Staphylococcal scalded skin syndrome (SSSS)

ICD-11

EA50.2

Synonyms

Staphylogenic Lyell`s syndrome, Dermatitis Exfoliativa Neonatorum Ritter von Rittershain.

Epidemiology

SSSS: no exact data.

Definition

SSSS: acute exfoliative dermatitis primarily in small children following staphylogenic infections with lysophage group II exfoliative toxins.

Aetiology & Pathogenesis

Toxin-mediated epidermolytic dermatosis, characterized by erythema and widespread loss of the superficial epidermal layers, resembling burn. Causative agent is toxin-producing (exfoliative toxins A and B) Staphylococcus aureus toxins acting via serin proteases desmoglein 1 in the upper epidermal layers.

Signs & Symptoms

Following a staphylococcal infection, initially erythema resembling scarlet fever followed by small unstable blisters which quickly erode and lead to widespread skin loss similar to grade II burns. Acute course, systemically ill patient. Nikolski sign positive.

Localisation

All areas.

Classification

None.

Laboratory & other workups

Intensive care laboratory data regarding kidney, liver and blood, albumin.

Dermatopathology

Acantholytic cleft in the S.granulosum and blister roof only contains stratum corneum (in contrast to TEN with full-thickness epidermal damage), subcorneal blisters and bullae.

Course

Rapid onset within hours to two days.

Complications

Sepsis, pneumonia.

Diagnosis

History, clinical feature, histology (frozen section of blister roof).

Differential diagnosis

TEN (drug--induced), epidermolysis bullosa, chronic bullous disease of childhood (linear IgA disease). Streptogenic toxic syndrome; Kawasaki syndrome.

Prevention & Therapy

Children are to be admitted immediately to intensive care unit. Antibiotics covering resistant staphylococci according to resistogram; immunoglobulins are recommended. Fluid replacement as in burn patients, elimination of staphylococcal foci; antimicrobial disinfectant therapy (baths, compresses). Paracetamol, no NSAIDs.

Special

Immediate biopsy for cryosection to visualize the location of blister for differential diagnosis.

2.2.6 Staphylococcal scalded skin syndrome (SSSS)