A 2-Year-Old-Boy With an Alarming Facial Rash

Buraa Kubaisi, MD; Nakhoul Nakhoul, MD; C. Stephen Foster, MD

Disclosures

January 22, 2019

The SCORTEN score calculates the risk for death in both SJS and toxic epidermal necrolysis, on the basis of the following variables[7]:

  • Age > 40 years

  • Cancer

  • Heart rate > 120 beats/min

  • Initial percentage of epidermal detachment > 10%

  • Blood urea nitrogen level > 10 mmol/L

  • Serum glucose level > 14 mmol/L

  • Bicarbonate level < 20 mmol/L

Each variable is assigned a value of 1 point. Mortality rates are as follows:

  • 1 point: ≥ 3.2%

  • 2 points: ≥ 12.1%

  • 3 points: ≥ 35.3%

  • 4 points: ≥ 58.3%

  • ≥ 5 points: ≥90%

In a matter of a few days, individuals with SJS lose large areas of epidermis. Although this progresses rapidly, it usually ceases suddenly and the reepithelialization process starts. It is impossible to predict the course of a patient at presentation. The reepithelialization phase usually lasts 3-4 weeks.

Management of patients with SJS is usually provided in intensive care units or burn centers. Supportive care must include management of fluid and electrolyte requirements. Withdrawal of the suspected offending agent is critically important. The earlier the drug is withdrawn, the better the prognosis.[8]

Routine antibiotics are not usually indicated unless infection is evident because fever may be part of the disease. Debridement of necrotic skin is not recommended before disease activity ceases.

The use of corticosteroids in the management of SJS is controversial. Some physicians believe that these agents can delay wound healing and increase the chance of infection. If steroids are used, they should be initiated during the initial stage with a prophylactic antibiotic and rapidly tapered off.[9,10]

Plasmapheresis, immunosuppressive therapy, and intravenous immunoglobulin have been used, with variably successful results.[11,12]

The patient in this case was kept in the intensive care unit for 2 weeks, with special attention given to his hemodynamic stability and fluid status. The causative drug was withdrawn early. He was started on steroids, with a prophylactic antibiotic, and rapidly tapered off within 2 weeks. He had significant healing of the oral and skin lesions at the end of 2 weeks and was discharged afterward.

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