bubble_chart Overview

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 large, flaccid, scalded-like blisters 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 established as a distinct disease entity, separate from Toxic Epidermal Necrolysis.

bubble_chart Etiology

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

bubble_chart Pathological Changes

The histopathology of this disease shows parakeratosis, with the stratum corneum presenting a reticular pattern. There is edema in the stratum spinosum, where vacuolization and nuclear condensation of spinous cells occur, along with spaces between the stratum corneum and the spinous layer. 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 it 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. There is significant tenderness, and the blister walls are thin, loose, and prone to rupture, with 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 also appear turbid, resembling impetigo. Bacterial cultures of the blister fluid often reveal Staphylococcus aureus, Streptococcus, or hemolytic streptococci. Affected facial areas may develop light yellow crusts, with radial rhagades around the mouth. The scalp is rarely involved. The mucous membranes of the mouth, nasal cavity, and conjunctiva may also be affected, leading to inflammation, 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 predominantly affects infants and young children, progresses rapidly, and has a high mortality rate.

bubble_chart Diagnosis

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 nursing care and keep the baby warm. Pay attention to oral and eye care.

3. Early use of sufficient and effective antibiotics is necessary to eliminate Staphylococcus aureus infections in the body and stop bacterial toxin production. Conduct antibiotic sensitivity tests 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 kg 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, cephalosporin V, cloxacillin, or other second- or third-generation cephalosporins can be used.

4. Maintain water and electrolyte balance, provide nutritional support, and strengthen supportive therapy, such as blood transfusions.

5. Opinions on the use of hormones vary. The standalone use of hormones is prohibited, as they may cause immunosuppression and be harmful rather than beneficial. However, some advocate for the combined use of hormones with antibiotics in the early stages to mitigate bacterial toxin effects. For patients with unclear disease causes or diagnoses, antibiotics and hormones may be used together. Once Staphylococcus aureus-induced TEN is confirmed, hormone treatment should be discontinued immediately.

6. Topical application of non-irritating bactericides, such as 0.5–1% neomycin emulsion, is recommended. 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. {|105|}

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 impetigo: Some clinical manifestations are similar to this disease, and some consider it a variant of the same condition. However, neonatal impetigo is primarily characterized by pustules, does not develop into generalized erythroderma, has a negative Nikolsky sign, and lacks epidermal detachment. It usually occurs within the first half-month after birth.

3. Non-staphylococcal toxic epidermal necrolysis: Distinguishing between staphylococcal and non-staphylococcal types is crucial because 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 with subepidermal blisters, whereas the staphylococcal type shows superficial epidermal necrosis with intraepidermal blisters.