Many pediatric skin conditions can be safely monitored with minimal intervention, but certain skin conditions are emergent and require immediate attention and proper assessment of the neonate, infant, or child. The skin may provide the first presentation of a potentially fatal disease with serious sequelae. Cutaneous findings may indicate the need for further evaluation. Therefore, it is important to differentiate skin conditions with benign etiologies from those that require immediate diagnosis and treatment, as early intervention of some of these conditions can be lifesaving. Herein, we discuss pertinent pediatric dermatology emergencies that dermatologists should keep in mind so that these diagnoses are never missed.
Staphylococcal Scalded Skin Syndrome
Presentation
Staphylococcal scalded skin syndrome (SSSS), or Ritter disease, is a potentially fatal pediatric emergency, especially in newborns.1 The mortality rate for SSSS in the United States is 3.6% to 11% in children.2 It typically presents with a prodrome of tenderness, fever, and confluent erythematous patches on the folds of the skin such as the groin, axillae, nose, and ears, with eventual spread to the legs and trunk.1,2 Within 24 to 48 hours of symptom onset, blistering and fluid accumulation will appear diffusely. Bullae are flaccid, and tangential and gentle pressure on involved unblistered skin may lead to shearing of the epithelium, which is a positive Nikolsky sign.1,2
Causes
Staphylococcal scalded skin syndrome is caused by exfoliative toxins A and B, toxigenic strains of Staphylococcus aureus. Exfoliative toxins A and B are serine proteases that target and cleave desmoglein 1, which binds keratinocytes in the stratum granulosum.1,3 Exfoliative toxins disrupt the adhesion of keratinocytes, resulting in bullae formation and subsequently diffuse sheetlike desquamation.1,4,5 Although up to 30% of the human population are asymptomatically and permanently colonized with nasal S aureus,6 the exfoliative toxins are produced by only 5% of species.1
In neonates, the immune and renal systems are underdeveloped; therefore, patients are susceptible to SSSS due to lack of neutralizing antibodies and decreased renal toxin excretion.4 Potential complications of SSSS are deeper soft-tissue infection, septicemia (blood-borne infection), and fluid and electrolyte imbalance.1,4
Diagnosis and Treatment
The condition is diagnosed clinically based on the findings of tender erythroderma, bullae, and desquamation with a scalded appearance, especially in friction zones; periorificial crusting; positive Nikolsky sign; and lack of mucosal involvement (Figure 1).1 Histopathology can aid in complicated clinical scenarios as well as culture from affected areas, including the upper respiratory tract, diaper region, and umbilicus.1,4 Hospitalization is required for SSSS for intravenous antibiotics, fluids, and electrolyte repletion.
Figure 1. Staphylococcal scalded skin syndrome. Erythema of the axilla and antecubital fossa and an erosion on the right flank. The skin was tender to the touch.
Differential Diagnosis
There are multiple diagnoses to consider in the setting of flaccid bullae in the pediatric population. Stevens-Johnson syndrome or toxic epidermal necrolysis also can present with fever and superficial desquamation or bullae; however, exposure to medications and mucosal involvement often are absent in SSSS (Figure 2).2 Pemphigus, particularly paraneoplastic pemphigus, also often includes mucosal involvement and scalding thermal burns that are often geometric or focal. Epidermolysis bullosa and toxic shock syndrome also should be considered.1
Figure 2. Stevens-Johnson syndrome secondary to trimethoprimsulfamethoxazole exposure. Ulceration of the upper and lower lips highlight mucosal involvement.