jurding SJS.pptx

8/14/2019 jurding SJS.pptx

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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


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Clinical History

Nonspecific upper respiratory tractinfection (Cough, thick purulentsputum)

1

14 day prodrome sign

Headache, malaise, arthralgia, sorethroat, chills, fevers, vomiting,

diarrhea

Mucocutaneous lesions developabruptly last from 2-4 weeks


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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-the-counter medication

Target lesions are not always seen in SJS


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Signs and Symptoms

Orthostasis

Tachycardia

Hypotension

Altered level of consciousness

Epistaxis

Conjunctivitis

Corneal ulcerations

Erosive vulvovaginitis or balanitis

Seizures, coma


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Physical Findings

Typical lesion : target lesion. The core may be

vesicular, purpuric, or necrotic; surrounded by

macular erythema

Macules

Vesicles,bullous

Urticarialplaques

ConfluentErythema

Rupture ofbullae/vesicles

Location: palms, soles, dorsum of the

hands, extensor, trunk

Mucosal: erythema, edema, sloughing,

blistering, ulceration, and necrosis


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Physical Findings

Erythema multiforme Stevens - Johnson Syndrome


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Physical Findings

Erythema multiforme Confluent Erythema


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Physical Findings

Vesicles Bullous Manifestasion


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Physical Findings

Ruptured of Vesicles or Bullae; crust as an secondary lesion


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Physical Findings

Toxic Epidermal Necrolysis; Picture of detachment of epidermal layer


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Physical Findings

Clinical Entity SJS SJS-TEN Overlap TEN

Primary Lesion Dusky red lesions Flat atypical targets

Dusky red lesions Flat atypical targets

Poorly delineatederythematous

plaques

Epidermal

detachment

Dusky red lesions

Flat atypical targets

Distribution Isolated lesions

Confluence (+) on

face and trunk

Isolated lesions

Confluence (++) on

face and trunk

Isolated lesions

(rare)

Confluence (+++)

on face, trunk, and

elsewhere

Mucosal involvement Yes Yes Yes

Systemic Symptoms Usually Always Always

Detachment (%BSA) 30


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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 urinefor blood

Electrolytesand other chemistries test

Cultures of blood, urine, and wounds infection suspected

Bronchoscopy, esophagogastroduodenoscopy (EGD), and

colonoscopy may be indicated


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Management

Airway Breathing and Circulation evaluation

Fluid replacement and electrolytecorrection(include Phosphorus Level)

Environmental temperature control, careful and aseptichandling, sterile field creation, avoidance of any adhesivematerial, maintenance of venous peripheral accessdistant from affected areas (no central line when

possible), initiation of oral nutrition by nasogastric tube,anticoagulation, prevention of stress ulcer

Skin lesions are treated as burns

Pain and anxiety control


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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


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Management

Manage oral lesions with mouthwashes, antiseptics

Reducing pain and allowing the patient to take in fluids

Topical anesthetics

Staphylococcus aureus, Pseudomonas aeruginosa, &

Enterobacteriaceae

Prophylactic antibiotics ?

Compresses of saline or Burow solution

Covered areas of denuded skin

Offending drugs must be stopped


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Management

Address tetanus prophylaxis

Hyperglycemialeads to overt glycosuria or to increased

osmolarity

Insulin

oxandrolone and human growth factor are effective fordecreasing hypercatabolism and net nitrogenous loss

ornithine alpha-ketoglutarate supplementation of enteralfeeding is effective to reduce wound healing time

high dose ascorbic acid (66 mg/kg per hour) given during thefirst 24 hours reduces fluid volume requirements

Intravenous and oral supplementation on burn care


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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

Topical Management


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Management

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

Others


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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


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Prognosis

Risk factor 0 1

Age < 40 years > 40 years

Associated malignancy no yes

Heart rate (beats/min) 120

Serum BUN (mg/dL) 27

Detached or compromised body surface 10%

Serum bicarbonate (mEq/L) >20


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Prognosis

No of risk factors Mortality rate

0-1 3.2%

2 12.1%

3 35.3%

4 58.3%

5 or more >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

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