Stevens-Johnson Syndrome-A life threatening skin disorder: A review

Tyagi S, Kumar S, Kumar A, Singla M and Singh A J. Chem. Pharm. Res., 2010, 2(2): 618-626

Supervisor Presentant

: dr. Vitalis Pribadi, M.Kes, Sp.KK : Frans Jeremy (2011.061.128)

Introduction
Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis
(TEN) Acute skin blisters and mucous membrane erosions Necrosis of the epidermis and other epithelia The extent of skin detachment: 10% for SJS and 30% for TEN

Introduction
An immune-complexmediated hypersensitivity complex that is
a severe expression of erythema multiforme Many drugs (antidepressant, TNF), viral infections, and malignancies. 25-50 % idiopathic cause Involves the skin and the mucous membranes GI and respiratory involvement may progress to necrosis Severe morbidity and even death

Classification

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

Clinical History
Nonspecific upper respiratory tract infection (Cough, thick purulent sputum)

1 14 day prodrome sign

Headache, malaise, arthralgia, sore throat, chills, fevers, vomiting, diarrhea

Mucocutaneous lesions develop abruptly last from 2-4 weeks

Signs and Symptoms

Rash, blisters, or red splotches on skin Persistent fever Blisters in mouth, eyes, ears, nose, genital area Swelling of eyelids, red eyes Flu-like symptoms Recent history of having taken a prescription or over-thecounter medication Target lesions are not always seen in SJS

Signs and Symptoms

Orthostasis Tachycardia Hypotension Altered level of consciousness Epistaxis

Conjunctivitis
Corneal ulcerations Erosive vulvovaginitis or balanitis Seizures, coma

Physical Findings
Typical lesion : target lesion. The core may be vesicular, purpuric, or necrotic; surrounded by macular erythema
Confluent Erythema Urticarial plaques Vesicles, bullous Macules

Rupture of bullae/vesic les

Location: palms, soles, dorsum of the hands, extensor, trunk

Mucosal: erythema, edema, sloughing,

blistering, ulceration, and necrosis

Physical Findings

Erythema multiforme

Stevens - Johnson Syndrome

Physical Findings

Erythema multiforme

Confluent Erythema

Physical Findings

Vesicles Bullous Manifestasion

Physical Findings

Ruptured of Vesicles or Bullae; crust as an secondary lesion

Physical Findings

Toxic Epidermal Necrolysis; Picture of detachment of epidermal layer

Physical Findings
Clinical Entity Primary Lesion SJS SJS-TEN Overlap TEN Dusky red lesions Dusky red lesions Poorly delineated Flat atypical targets Flat atypical targets erythematous plaques Epidermal detachment Dusky red lesions Flat atypical targets Isolated lesions Confluence (+) on face and trunk Isolated lesions Isolated lesions Confluence (++) on (rare) face and trunk Confluence (+++) on face, trunk, and elsewhere Yes Always 10-30 Yes Always >30

Distribution

Mucosal involvement Yes Systemic Symptoms Detachment (%BSA) Usually <10

Laboratory Studies
No specific laboratory studies other than biopsy existed CBC : normal white blood cell (WBC) count or a nonspecific leukocytosis (severe elevation superimposed bacterial infection Determine renal function and evaluate urine for blood Electrolytes and other chemistries test Cultures of blood, urine, and wounds infection suspected

Bronchoscopy, esophagogastroduodenoscopy (EGD), and

colonoscopy may be indicated

Management
Airway Breathing and Circulation evaluation Fluid replacement and electrolyte correction (include Phosphorus Level) Skin lesions are treated as burns
Environmental temperature control, careful and aseptic handling, sterile field creation, avoidance of any adhesive material, maintenance of venous peripheral access distant from affected areas (no central line when possible), initiation of oral nutrition by nasogastric tube, anticoagulation, prevention of stress ulcer

Pain and anxiety control

Management
Treatment is primarily supportive and symptomatic
Some have advocated cyclophosphamide, plasmapheresis, hemodialysis, and immunoglobulin

Corticosteroid
Corticosteroids are contraindicated ? Associated with an
increased prevalence of complications 400 or 200 mg prednisone/day, gradually diminished over a 4 to 6

week period

Management
Manage oral lesions with mouthwashes, antiseptics

Topical anesthetics
Reducing pain and allowing the patient to take in fluids

Prophylactic antibiotics ?
Staphylococcus aureus, Pseudomonas aeruginosa, & Enterobacteriaceae

Covered areas of denuded skin

Compresses of saline or Burow solution

Offending drugs must be stopped

Management
Address tetanus prophylaxis Insulin
Hyperglycemia leads to overt glycosuria or to increased osmolarity

Intravenous and oral supplementation on burn care

oxandrolone and human growth factor are effective for decreasing hypercatabolism and net nitrogenous loss ornithine alpha-ketoglutarate supplementation of enteral feeding is effective to reduce wound healing time high dose ascorbic acid (66 mg/kg per hour) given during the first 24 hours reduces fluid volume requirements

Management
Topical Management
Use dressings to protect the detached skin, compresses of saline or Burow solution Topical antiseptics (0.5% silver nitrate or 0.05% chlorhexidine) are used to paint, bathe, or dress the patients Dressings may be gauzes with petrolatum, silver nitrate,

polyvidoneiodine, or hydrogels
Oral, nose and eyes care

Management
Others
Surgery : biologic skin covers after epidermal stripping (cadaveric allografts, cultured human allogeneic or autologous epidermal sheets)

New dressings are being investigated: human newborn

fibroblasts cultured on the nylon mesh of Biobranee In burns, topical recombinant bovine basic fibroblast

growth factor faster granulation tissue formation and

epidermal regeneration

Complications
Ophthalmologic : Corneal ulceration, anterior uveitis, panophthalmitis, blindness Gastroenterologic : Esophageal strictures Genitourinary : Renal tubular necrosis, renal failure, penile scarring, vaginal stenosis Pulmonary : Tracheobronchial shedding with resultant respiratory

failure
Cutaneous : Scarring and cosmetic deformity, recurrences of infection through slow-healing ulcerations

Prognosis
Risk factor Age Associated malignancy Heart rate (beats/min) Serum BUN (mg/dL) Detached or compromised body surface Serum bicarbonate (mEq/L) Serum glucose (mg/dL) 0 < 40 years no <120 <27 <10% >20 <250 1 > 40 years yes >120 >27 >10% <20 >250

The SCORTEN Scale is a severity-of-illness scale with which the severity of certain bullous conditions can be systematically determined

Prognosis
No of risk factors 0-1 2 3 4 5 or more Mortality rate 3.2% 12.1% 35.3% 58.3% >90%

Individual lesions typically should heal within 1-2 weeks (without sequelae) Respiratory failure, renal failure, and blindness Tromboembolism 15% of mortality : bacteremia and sepsis