Staphylococcal scalded skin syndrome is a skin toxicosis caused by staphylococci. Infants, small children or immunocompromised adults are usually affected. Without treatment, the prognosis of the disease is very unfavorable.
What is Staphylococcal scalded skin syndrome?
Staphylococcal scalded skin syndrome is the name for skin toxicosis caused by staphylococci. Infants and young children are mostly affected. In very rare cases, severely immunocompromised adults can also develop this syndrome. For comprehensive guide to hydrops fetalis, please visit growtheology.com.
Translated, this term means skin scalding syndrome caused by staphylococci, because the skin changes look as if a scald had previously occurred. The top layers of skin die off, blister, and peel off easily. Before hospital hygiene became widespread, Staphylococcal scalded skin syndrome was a very common infectious disease of newborns.
It played a large part in the infant mortality rate that prevailed in the past. Today it occurs only rarely, but has lost none of its danger. The infection was first described by the doctor Gottfried Ritter von Rittershain in 1878. A second description was made by the Scottish doctor Alan Lyell in 1956, when he recognized that the Lyell syndrome named after him with similar symptoms has other causes. Therefore, there are also synonyms for this disease, Ritter syndrome or staphylogenic Lyell syndrome.
Causes
Staphylococcal scalded skin syndrome is a systemic disease because the actual toxin reaches the appropriate sites through the bloodstream. The cause of this disease is an infection with staphylococci, which form so-called exfoliants as toxins. The exfoliants are serine proteases that catalyze the breakdown of certain adhesive proteins in cell structures in the top layer of the skin.
There are special enzymes that specialize in catalyzing the breakdown of the adhesive protein desmoglein 1. Desmoglein 1 is located in the so-called desmosomes (connecting structures) of epithelial cells and causes the cells to stick together. When the adhesive protein is broken down, the cell structures loosen and the skin can peel off.
Exactly this is the effect of the exfoliants formed by the corresponding staphylococci, which trigger skin toxicosis far from where they originate. There is no evidence of an inflammatory reaction at the site of the skin necrosis, since the destruction of the skin is not triggered by immune reactions, but only by the breakdown of the adhesive proteins. As a rule, infants and small children up to the age of four are affected, since their immune system is not yet fully developed and the toxin is broken down too slowly.
Older children and adults rarely get sick. The prerequisite for this is an immune deficiency due to underlying diseases such as tumors, renal insufficiency, alcoholism or organ transplants. In the past, omphalitis (inflammation of the belly button) caused by staphylococci usually led to staphylococcal scalded skin syndrome in infants and small children.
Today, this disease hardly plays a role due to the introduction of hospital hygiene. However, pus-forming staphylococcal infections of the nose and throat ( tonsillitis, rhinitis ), the eyes ( conjunctivitis ) or the ears (otitis) are sometimes still the starting point for Staphylococcal scalded skin syndrome.
Symptoms, Ailments & Signs
Staphylococcal scalded skin syndrome is sometimes initially indicated by a scarlet-like exanthema a few days after a staphylococcal infection . The skin lesions later form at the site of this exanthema. Large, flaccid blisters form that rupture easily. The skin comes off easily.
This is already done by lightly rubbing the skin affected by the exanthema (Nikolski’s sign). Even on externally healthy skin, rubbing can already lead to skin detachment. If you rub your finger more firmly over the skin, it can be pulled off. However, only the top layers of skin are affected. There is the adhesive protein Desmoglein 1, which is broken down with the help of the Exfoliantine.
The mucous membranes are not affected. If left untreated, the disease can cause serious, potentially fatal complications. Secondary infections can develop at the site of skin necrosis. Massive fluid loss can result in hypovolemic shock. Other complications include sepsis and pneumonia.
Diagnosis & course of disease
In terms of differential diagnosis, the disease must be differentiated from scalding. A previous purulent infection and the Nikolski sign indicate a Staphylococcal scalded skin syndrome. It is also very important to make a reliable differential diagnosis against the so-called Lyell’s syndrome, which shows largely similar symptoms but is caused by the influence of medication. In contrast to Staphylococcal scalded skin syndrome, Lyell’s syndrome also affects the mucous membranes.
Complications
If the staphylococcal scalded skin syndrome remains untreated, serious complications arise over the course of the disease. First, there is a risk that the skin lesions will peel off, causing inflammation of the lower layers of the skin. The skin necrosis can cause secondary infections. If the pathogens get into the bloodstream, life-threatening blood poisoning occurs.
