Scalded Skin Syndrome

Scalded Skin Syndrome

Article
Skin Care
Diseases & Conditions
+1
Contributed byLester Fahrner, MD+1 moreMay 19, 2022

What are the other Names for this Condition? (Also known as/Synonyms)

  • Localized Bullous Impetigo
  • Lyell’s Disease
  • Staphylococcal Scalded Skin Syndrome (SSSS)

What is Scalded Skin Syndrome? (Definition/Background Information)

  • Scalded Skin Syndrome is a localized bacterial infection that causes diffuse skin damage with superficial fragility and skin peeling, which resembles a widespread first-degree burn
  • The condition is caused by Staphylococcus aureus bacteria, which produce a toxin that causes the skin to peel off. Hence, the condition is also known as Staphylococcal Scalded Skin Syndrome (SSSS). The toxins cause the skin to become fragile, and the skin layers begin to peel off or blister easily, when only a slight pressure is applied on them
  • SSSS commonly affects infants and young children below the age of 5 years. It is rare in adults, but those with renal failure, immune system deficiency, and other chronic illnesses may be vulnerable to Scalded Skin Syndrome. The infection can spread from one individual to another through close contact
  • The toxin produced by the S. aureus is generally removed by the kidneys or blocked by an antibody response from prior exposure of the individual. When either of them is not functioning properly, the toxin gets circulated in the blood stream and can affect most of the body surface
  • Scalded Skin Syndrome can be a serious and life-threatening infectious disease. Treatment of the disease depends on the affected individuals age and health parameters. Systemic antibiotics, the use of topical medications, and intravenous fluid administration form part of treatment

There are two forms of Scalded Skin Syndrome, which include:

  • Localized form of Scalded Skin Syndrome
  • Generalized form of Scalded Skin Syndrome

Who gets Scalded Skin Syndrome? (Age and Sex Distribution)

  • Infants and young children generally below the age of 5 years are mostly affected by Scalded Skin Syndrome (SSS). Adults with kidney failure, immune system deficiency, and those undergoing chemotherapy are also prone to this manifestation of a bacterial infection
  • A male prevalence is observed when children are affected by Staphylococcal SSS (2:1 in sporadic cases). When SSS spreads in a neonatal nursery, males are affected 4:1. When adults are affected, males are also affected more than females
  • A recent study reveals that Caucasians are more affected than individuals of other races

What are the Risk Factors for Scalded Skin Syndrome? (Predisposing Factors)

The risk factors for Scalded Skin Syndrome include:

  • Children below age 5 years have the highest risk
  • Adults suffering from kidney failure and poor/deficient immune system are also vulnerable
  • Adults taking immunosuppressant drugs can easily contract Staphylococcal infections
  • Adults undergoing chemotherapy also have a high risk of acquiring this infection

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Scalded Skin Syndrome? (Etiology)

  • Scalded Skin Syndrome is caused by strains of the bacteria Staphylococcus aureus that produce a toxin causing the skin to peel off or form superficial blisters
  • Staphylococcus aureus bacteria produce two types of toxins that may be Type A or ETA and Type B- ETB, which cause separation of the skin from its basal layer. These toxins act as proteases (group of enzymes whose catalytic function is to break down peptide bonds of proteins) and target the protein desmoglein1 (DG-1 which is an important cell-to-cell attachment protein found only in the epidermis)
  • When the toxins attack desmoglein 1, the skin becomes very fragile and easily peels off from the lower layers of the epidermis (epidermis is the superficial part of the skin)
  • Infants and young children are mostly affected by Scalded Skin Syndrome due to:
    • Lack of antibodies and weak immune system to counterattack the toxins released by the Staphylococcus bacteria
    • Underdevelopment of kidney function, which affects the (otherwise) easy removal of the toxins from the body
  • A close contact with the affected individual may cause transmission or spread of the infection

What are the Signs and Symptoms of Scalded Skin Syndrome?

