Staphylococcal Scalded Skin Syndrome (SSSS), previously known as Dermatitis Exfoliativa Neonatorum, Staphylococcal Toxic Epidermal Necrolysis, Bacterial Toxic Epidermal Necrolysis, Ritter's Disease, or Keratolysis Neonatorum, is a severe acute generalized exfoliative pustular disease occurring in newborns. It is characterized by widespread erythema with the formation of flaccid, scalded-like bullae and extensive epidermal detachment. It is occasionally seen in adults. In 1966, it was discovered that Toxic Epidermal Necrolysis (TEN) could also be caused by staphylococci, and Ritter's disease was found to share identical clinical and pathological features with TEN. In 1967, Lyell classified TEN into four types based on etiology: staphylococcal, drug-induced, other causes, and idiopathic, identifying Ritter's disease as the staphylococcal type. By 1977, SSSS was recognized as a distinct condition separate from Toxic Epidermal Necrolysis.

bubble_chart Etiology

This disease is primarily a severe skin infection caused by coagulase-positive Staphylococcus aureus of phage group II type 71. This strain of Staphylococcus can produce exfoliative toxin, leading to skin damage. It has also been discovered that certain strains of group I or III Staphylococcus can produce exfoliative toxin. Experiments have shown that exfoliative toxin is mainly excreted through the kidneys. Infants and young children excrete it very slowly, causing the toxin levels in the blood to rise and resulting in skin damage and exfoliation. Staphylococcal scalded skin syndrome in adults is more common among those suffering from nephritis, uremia, physical weakness, immune dysfunction, or severe staphylococcal septicemia, which may be related to impaired kidney excretion and weakened immune function.

The histopathology of this disease is characterized by parakeratosis, with the stratum corneum presenting a reticular pattern. The stratum spinosum shows edema, with vacuolation and nuclear condensation of the spinous cells. There are spaces between the stratum corneum and the stratum spinosum. The dermis exhibits edema and congestion, with moderate to high inflammatory infiltration around the blood vessels.

bubble_chart Clinical Manifestations

Damage can begin in any area but often starts on the face, particularly around the mouth or neck. The local skin becomes flushed and rapidly spreads to surrounding areas. Within two to three days, the entire body may turn red, with blisters of varying sizes appearing on the erythematous base. These blisters can merge to form larger ones. The affected area is notably tender, with thin, loose, and easily ruptured blister walls, showing a positive Nikolsky sign. The epidermis is extremely prone to peeling, exposing a bright red, moist surface resembling a scald. The blister fluid is serous but may appear turbid, resembling impetigo. Bacterial cultures of the blister fluid often reveal Staphylococcus aureus, Streptococcus, or hemolytic streptococci. When the face is affected, light yellow crusts may form, with radial rhagades around the mouth. The scalp is rarely involved. The mucous membranes of the mouth, nose, and conjunctiva may also be affected, leading to stomatitis, rhinitis, and corneal ulcers. Patients often experience systemic symptoms such as fever and diarrhea. Some may die due to complications like bronchopneumonia, sepsis, abscesses, or gangrene. This condition primarily affects infants and young children, progresses rapidly, and has a high mortality rate.

The diagnosis can be made based on the characteristics of sudden onset, widespread erythema, flaccid bullae, epidermal detachment, positive Nikolsky's sign, and frequent occurrence in infants and young children.

bubble_chart Treatment Measures

1. Pay attention to the cleanliness and hygiene of infants. Diapers should be clean, and medical staff or family members with purulent skin diseases should not come into contact with newborns.
2. Strengthen care and keep warm. Pay attention to oral and eye care.
3. In the early stage, sufficient and effective antibiotics should be used to eliminate the existing Staphylococcus aureus infection in the body and stop the production of bacterial toxins. Antibiotic sensitivity tests should be conducted to select appropriate antibiotics. Methicillin can be administered, with adults receiving 1–1.5g intramuscularly every 4–6 hours, and children receiving 150–250mg per kilogram of body weight per day, divided into 4 intramuscular injections. Alternatively, erythromycin can be given at a dose of 80mg/(kg·d) intravenously. For penicillinase-resistant strains, cefazolin, cloxacillin, or other second- or third-generation cephalosporins can be selected.
4. Maintain water and electrolyte balance, provide nutritional supplementation, and strengthen supportive therapy, such as blood transfusions.
5. Opinions on the use of hormones vary. The use of hormones alone is prohibited, as they can cause immunosuppression and may be harmful rather than beneficial. However, some advocate for the combined use of hormones with antibiotics in the early stages to mitigate the effects of bacterial toxins. For patients whose disease cause and diagnosis are temporarily unclear, antibiotics and hormones can be used together. Once it is confirmed to be a Staphylococcus aureus infection, hormone treatment should be discontinued immediately.
6. Topical non-irritating bactericides, such as 0.5–1% neomycin emulsion, should be used externally. The membrane of large blisters should preferably be removed, followed by wet compresses with 1:5000–1:10000 potassium permanganate solution or 1:2000 Coptis Rhizome solution. For cleaning and dressing changes, apply 1% Chinese Gentian violet solution.

bubble_chart Differentiation

1. Exfoliative Erythroderma: The lesions present as diffuse erythema with a large amount of bran-like scales on the surface, without pustules or erosions. Seborrheic dermatitis-like changes are observed on the scalp, eyebrows, and flexural areas of the limbs. The course is chronic, and treatment with adequate antibiotics is ineffective.
2. Neonatal Pustulosis: Some clinical manifestations resemble this disease, and some consider it a variant of the same condition. However, neonatal pustulosis is primarily characterized by pustules, does not develop into generalized erythroderma, has a negative Nikolsky sign, and lacks epidermal detachment. It typically occurs within the first half-month after birth.
3. Non-Staphylococcal Toxic Epidermal Necrolysis: Distinguishing between staphylococcal and non-staphylococcal types is crucial, as their treatments and prognoses differ. The non-staphylococcal type is mostly drug-induced and is essentially a form of drug rash, primarily seen in adults. The skin lesions are polymorphic, resembling erythema multiforme, and the Nikolsky sign is positive only at the lesion sites. In contrast, the staphylococcal type shows a positive Nikolsky sign even on unaffected skin. The pathological changes also differ: the non-staphylococcal type exhibits full-thickness epidermal necrosis and subepidermal blisters, whereas the staphylococcal type shows superficial epidermal necrosis and intraepidermal blisters.