Figure 3
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Figure 4
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"Skin Rash and Sore Throat"
Moustafa H, Moustafa, M.D. and Leon Salem, M.D.,M.S.
Diagnosis
Stevens-Johnson Syndrome (SJS) secondary to carbamezapine ingestion.
Hospital Course
The patient was admitted to the hospital and managed conservatively. Carbamezapine was discontinued. The
patient was discharged to home on the sixth day of hospitalization and had no complications on follow-up two
weeks later.
Pathophysiology
SJS is a multisystem immune-complex-mediated hypersesitivity reaction. Organ systems involved included the
skin, eyes, oral and nasal mucosa, lower respiratory tract, gastrointestinal tract as well as the vaginal and
urethral tracts. It is by definition the most severe form of erythema multiforme presenting with characteristic
generalized cutaneous maculopapular vesiculation and necrolysis with incapacitating mucous membrane
involvement. SJS typically presents initially with a prodrome of fever, malaise and respiratory symptoms
suggesting a flu-like illness. One to three days thereafter, generalized cutaneous lesions erupt beginning as
symmetric, acral, erythematous macules and papules. Vesiculation, purpuric regions and necrosis are seen in the
center of these lesions surrounded by an erythematous border, also known as the 'target lesion,' is considered
pathognomonic for SJS. Erosions, ulcerations and blistering in themouth and hemorrhagic crusting of the lower
lip are often seen and can be so severe that the patient may not be able to tolerate oral intake. Ocular findings in
SJS include conjunctivities, corneal ulceration, anterior uveitis and panophthalmitis. Opthalmology consultation
is mandaroty in all patients with concomitant ocular involvement due to the fact that blindness may develop in up
to 10% of cases. The eruption of SJS may be associated with a variety of infections, malignancies and medications.
Common drup etiologies include penicillins, sulfas, barbiturates, henytoin, and carbamazepine.
Treatement
Although there is no specific drup therapy for SJS, treatment is aimed at supportive measures and avoiding any
future exposure to the offending agent or drug. All patients with SJS must be admitted and patients with severe
mucious membrane involvement may require admission to a burn unit where fluids and electrolytes can be managed
aggressively. The use of systemic steroids for symptomatic relief is controversial and in some cases
has been thought to increase the incidence of complications. Antibiotics are indicated only if there is a secondary
bacterial infection present which may lead to bacteremia. Individual lesions should typically heal within 1-2
weeks unless secondary infection occurs and the majority of patients recover without sequelae.
Clinical Pearls
- SJS represents the most severe form of erythema multiforme characterized by 'target lesions' and incapacitating
mucious membrane involvement.
- Opthalmologic consultation is necessary if ocular involvement is suspected
- Care in teh ED is aimed primarily at aggressive fluid replacement and electrolyte correction
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