Stevens Johnson Syndrome

Syndrome: Stevens Johnson Syndrome (Erythema Multiforme Major)
-a life threatening condition of the skin in which cell death causes the epidermis to separate from the dermis. Thought to be a hypersensitivity reaction
-it’s the more severe mucocutaneous form (nasty older brother) of erythema multiforme, largely affecting the mucous membranes (oral, pharyngeal, ocular)
Etiology: Thought to arise from a disorder of the immune system. Can follow various viral infections (Herpes Simplex, mumps, influenza, EBV), but the leading cause appears to be the use of certain medications (NSAIDS, sulfonamides, penicillins, barbiturates, Dilantin)
Diagnosis: Usually a diagnosis of exclusion, ie after ruling out other diseases that present similarly (mucous membrane pemphigoid, pemphigus, lichen planus). Biopsy specimen necessary to make the definitive diagnosis. People with Lupus and HIV are at higher risk.
-much more common in older patients (increased drug exposure), however the literature does report its occurrence in children. Search of pediatric dental literature found no results.
- Overall incidence is very low (about 6:1,000,000)
Systemic/Medical Conditions: Begins with fever, sore throat, fatigue. Conjunctivitis occurs in 30% of kids with SJS. This can lead to scarring/significant morbidity.
Oral Manifestations: These are the most common signs (95-100%) of the syndrome. Present as erythematous and edematous lesions that quickly develop into blisters that rupture, leaving PAINFUL erosions all over, especially on the lips. These erosions are typically covered by yellowish white pseudomembranes or hemorrhagic crusts.
Treatment: Systemic steroids. Secondary infections treated with oral antibiotics.
Dental needs/considerations: lesions are extremely painful, so probably best not to treat until symptoms have resolved.
Recent Info:
--Manute Bol died from complications of this disease
--laser phototherapy recently used as a successful therapy in Brazil (Jan 2011)