The massive dehydration can lead to hypovolemic shock. If there is no treatment by then at the latest, there is a risk of cardiovascular failure and the death of the patient. In less severe cases, the skin changes cause itching, redness and pain. The aesthetic changes promote mental illnesses and can lead to the development of inferiority complexes and social fears with the appropriate predisposition.
When treating the syndrome, the risks come from the drugs administered. The typically prescribed antibiotics can lead to headaches, muscle and body aches, gastrointestinal problems, skin irritations (e.g. itching, redness or inflammation) and muscle weakness. If the preparations are taken over a longer period of time, there is a risk of serious damage to the kidneys, liver and heart.
Allergic reactions can also occur. Complications can also occur during the infusion: inflammation at the puncture site, formation of blood clots and shock reactions in the body.
When should you go to the doctor?
Staphylococcal scalded skin syndrome should always be evaluated and treated by a doctor. Since it is a serious skin disease, it cannot heal on its own. Therefore, at the very first signs of the disease, a doctor must be consulted in order to prevent further complications and discomfort. If the staphylococcal scalded skin syndrome is not treated, in the worst case it can even lead to the death of the person concerned.
A doctor should be consulted if a large exanthema develops on the affected person’s skin. In most cases, this can also be seen with the naked eye. If this symptom occurs, a doctor must be consulted immediately. Furthermore, there can also be various inflammations in the body, which also have to be treated by a doctor.
Staphylococcal scalded skin syndrome is usually treated by a dermatologist or a general practitioner. In emergencies or in the case of very severe symptoms, an ambulance can be called or a hospital can be visited directly. Life expectancy depends on when the disease was diagnosed, so no general prediction is possible.
Treatment & Therapy
Staphylococcal scalded skin syndrome is treated with high doses of antibiotics against Staphylococcus aureus. These include penicillinase -resistant penicillins such as methicillin, flucloxacillin or oxacillin as well as other antibiotics such as erythromycin, cephalosporins or minocycline. Multi-resistant strains rarely develop in these staphylococci, so the treatment is very effective.
If the skin necrosis is very large, it is often necessary to compensate for fluid and electrolyte losses with an infusion. At the same time, the affected skin areas must be treated with a disinfectant to prevent secondary infections. Corticosteroids must not be used because they are not inflammation and the immune system would be additionally weakened.
Prevention
The best prevention of Staphylococcal scalded skin syndrome is strict adherence to hospital hygiene. In the past, when this was not an issue, staph infections often resulted in the death of the newborn child. Today’s high hygiene standards in hospitals have helped to drastically reduce child mortality.
Aftercare
If a staphylococcal scalded skin syndrome (SSSS) has been successfully treated, special follow-up measures are necessary to prevent secondary diseases and a recurrence of the staphylococcal scalded skin syndrome. Since a weak immune system is crucial for the onset of Staphylococcal Scalded Skin Syndrome, the immune system must be strengthened.
In addition to vaccinations and a plentiful supply of vitamins that strengthen the immune system, the use of various medications can be indicated. In addition, a high level of skin hygiene should be maintained, which consists of daily showering or, in the case of children, daily bathing, as well as regular hand disinfection. As a precaution, this should take place several times a day, as well as after contact with public toilets or washrooms.
In addition, regular check-ups should take place at the dermatologist’s, during which, in addition to the intensive examination of the skin, swabs of the mucous membranes should also be taken in order to be able to recognize a renewed spread of the pathogens in good time and to be able to take countermeasures. To avoid the development of other skin diseases (acne inversa, acne vulgaris), which can also be caused by the pathogens of the Staphylococcal scalded skin syndrome, smoking should be avoided for life and obesity should be avoided. Hair on the skin should be removed as a precaution. In addition, regular examinations of the inflammation levels in the blood should be carried out in order to detect secondary diseases at an early stage.
You can do that yourself
Staphylococcal scalded skin syndrome is life-threatening and requires immediate medical attention in the hospital. After intensive care therapy, a number of self-help measures can be used to support recovery.
First of all, the affected skin areas must be protected. There is a high risk of infection and bleeding, especially shortly after treatment. The large loss of fluid and blood salt is compensated for by an adapted diet. It is best for patients to discuss with a nutritionist how the diet can be designed. In addition, attention must be paid to any side effects of the drugs used. The extensive skin damage requires comprehensive and long-term care. Both ointments from the pharmacy and natural remedies such as calendula ointment are suitable for this. The doctor should be informed about the chosen preparation and the treatment should be monitored.
In addition, therapeutic advice must be obtained in order to avoid the development of psychological complaints. The combination of chronic pain and external changes repeatedly causes depression and other illnesses in patients. Comprehensive care that goes beyond the actual treatment of Staphylococcal scalded skin syndrome is all the more important.
