Stevens-Johnson Syndrome (SJS)

Content of This Page

 1- Introduction

2- Causes

3- Symptoms

4- Investigations & Lab Results

5- Drugs Associated With Stevens-Johnson Syndrome

6- Complications

7- Treatment

Introduction

Stevens-Johnson Syndrome (SJS) is a rare but serious and potentially life-threatening disorder characterized by a severe hypersensitivity reaction that affects the skin and mucous membranes. It usually presents as widespread painful rash, blisters, and peeling of the skin, along with involvement of mucosal surfaces such as the mouth, eyes, and genitals. SJS is most often triggered by adverse reactions to medications, infections, or, rarely, other factors. Because it causes extensive skin damage similar to a severe burn, prompt recognition and treatment are critical to reduce complications and improve outcomes.

 
 
 
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Causes

  • Medications (most common cause):

    • Antibiotics (e.g., sulfonamides, penicillins, cephalosporins)

    • Anticonvulsants (e.g., phenytoin, carbamazepine, lamotrigine)

    • Nonsteroidal anti-inflammatory drugs (NSAIDs)

    • Allopurinol

    • Antiretroviral drugs

  • Infections:

    • Mycoplasma pneumoniae

    • Herpes simplex virus

    • Other viral infections (e.g., HIV, influenza)

    • Bacterial infections

  • Idiopathic:

    • In some cases, no clear cause is identified.

  • Other triggers:

    • Vaccinations (rare)

    • Malignancies (rare association)

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Symptoms

  • Initial symptoms (prodromal phase):

    • Fever

    • Malaise, fatigue

    • Sore throat

    • Cough

    • Headache

  • Skin and mucous membrane involvement:

    • Painful red or purplish rash that spreads rapidly

    • Blisters and target-like (bull’s-eye) lesions on the skin

    • Widespread skin peeling and sloughing (epidermal detachment)

    • Mucosal erosions and ulcers affecting the mouth, eyes, lips, genitalia, and sometimes the respiratory or gastrointestinal tract

  • Other symptoms:

    • Difficulty swallowing or eating due to painful oral ulcers

    • Eye redness, pain, swelling, and possible vision problems

    • Painful urination or genital discomfort

Investigations & Lab Results

1. Clinical Diagnosis

  • Diagnosis is primarily clinical, based on the characteristic skin and mucosal findings along with history of drug exposure or infection.

2. Laboratory Tests

  • Complete blood count (CBC):

    • May show leukopenia or leukocytosis, anemia

    • Elevated inflammatory markers (ESR, CRP)

  • Liver and renal function tests:

    • To assess organ involvement or drug toxicity

  • Electrolytes:

    • To monitor dehydration and electrolyte imbalances from skin loss and mucosal involvement

  • Coagulation profile:

    • To check for bleeding tendencies or DIC (disseminated intravascular coagulation)

3. Microbiological Studies

  • Blood cultures and wound cultures:

    • To identify secondary infections and guide antibiotic therapy

  • Tests for underlying infections:

    • Mycoplasma pneumoniae serology or PCR

    • Viral cultures or PCR if herpes simplex or other viruses suspected

4. Skin Biopsy

  • Confirms diagnosis by showing:

    • Full-thickness epidermal necrosis

    • Subepidermal blister formation

    • Minimal inflammation in the dermis

5. Other Tests

  • Chest X-ray:

    • To assess for respiratory complications if symptoms present

Drugs Associated With Stevens-Johnson Syndrome

1. Antibiotics

  • Sulfonamides (e.g., sulfamethoxazole-trimethoprim) – most frequently implicated

  • Penicillins

  • Cephalosporins

  • Quinolones (e.g., ciprofloxacin)

2. Anticonvulsants (antiepileptics)

  • Phenytoin

  • Carbamazepine

  • Lamotrigine

  • Phenobarbital

  • Valproic acid (less common)

3. Nonsteroidal Anti-Inflammatory Drugs (NSAIDs)

  • Especially oxicam derivatives (e.g., piroxicam, meloxicam)

  • Ibuprofen and naproxen (rare, but reported)

4. Allopurinol

  • A uric acid–lowering agent commonly used in gout

  • High risk, especially in patients with renal impairment

5. Antiretroviral Drugs

  • Nevirapine

  • Abacavir (hypersensitivity reaction)

6. Others

  • Methotrexate

  • Dapsone

  • Modafinil

  • Sertraline and other SSRIs (rarely)

Complications

1. Skin and Mucosal Complications

  • Secondary skin infections, including cellulitis and sepsis

  • Scarring, hyperpigmentation, or hypopigmentation

  • Nail loss or dystrophy

  • Chronic mucosal damage, such as strictures or adhesions in the eyes, mouth, or genitals

2. Ocular Complications

  • Conjunctivitis, corneal ulceration, or uveitis

  • Dry eyes, symblepharon (adhesion of eyelid to eyeball)

  • Blindness (in severe or untreated cases)

3. Respiratory Complications

  • Bronchial epithelial sloughing, pneumonia, or acute respiratory distress syndrome (ARDS)

  • Risk of long-term pulmonary fibrosis

4. Renal and Hepatic Complications

  • Acute kidney injury

  • Liver dysfunction or failure, especially if drug-induced

5. Gastrointestinal Complications

  • Esophageal strictures, erosions, or bleeding

  • Malnutrition due to oral pain and feeding difficulty

6. Psychosocial and Functional Impact

  • Chronic pain and fatigue

  • Psychological trauma, depression, anxiety

  • Long-term disability due to scarring or vision loss

7. Mortality

  • SJS carries a mortality rate of around 5–10%, which increases significantly in more severe forms such as Toxic Epidermal Necrolysis (TEN).

Treatment

1. Immediate Measures

  • Immediate discontinuation of the suspected causative drug

    • This is the most critical step in halting progression.

2. Supportive Care (Mainstay of Treatment)

  • Hospitalization in ICU or burn unit

  • Fluid and electrolyte management to prevent dehydration from skin loss

  • Nutritional support, possibly with feeding tube if oral ulcers are severe

  • Pain control with analgesics

  • Wound care with non-adhesive dressings and aseptic technique

  • Temperature regulation to compensate for impaired skin barrier

  • Eye care with lubricants and ophthalmologic consultation

  • Prevention and treatment of secondary infections, including sepsis monitoring

3. Pharmacologic Treatments (controversial and case-dependent)

  • Systemic corticosteroids

    • May be used early in the course but carry a risk of infection; use remains debated.

  • Intravenous immunoglobulin (IVIG)

    • Believed to inhibit Fas-mediated apoptosis; used in some cases with variable results.

  • Cyclosporine

    • Immunosuppressive drug shown in some studies to reduce mortality.

  • Plasmapheresis

    • May be used to remove circulating drug metabolites and immune complexes (rarely used).

  • TNF-alpha inhibitors (e.g., etanercept)

    • Emerging treatment, still under investigation.

4. Long-Term Management

  • Multidisciplinary follow-up (dermatology, ophthalmology, urology, etc.)

  • Treatment of sequelae such as ocular damage, mucosal scarring, or psychological trauma

  • Avoidance of triggering drugs in the future

  • Genetic screening (e.g., HLA-B*1502 in Asians before carbamazepine use)

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