The signs and symptoms of Staphylococcal Scalded Skin Syndrome (SSSS) may include fever and skin changes or skin condition that progressively worsens. These include:

  • Redness of the skin
  • Appearance of fluid-filled blisters
  • Top layer of the skin starts peeling off
  • Tenderness to palpation and the presence of warm skin: The skin in the affected area feels tender and warm to touch (that may be undertaken as part of a physical examination)
  • Facial edema: Swelling, due to the accumulation of excess fluid in the face
  • Perioral crusting: Crust formation around the skin around the mouth
  • Dehydration: When large areas of skin peels off, it causes a loss of fluids from the body that can lead to dehydration
  • Widespread painful erythroderma (redness and scaling of the skin)
  • Erythematous rashes, rashes that are reddish in color
  • General feeling of tiredness and irritability

How is Scalded Skin Syndrome Diagnosed?

The diagnosis of Scalded Skin Syndrome may involve:

  • A thorough physical examination correlated with complete medical history (including family and social history)
  • Checking for Nikolsy’s sign: Patients exhibit positive  Nikolsky’s sign, where the skin is so fragile that the layers peel off easily, even when only a slight tangential pressure is applied onto the skin
  • Complete blood count (CBC) test
  • Cultures of the skin, nose, throat, and other sites that show clinical signs of infection. In newborns, the umbilical cord stump, and in newborn boys, a circumcision site of the penis may be cultured
  • Erythrocyte sedimentation rate (ESR) may be checked
  • Electrolyte and renal functions need to be carefully monitored
  • Imaging tests may include chest radiograph to rule out pneumonia
  • Skin biopsy to determine the level of the separation of the skin layers

Many clinical conditions may have similar signs and symptoms. Your health care provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Scalded Skin Syndrome?

The complications due to Scalded Skin Syndrome may include:

  • Dehydration: The management of fluid levels in the body may become a problem. When the fluid levels are not properly maintained, it can lead to dehydration and electrolyte imbalances
  • Difficulties with temperature control, especially in infants
  • Septicemia, when the entire bloodstream becomes infected
  • Cellulitis: A medical condition characterized by deeper skin infection

If the bacterial infection or the fluid loss from epidermal disruption by the Staphylococcal toxin becomes severe and untreatable, it may lead to death.

How is Scalded Skin Syndrome Treated?

The mode of treatment of Scalded Skin Syndrome depends upon the following factors:

  • Age of the individual
  • Overall health of the individual
  • Medical history of the individual
  • Severity of the condition
  • Tolerance to specific medicines and procedures
  • The individual’s preference

The treatment of Scalded Skin Syndrome will involve antibiotics, which are the mainstay of treatment for the infection.

  • Staphylococcal infections are generally resistant to penicillin, and hence, other medications such as nafcillin, oxacillin, vancomycin, and clindamycin are generally used
  • Tylenol and other drugs to reduce the fever
  • Fluids are administered intravenously to prevent dehydration
  • Therapy with topical antibiotics, such as mupirocin should  be used on skin wounds
  • Moist compresses to the skin can give comfort, relieve pain and irritation
  • Use of moisturizing ointment will help keep the skin moist

The skin healing may take 10 days or more after the treatment course. Severely affected individuals may require hospitalization in the burn unit of the hospital.

How can Scalded Skin Syndrome be Prevented?

Preventive measures for Scalded Skin Syndrome include:

  • Identification and treatment of asymptomatic carriers (individuals who carry the bacteria, but have no signs of infection) should be the first aim in preventive methods
  • The places where the infected individuals stay should be kept clean and hygienic
  • Strict hand washing with antibacterial soap should be followed

What is the Prognosis of Scalded Skin Syndrome? (Outcomes/Resolutions)

  • The prognosis of Scalded Skin Syndrome in most children is excellent. A complete cure within 10 day, without scarring is possible
  • The prognosis in adults depend on the following factors:
    • Immunity level
    • The speed with which the treatment for the infection is started.
    • Other underlying medical conditions
  • The morbidity and mortality rate is high in adults. The mortality rate in infants is as low as 4%, but it is as high as 67% in adults
  • However, most SSSS cases are mild and if diagnosed and treated with antibiotics early, they are easily managed. The skin heals with no scarring, even when the area of skin affected is large

Additional and Relevant Useful Information for Scalded Skin Syndrome:

A special study and report revealed that hospitalization due to Scalded Skin Syndrome increased by 5-folds from 1989 through 2004 and it was seen primarily in individuals older than 16 years.

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On the Article

Krish Tangella MD, MBA picture
Approved by

Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team
Lester Fahrner, MD picture
Reviewed by

Lester Fahrner, MD

Chief Medical Officer, DoveMed Team

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