| Staphylococcal scalded skin syndrome | |
|---|---|
| Other names | Pemphigus neonatorum, Ritter's disease,[1] localizedbullous impetigo |
 | |
| An infant with Staphylococcal scalded skin syndrome | |
| Specialty | Dermatology |
Staphylococcal scalded skin syndrome (SSSS) is adermatological condition caused byStaphylococcus aureus.
The disease presents with the widespread formation of fluid-filled blisters that are thin walled and easily ruptured, and the patient can be positive forNikolsky's sign. SSSS bears a resemblance tothermal burns orscalding, hence the condition's name.[2]
Ritter's disease of the newborn is the most severe form of SSSS, with similar signs and symptoms. SSSS often includes a widespread painfulerythroderma, often involving the face, diaper, and otherintertriginous areas. Extensive areas ofdesquamation might be present. Perioral crusting and fissuring are seen early in the course. Unliketoxic epidermal necrolysis, SSSS spares the mucous membranes.
Children with SSSS may exhibit fussiness or irritability, tiredness, fever, redness of the skin, easily broken fluid-filled blisters that leave an area of moist, tender, painful skin, and large sheets of the top layer of skin that easily peel away.[3]
The condition is most common in children under 6 years, but can be seen in adults who areimmunosuppressed or havekidney failure.[citation needed]
Thesyndrome is induced by epidermolyticexotoxins (exfoliatin)[4] A and B, which are released byS. aureus and cause detachment within the epidermal layer, by breaking down thedesmosomes. One of the exotoxins is encoded on the bacterial chromosome, while the other is encoded on aplasmid. These exotoxins are proteases that cleavedesmoglein-1, which normally holds thegranulosum andspinosum layers together, similar to the pathophysiology of the autoimmune skin disease,pemphigus vulgaris.[citation needed]
SSSS is a clinical diagnosis. This is sometimes confirmed by isolation ofS. aureus from blood, mucous membranes, or skin biopsy; however, these are often negative. Skin biopsy may show separation of the superficial layer of the epidermis (intraepidermal separation), differentiating SSSS from TEN, wherein the separation occurs at the dermo-epidermal junction (subepidermal separation). SSSS may be difficult to distinguish from toxic epidermal necrolysis and pustular psoriasis.[citation needed]
The mainstay of treatment for SSSS is supportive care along with eradication of the primary infection. Conservative measures include rehydration, antipyretics (e.g.,ibuprofen orparacetamol), management of thermal burns, and stabilization. Parenteral antibiotics to coverS. aureus should be administered. Most strains ofS. aureus implicated in SSSS havepenicillinases, so are penicillin resistant. Therefore, treatment withnafcillin,oxacillin, orvancomycin is typically indicated.Clindamycin is sometimes also used because of its inhibition of exotoxins.[citation needed]
The prognosis of SSSS in children is excellent, with complete resolution within 10 days of treatment, and without significant scarring. However, SSSS must be differentiated carefully fromtoxic epidermal necrolysis, which carries a poor prognosis. The prognosis in adults is generally much worse, and depends upon various factors such as time to treatment, host immunity, and comorbidities.[citation needed]
The clinical features were first described in 1878 by Baron Gottfried Ritter von Rittershain, who observed 297 cases among children in a single Czechoslovak children's home over a 10-year period.[5]
In 1885, Nil Filatow, and in 1894, Clement Dukes, described an exanthematous disease which they thought to be a form of rubella, but in 1900, Dukes identified it to be a separate entity which came to be known by the namesDukes' disease,[6] Filatov's disease, or fourth disease. Although Dukes identified it as a separate entity, it is thought not to be different fromscarlet fever caused by staphylococcal exotoxin after Keith Powell proposed equating it with the condition currently known as staphylococcal scalded skin syndrome in 1979.[7][8][9][10]