Stevens-Johnson Syndrome


American academy of opthalmology


 Stevens-Johnson syndrome (erythema multiforme) is an

acute inflammatory polymorphic disease affecting skin and
mucous membranes. All ages may be affected, and the
incidence is equal in both sexes. This is a severe disease with
a 5%–15% mortality rate. Ocular involvement, which occurs in
as many as half of patients, varies from a mild mucopurulent
conjunctivitis to severe perforating corneal ulcers. Blindness
occasionally occurs in patients with severe late-phase corneal
complications, such as ulceration, vascularization, and
perforation.
 Stevens-Johnson syndrome has been associated
with various bacterial, viral, mycotic, and protozoal infections.
Vaccines, collagen diseases, and many drugs have also been
implicated. The pathogenesis consists of angiitis leading to
erythematous lesions that become edematous or bullous and
darken, leaving concentric rings in a target shape. When
bullae are present, they are subepidermal and without
acantholysis.
 Clinical manifestations range from mild to
severe. A prodrome of chills is followed by pharyngitis,
headache, tachypnea, and tachycardia. In several days,
bullous mucosal lesions develop, especially in the oropharynx.
These lesions rupture and ulcerate and become covered by
gray-white membranes and a hemorrhagic crust.
 Ocular
involvement in Stevens-Johnson syndrome begins with
edema, erythema, and crusting of the eyelids. The palpebral
conjunctiva becomes hyperemic, and distinct vesicles or
bullae may occur. In many instances, a concomitant
conjunctivitis appears that is characterized by watery
discharge with mucoid strands (Fig 16-13). Secondary
infection, most commonly with Staphylococcus species, may
develop. In severe cases, a membranous or
pseudomembranous conjunctivitis may result from
coalescence of fibrin and necrotic cellular debris.
Symblepharon formation may occur with severe
pseudomembranous conjunctivitis. Primary corneal
involvement and iritis are rare ocular manifestations of
Stevens-Johnson syndrome.
 Late ocular complications occur
in approximately 20% of patients and include structural
anomalies of eyelid position (ectropion and entropion),
trichiasis, and symblepharon. Dry eye syndrome may also
result from deficiencies in the tear film—either in the aqueous
layer, from scarring of lacrimal duct orifices, or, more
commonly, in the mucin layer, from destruction of the
conjunctival goblet cells.
 Early intervention is important in
preventing the late ocular complications of Stevens-Johnson
syndrome. Systemic therapy with corticosteroids is
controversial. Antiviral treatment for cases associated with
herpes simplex infection may be required. A discussion of
systemic treatment is beyond the scope of this book. A
dermatologist and pediatric infectious disease expert should
be consulted.
 Local measures should be instituted early in
the course of the disease. Ocular lubrication with artificial
tears and ointments (preferably preservative-free) should be
applied regularly. Under topical anesthesia, the superior and
inferior fornices should be inspected and debrided daily. A
glass rod can be used for symblepharon lysis, although this
may be ineffective. A symblepharon ring can be useful in
severe cases in cooperative patients. Surveillance cultures for
microbial infection should be taken as needed. Amniotic
membrane grafting may be considered in patients with
advanced disease. See also BCSC Section 8, External
Disease and Cornea.