Stevens-Johnson syndrome is a rare but serious disorder causing skin and mucous membrane detachment. It is usually caused by an unpredictable adverse drug reaction or infection. The main symptoms include painful sores or lesions in the mouth, eyes, genitals and other mucus membranes. Treatment focuses on identifying and stopping the causative agent, providing supportive care, and managing complications which can include infections, organ damage and even death in severe cases.

2. STEVENS-JOHNSON
SYNDROME
PRESENTEDBY:
Mr. mAnu chacko

3. Stevens-Johnson Syndrome
Stevens-Johnson syndrome (SJS) is an
immune-complex–mediated hypersensitivity
complex that typically involves the skin and the
mucous membranes. Stevens-Johnson syndrome is
a serious systemic disorder with the potential for
severe morbidity and even death.

4. INCIDENCE
• The incidence rate is 7 cases per million
population per year.
• Cases tend to have a propensity for the early
spring and winter.
• Stevens-Johnson syndrome has been described
worldwide in all races, although it may be more
common in whites. Interestingly, disease is not
limited to humans;

5. TYPES
• Stevens-Johnson syndrome - A "minor form of
TEN," with less than 10% body surface area
(BSA) detachment.
• Overlapping Stevens-Johnson syndrome/toxic
epidermal necrolysis (SJS/TEN) - Detachment
of 10-30% BSA
• Toxic epidermal necrolysis - Detachment of
more than 30% BSA

6. Etiology
The 4 etiologic categories are as follows:
• Infectious
• Drug-induced
• Malignancy-related
• Idiopathic

7. Infectious causes
• Viral diseases that have been reported to cause
Stevens-Johnson syndrome include the following:
• Herpes simplex virus
• AIDS
• Coxsackie viral infections
• Influenza
• Hepatitis
• Mumps

8. Infectious causes
• Bacterial etiologies include the following:
• Group A beta-hemolytic streptococci
• Diphtheria
• Brucellosis
• Mycobacteria
• Mycoplasma pneumoniae
• Rickettsial infections
• Typhoid

9. Drug-induced
• Antibiotics are the most common cause
of Stevens-Johnson syndrome, followed
by analgesics, cough and cold
medication, NSAIDs, psycho epileptics,
and anti gout drugs. Of antibiotics,
penicillins and sulfa drugs are
prominent; ciprofloxacin has also been
reported.

10. Drug-induced
• Stevens-Johnson syndrome has also been
reported in patients taking the following drugs:
• alpha antagonists
• Cocaine
• Sertraline
• Pantoprazole
• Tramadol

11. Genetic factors
• Carriage of the certain human leukocyte
antigens has been associated with increased
risk.

12. Idiopathic
• Stevens-Johnson syndrome is idiopathic in
25-50% of cases.

13. Pathophysiology
• A delayed hypersensitivity reaction has been
implicated in the pathophysiology of Stevens-
Johnson syndrome.
• Antigen presentation and production of tumor
necrosis factor (TNF)–alpha by the local tissue
dendrocytes
• results in the recruitment and augmentation of
T-lymphocyte proliferation and enhances the
cytotoxicity of the other immune effector cells.

14. Pathophysiology
• A "killer effector molecule" has been identified
that may play a role in the activation of cytotoxic
lymphocytes. It causes death of keratinocytes
• The death of keratinocytes causes separation of
the epidermis from the dermis.
• This can perpetuate the inflammatory process,
which leads to extensive epidermal necrolysis.

15. Clinical MANIFESTATIONS
• Cough productive of a thick purulent sputum
• Headache
• Malaise
• Arthralgia
• Fever
• Tachycardia
• Hypotension

16. Clinical MANIFESTATIONS
• Altered level of consciousness
• Epistaxis
• Conjunctivitis
• Corneal ulcerations
• Seizures
• Coma

17. Clinical MANIFESTATIONS
• Foreign body sensation
• Decreased vision
• Burn sensation
• Photophobia
• Diplopia

18. Clinical MANIFESTATIONS
In addition to the skin, lesions in Stevens-Johnson
syndrome may involve the following parts of the body:
• Oral mucosa
• Esophagus
• Pharynx
• Larynx
• Anus
• Trachea
• Vagina
• Urethra

19. DIAGNOSTIC EVALUATION
• There are no specific laboratory studies (other than
biopsy) that can definitively establish the diagnosis of
Stevens-Johnson syndrome.
• Serum levels of the following are typically elevated in
patients with Stevens-Johnson syndrome:
• Tumor necrosis factor (TNF)-alpha
• Soluble interleukin 2-receptor
• Interleukin 6
• C-reactive protein

20. Treatment & Management
Most patients are treated symptomatically
• Identify and stop the offending agent.
• Give IV fluids.
• Give corticosteroids.
• Antibiotic to treat secondary infection.
• Manage oral lesions with mouthwashes. Topical
anesthetics are useful in reducing pain and
allowing the patient to take in fluids.

21. Treatment & Management
• Skin lesions are treated as burns. Areas of
denuded skin must be covered with
compesses of saline.
• Address tetanus prophylaxis.
• Treatment of acute ocular manifestations usually
begins with aggressive lubrication of the ocular
surface, topical steroids, antibiotics

22. Complications
• Corneal epithelial defects
• Corneal stromal ulcers
• Corneal perforation
• Endophthalmitis
• Renal tubular necrosis,
• renal failure,