Steven johnson syndrome-

Stevens johnsonsyndrome published on 3/5/17, rare conditions due to drug allergy

1. Good Evening

2. STEVENS-JOHNSON SYNDROME   PRESENTED BY: 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,