Pediatric Dermatology Consult

Pediatric Dermatology Consult - October 2015

Publish date: August 15, 2015
By
Dr. Catalina Matiz
David Ginsberg

A 10-day-old term female neonate is brought in for evaluation of a worsening rash. The mother reports no complications during the pregnancy or birth and says that the patient was previously healthy. The mother first noticed a perioral erythematous eruption 4 days ago, which progressively spread to the neck, trunk, diaper area, and arms. Two days after the onset of erythema, the mother began noticing sloughing of the skin in the patient’s neck and axillae. The patient’s temperature, taken via rectal thermometer at home, was reported to be 38.8° C. On exam, the patient appears well nourished and irritable, with blanching erythroderma. The perioral area is erythematous with radial fissuring and crusting. The skin is denuded with signs of superficial desquamation in the neck fold, axillae, flexural arm surfaces, hands, and in the perineal and perianal areas. Nikolsky sign (when the upper layers of the epidermis easily move laterally with slight pressure or rubbing of diseased skin) is positive. There is no involvement of the oral, nasal, or vaginal mucosa.

What’s your diagnosis?

Kawaski disease

Toxic epidermal necrolysis

Epidermolysis bullosa

Staphylococcal scalded skin syndrome

Candida infection

Diagnosis: Staphylococcal scalded skin syndrome (SSSS)

Staphylococcal scalded skin syndrome (SSSS), also known as Ritter’s disease and pemphigus neonatorum, is a blistering skin disease seen most commonly in neonates and young children, but it can be seen in older children and rarely in adults1. The severity of SSSS can range from mild localized bullae to severe generalized erythroderma and exfoliation.

Fragile bullae rupture easily, leaving behind denuded, erythematous skin, giving the scalded appearance for which the disease is named.

The most common early symptoms in neonates and children are fever, malaise, irritability, and skin tenderness followed by erythema at the site of infection, usually in the perioral, periorbital, or umbilical areas1. The erythema becomes well demarcated, and more erythematous patches develop and spread over the body and subsequently coalesce. One to two days later, fragile bullae form over the erythematous skin, most commonly in intertriginous areas; these bullae rupture easily, leaving behind denuded, erythematous skin, giving the scalded appearance for which the disease is named.

The causative factor is an exfoliating (epidermolytic) toxin (ET) produced by phage II Staphylococcus aureus2. There are two types of ETs, ETA and ETB. In western countries, the vast majority of toxin-producing strains of S. aureus produce ETA. Both ETA and ETB target and destroy desmoglein-1, a molecule found in desmosomes that plays an important role in cell-cell adhesion in the stratum granulosum of the epidermis2. This compromise in the structural integrity of the superficial epidermis is responsible for the fragile nature of the bullae seen in SSSS and the positive Nikolsky sign.

Differential diagnosis

The differential diagnosis includes toxic epidermal necrolysis (TEN), drug reaction with eosinophilia and systemic symptoms (DRESS), and staphylococcal toxic-shock syndrome (STSS)3.

TEN is a severe condition, most commonly caused by adverse drug reactions. Unlike in TEN, SSSS patients the lack mucosal involvement and have more superficial epidermal peeling4.

DRESS is also a medication-induced reaction, commonly associated with anticonvulsants and sulfonamides. Only severe cases of DRESS can present similarly to SSSS with exfoliation and erythroderma, but there is often mucosal involvement and signs of visceral organ involvement, most commonly the liver5.

The additional findings inSTSS that help distinguish it from SSSS include petechiae and preferential desquamation of the palms and soles6.

SSSS can usually be diagnosed clinically, but biopsies are important in differentiating difficult cases. Sampling the crusted areas as well as the nasal, periumbilical, and perianal sites for culture and sensitivities is recommended prior to starting antibiotic therapy.

Treatment

For neonates and children with severe disease, inpatient treatment in an intensive care or burn unit setting is sometimes required. SSSS can be caused by both methicillin-sensitive and -resistant strains of S. aureus, so antibiotic treatment must be tailored to the sensitivity.

In either case, beginning broad-spectrum antibiotic treatment early is essential. For methicillin-sensitive strains, penicillinase-resistant penicillins like flucloxacillin, nafcillin, or oxacillin are the drugs of choice3,7.

In known MRSA infections or in areas with a high prevalence of resistance and suspected MRSA, clindamycin or vancomycin should be used3,8. Supportive care in an inpatient setting also is important, especially in younger or sick children because they are more susceptible to sepsis and pneumonia and are at risk for complications because of problems with temperature, fluid, and electrolyte regulation while their skin barrier function is compromised7.

References

  1. Arch Dis Child. 1998;78(1):85-8.
  2. N Engl J Med. 2006;355(17):1800-10.
  3. J Eur Acad Dermatol Venereol. 2014;28(11):1418-23.
  4. JAAD. 2013;69(2):173.e1-173.e13.
  5. JAAD. 2013;68(5):693.e1-693.e14.
  6. Clin Infect Dis. 2006;42(2):181-5.
  7. Am J Clin Dermatol. 2003;4(3):165-75.
  8. Pediatr Dermatol. 2014;31(3):305-08.

Dr. Matiz is assistant professor of dermatology at Rady Children's Hospital San Diego-University of California San Diego and Mr. Ginsberg is a research associate at the hospital. Dr. Matiz and Mr. Ginsberg said they have no relevant financial disclosures.

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