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Mortality Related to Stevens-Johnson Syndrome and TEN Is Lower in Children than in Adults

Summary and Comment |
October 7, 2011

Mortality Related to Stevens-Johnson Syndrome and TEN Is Lower in Children than in Adults

  1. Mary Wu Chang, MD

But long-term complications are common.

  1. Mary Wu Chang, MD

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) represent a spectrum of rare, life-threatening cutaneous adverse reactions — usually drug reactions — characterized by epidermal detachment resulting from keratinocyte apoptosis. SJS is defined as epidermal detachment of <10% of body surface area (BSA), TEN as >30% of BSA, and SJS/TEN overlap as 10% to 30% of BSA. In adults, the associated mortality rates are as high as 5% with SJS and >20% with TEN. No standardized treatment protocol exists. Investigators from two tertiary pediatric dermatology centers reviewed records of children with SJS, TEN, or SJS/TEN overlap (age 0–21 years) admitted for management over an 8-year period.

They identified 55 cases: 47 SJS, 5 TEN, and 3 SJS/TEN. Drugs were the most likely cause in 29 children (53%): antiepileptic drugs in 16, sulfonamide antibiotics in 7, other antibiotics in 4, and chemotherapy drugs in 2. Infectious etiologies included acute Mycoplasma pneumoniae infection and herpes simplex virus (HSV) infection, confirmed in 12 (22%) and 5 children (9%), respectively. Treatment included systemic antimicrobial agents (67%), systemic corticosteroids (40%), and antiviral drugs (31%). Intravenous immunoglobulin (IVIG) was given to 21 children (38%), 8 of whom received concurrent systemic corticosteroids.

Ten children (21% of the SJS group) had recurrent SJS up to 7 years later, three of whom had three recurrences. Reinfection with M. pneumoniae or HSV was associated with some recurrences; others were triggered by an anticonvulsant of a different class. Twenty-six children (45%) had long-term sequelae affecting the skin (e.g. hypopigmentation, scarring) in 42% and the eyes (e.g., uveitis, keratitis, corneal defects, conjunctivitis) in 27%. One child died from graft-versus-host disease secondary to bone marrow transplantation for severe combined immunodeficiency.

Comment

This is the largest pediatric series of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis to date. Although the mortality rate was lower than in adults, half the children had long-term complications. The high recurrence of SJS suggests a possible genetic predisposition. Treatment differed greatly between the two institutions; conclusions about therapy were not possible from this series.

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