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Stevens-Johnson syndrome

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Dr A Galetto

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CASECASE

A 7 years old boy has fever and pain on swallowingsince five days. Two days ago an eruption began on

his thorax with very painful macules and yesterday

some blister developed on his face and began to be

confluent with area of epidermal detachment.

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His eyes are now red and recovered with purulent

discharge and his lips become swollen with erosions. He

can not drink anymore because of pain on oral lesions.

He is treated for epilepsy since 3 years and his treatment

changed 2 weeks ago for a treatment withcarbamazepine

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Question:

You are his doctor and you are wondering:

How to manage this case ?

What is the probable cause of his illness ?

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Definition

Stevens-Johnson and toxic epidermal necrolysisare

severe mucocutaneous reaction triggered by medications characterized by extensive necrosis and

detachment of the epidermis

Are variants of a same disease but are distinguished only by the severity and the

percentage of the body surface involved by

blisters and erosions

Are rare disorders: 1 per 1000000 Lyells syndrome is equivalent to toxic epidermal

necrolysis

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Clinical features

Acute phase (unspecific):

Begin in the 3 weeks following introduction of a medication or immunization

Fever Photophobia and conjonctival itching Pain on swallowing Malaise and myalgia During one to three days before cutaneous

lesions

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Clinical features

Cutaneous lesions:

Confluent erythematous macules with purpuriccenters

Skin pain out of proportion to the cutaneousfindings

Atypical target lesions with darker centers maybe present

As the disease progresses: Vesicules and bullae form Areas of epidermal detachment develop

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Generalized eruption of lesions that initially had a target-like

appearance but then became confluent, brightly erythematous,

and bullous.

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Multiple bulles overlying diffuse erythema are

present.

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Diffuse erythema and large areas of denuded

epidermis are present.

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Multiple bullae and areas of denuded epidermis

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widespread erythema and confluent vesiculation, leading

to detachment of the skin.

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Nikolsky sign:

Is the ability to extend the area of superficialskin detachment by applying gentle lateral

pressure on the surface of the skin at an

apparently uninvolved site.

The ultimate appearance of the skin is like an extensive thermal injury

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Clinical features

Mucosal lesions:

Painful crust and erosions may occur on anymucosal surface

Occurs in 90 % of cases

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Clinical features

Oral: Oral mucosa and the vermillon border are

almost invariably involved

Painful hemorragic erosions recovered witha grayish-white membrane

Stomatitis and mucositis lead to imparedoral intake malnutrition and dehydratation

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Multiple erosions and crusts are present on the lips of this

patient with Stevens-Johnson syndrome.

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Clinical features

Ocular

Pain and photophobia Severe conjonctivitis with purulent discharge

and bullae

Corneal ulceration is frequent

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Marked conjunctival injection and discharge

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Clinical features

Urogenital:

Urethritis and genital erosions In women: erosive and ulcerative vaginitis

Pharyngeal mucosa is affected in nearly all patients.

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Classification

Extension of skin involvement differenciates betweenStevens-Johnson syndrom, overlap syndrom and toxic

epidermal necrolysis

Only necrotic skin which is already detached (erosionsor blisters) or detachable skin ( nikolsky positive) should

be included in the surface of skin involvement

For helping in scoring the skin involvement, rememberthat the surface of the patients hand corresponds to

one percent of his total body surface area.

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Pathogenesis.

Drug hypersensitivity.

Genetic factors are linked to drug hypersensitivity

HIV infection: incidence is1000-fold higher

Mycoplasma pneumoniae and herpes virus: to cause Stevens-johnson in children without drug

exposure

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Drugs associated with Stevens-johnsonDrugs associated with Stevens-johnson

Strongly associated* Associated

Allopurinol

Carbamazepine

Lamotrigine

Meloxicam

Nevirapine

Phenobarbital, primidone

Phenytoin, fosphenytoin

Piroxicam, tenoxicam

Sulfadiazine, sulfadoxine,

sulfamethoxazole,

sulfasalazine

Amifostine

Amoxicillin, ampicillin

Azithromycin, clarithromycine,

erythromycin

Cefadroxil, cefixim,

ceftriaxone, cefuroxim

Ciprofloxacin, levofloxacin,

pefloxacin

Diclofenac

Doxycyclin

Etoricoxib

Metamizole

Oxcarbazepine

Pipemidic acid

Rifampicine

25% of cases cannot be clearly attributed to a drug

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Complications:

Acute

Massive loss of fluids and electrolytes through denudeskin

Electrolytes imbalance Hypovolemic shock with renal failure Bacteremia Insulin resistance Hypercatabolic state Hepatic involvment and pancreatitis Leukopenia, thrombocytopenia and anemia pneumonia Multiple organ failure

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Complications:

Long term sequelae

Dermatologic: scarring Ophtalmologic: corneal scarring Oral and genital: synechiae and stenosis

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Prognosis:

Mortality rate is near 25 % Disease severity is the main risk factor of death

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Laboratory abnormalities:

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

Case definition:

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Differential diagnosis:Differential diagnosis:

Erythema multiforme:Target lesions on the extremities

Associated with herpes infections

Target lesions with central bullae are

present on the hand in erythema

multiform

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Differential diagnosis:Differential diagnosis:

auto-immune blistering diseases:IgA dermatosis

pemphigus

skin biopsy: deposits of immunoglobulins

Linear IgA bullous dermatosis resembling toxic epidermal

necrolysis with extensive bullae and skin detachment

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Differential diagnosisDifferential diagnosis

Staphylococcal scalded skin syndrome (SSSS)

Caused by epidermolytic toxin produced by certain strains of Staphylococci

Neonates and young children Mucous membranes are not involved Skin biopsy: Necrosis of only the upper layers of the epidermis.

Diffuse erythema and

desquamation are present in

this child with staphylococcal

scalded skin syndrome.

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Differential diagnosisDifferential diagnosis

Skin biopsy of Stevens-Johnson syndrome: full thickness epidermal necrosis

(A) Diffuse confluent erythematous macules and patches with dusky

centers and multiple bullae in a patient recently started on several

new medications.

(B) There is a cell-poor subepidermal blister and full epidermal

necrosis.

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

management:

Immediate withdrawal of any potential causative agents

Supportive care wound care:

sterile handling of the wound, use antisepticsolutions and nonadherent gauze dressings

Ocular care: daily cleaning and lubrication with drops or

ointment

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Stevens-johnson and toxic epidermal necrolysisStevens-johnson and toxic epidermal necrolysis

management:

Fluid and electrolytes management Nutr