Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare hypersensitivity reactions that affect skin and mucous membranes. The most common etiologies of SJS and TEN in children are medications (usually anticonvulsants and sulfonamides) and infections (usually Mycoplasma species or herpes simplex virus). The pathogenesis of SJS and TEN is discussed in BCSC Section 8, External Disease and Cornea.
Systemic manifestations range from mild to severe. A prodrome of fever, malaise, and upper respiratory tract infection is followed by bullous mucosal and skin lesions. These lesions rupture, ulcerate, and become covered by gray-white membranes and a hemorrhagic crust.
Ocular involvement, which occurs in as many as 50% of patients, varies from mild mucopurulent conjunctivitis to severe perforating corneal ulcers. Ocular involvement in SJS and TEN begins with edema, erythema, and crusting of the eyelids. The palpebral conjunctiva becomes hyperemic, and distinct vesicles or bullae may occur. In many instances, epithelial defects or ulcers involving the tarsus and fornices develop. In severe cases, membranous or pseudomembranous conjunctivitis may occur (Fig 20-15) and lead to symblepharon formation. Superinfection, most commonly with Staphylococcus species, may develop.
Late ocular complications, possibly accompanied by a decrease in vision, occur in approximately 27% of pediatric patients. These complications include anomalies of eyelid position (ectropion and entropion), dry eye disease, trichiasis, chronic conjunctivitis, corneal defects, corneal vascularization, and symblepharon formation.
SJS and TEN are considered a disease continuum, distinguished by severity. The current nomenclature is based on the amount of skin involvement, with SJS being of lesser severity; TEN, greater severity; and SJS-TEN in between these. They are diagnosed based on clinical presentation and skin biopsy results. Initial management includes treatment of any underlying infection and discontinuation of any inciting drug. Systemic therapy with corticosteroids or intravenous immunoglobulin is controversial. The mortality rate for these conditions is much lower in children than in adults: 0% in SJS, 4% in SJS-TEN, and 16% in TEN. A full discussion of systemic treatment is beyond the scope of this book. A dermatologist and a specialist in pediatric infectious diseases should be consulted.
Figure 20-15 Stevens-Johnson syndrome. Early, severe involvement of the conjunctiva, right eye.
Early intervention is important for preventing the late ocular complications of SJS, SJS-TEN, and TEN. Ocular lubrication with artificial tears and ointments (preferably preservative-free) should be applied frequently. Associated microbial infections should be treated. The fornices may be swept to lyse adhesions, although some ophthalmologists believe that doing so may stimulate inflammation and cause further scarring. In severe cases, a symblepharon ring may be useful in cooperative patients. In patients with significant ocular disease, a corneal bandage device using amniotic membrane or amniotic membrane grafting should be considered early to decrease the risk of late ocular complications.
Hsu DY, Brieva J, Silverberg NB, Paller AS, Silverberg JI. Pediatric Stevens-Johnson syndrome and toxic epidermal necrolysis in the United States. J Am Acad Dermatol. 2017;76(5):811–817.
Jain R, Sharma N, Basu S, et al. Stevens-Johnson syndrome: the role of an ophthalmologist. Surv Ophthalmol. 2016;61(4):369–399.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.