usually begins with general discomfort, fever, and irritability. After one to two days, a sore rash emerges, often starting on the face and areas of the body that bend, like the neck, groin, and armpits. This rash often develops into large, fragile blisters with a cloudy fluid or even yellow pus inside.
Unlike other similar conditions such as scarlet fever, the rash associated with SSSS is generally more sensitive, and the skin tends to peel off. The disease can sometimes only manifest as localized blisters, but it can also escalate, causing a widespread, noticeably red rash with considerable tenderness. In severe cases, the skin may form blisters and begin to shed, a condition known as desquamation.
The patient might look healthy or might show severe signs of infection like low blood pressure and shock. An important thing to remember is that Staphylococcal Scalded Skin Syndrome typically does not involve the mucous membranes, although they may appear reddened. This can differentiate it from Toxic Epidermal Necrolysis, which does involve these areas. With proper care, the skin usually recovers within two weeks.
- Irritability and fever
- Rash that typically starts on the face and body folds
- Rash develops into large, sensitive blisters
- Skin exfoliation
- Potential presentation of severe infection symptoms
- No mucous membrane involvement
- Complete skin recovery within two weeks
Testing for Staphylococcal Scalded Skin Syndrome (Ritter disease)
In order to check for a severe form of a condition known as Staphylococcal Scalded Skin Syndrome, your doctor may order some blood and urine tests, including a complete blood count and a urinalysis. These tests will help to figure out if you might have a severe infection known as sepsis. If they suspect that you may be dehydrated, they may also order a test to measure the levels of certain minerals in your blood.
Usually, if doctors test the fluid from your blisters or a sample of your blood, they wouldn’t find the bacteria that causes the skin condition. However, if you’re very ill, your doctor will take samples from any part of your body that they think may be infected to find out the kind of bacteria that’s causing your infection. They may also take a sample from the place they think the infection might have started.
Additionally, your doctor may do a skin biopsy, where they take a tiny piece of skin from one of your blisters to examine under the microscope. This test can help confirm the diagnosis and distinguish it from other conditions with similar symptoms, like toxic shock syndrome or other skin diseases.
Lastly, a chest x-ray might be done to check if an infection in your lungs might be the source of your illness.
Treatment Options for Staphylococcal Scalded Skin Syndrome (Ritter disease)
When it comes to treating infections caused by the Staphylococcus aureus bacteria, it’s essential to administer targeted antibiotics as early as possible. Examples of such antibiotics include Cefazolin, Nafcillin, and Oxacillin. Should the bacteria be resistant to methicillin (a type of antibiotic), usually indicated by recent healthcare exposure or being in an area with many such cases, then the antibiotic Vancomycin is used instead. It’s important to note that solely relying on antibiotics applied directly to the skin isn’t effective. Even with localized cases of Staphylococcal Scalded Skin Syndrome, a severe skin infection, systemic antibiotics that travel throughout the body are needed. If there’s an additional bacterial skin infection, more antibiotics will be used.
Patients can also experience dehydration, so providing fluids intravenously might be necessary. For skin care and comfort, emollients and non-sticky dressings can be applied to the skin. Also, taking care of dehydration, maintaining body temperature, and ensuring balanced nutrition are crucial steps in the healing process.
Patients with wide-spread skin issues need special attention, as they are more prone to hypothermia and fluid loss due to loss of the skin’s uppermost layer. One thing to avoid is the use of a cream called silver sulfadiazine, because it can be harmful if too much enters the body.
For minor and localized cases, patients might be able to go home and keep taking prescribed oral antibiotics under the vigilant follow-ups by their primary care physician. However, if the case is severe, hospital admission will be necessary; and admission to a burn unit can be beneficial. Those suffering from Staphylococcal Scalded Skin Syndrome need to be separated from others in the hospital to prevent spreading the infection. This level of isolation requires a well-disciplined approach to hand hygiene and a thorough cleaning of hospital instruments. It’s also essential to identify and treat individuals who might be carrying the bacteria without showing symptoms.
Favorably, patients who have their condition detected early and who receive appropriate treatment have a very promising outlook. In children, the mortality rate is less than 5%. A few days into treatment, fevers start to subside and the skin rash begins to improve. The skin usually starts peeling off within five days, completely healing within about two weeks, typically leaving no permanent damage. However, adults have a higher mortality rate, up to 59% in some cases.
What else can Staphylococcal Scalded Skin Syndrome (Ritter disease) be?
When a doctor encounters certain skin conditions, there are several possibilities they need to consider. These include:
- Bullous Impetigo
- Toxic Shock Syndrome
- Scarlet Fever
- Kawasaki Disease
- Cellulitis
- Impetigo
- Thermal Burn
- Stevens Johnson Syndrome/Toxic Epidermal Necrolysis (TEN)
- Epidermolysis Bullosa
What to expect with Staphylococcal Scalded Skin Syndrome (Ritter disease)
In general, children who undergo a medical treatment usually recover nicely with little to no scarring, and this healing typically happens within 2 weeks. The death rate for children because of medical procedures is less than 5%. However, adults face a higher risk, with a death rate as high as 60%.
Possible Complications When Diagnosed with Staphylococcal Scalded Skin Syndrome (Ritter disease)
Output: The complications after the procedure could be serious, including dehydration, further infections, balance issues within your body’s salts and minerals (electrolytes), bloodstream infections – known as sepsis, kidney failure, and scarring. Here are these complications listed:
- Dehydration
- Secondary infections
- Electrolyte imbalance
- Sepsis (bloodstream infection)
- Renal failure (kidney failure)
- Scarring
