Stevens-Johnson Syndrome - PowerPoint PPT Presentation

About This Presentation
Title:

Stevens-Johnson Syndrome

Description:

A Brief Summary Source Uptodate Articles Introduction Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe idiosyncratic reactions. – PowerPoint PPT presentation

Number of Views:5648
Avg rating:3.0/5.0
Slides: 21
Provided by: keofficia
Category:

less

Transcript and Presenter's Notes

Title: Stevens-Johnson Syndrome


1
Stevens-Johnson Syndrome
  • A Brief Summary

2
Source
  • Uptodate Articles

3
Introduction
  • Stevens-Johnson syndrome (SJS) and toxic
    epidermal necrolysis (TEN) are severe
    idiosyncratic reactions.
  • They are most commonly triggered by medications,
    which are characterized by fever and
    mucocutaneous lesions leading to necrosis and
    sloughing of the epidermis.
  • SJS and TEN are distinguished chiefly by severity
    and percentage of body surface involved.

4
Stevens-Johnson syndrome
  • SJS is the less severe condition, in which skin
    sloughing is limited to less than 10 percent of
    the body surface.
  • It is characterized by a prodrome of malaise and
    fever, followed by the rapid onset of
    erythematous or purpuric macules and plaque.
  • The skin lesions progress to epidermal necrosis
    and sloughing.
  • Mucosal membranes are affected in 92 to 100
    percent of patients, usually at two or more
    distinct sites (ocular, oral, and genital)

5
Toxic epidermal necrolysis
  • Toxic epidermal necrolysis (TEN), or Lyell's
    syndrome, involves sloughing of greater than 30
    percent of the body surface area.
  • TEN also begins with a prodrome of fever and
    malaise, although temperatures are typically
    higher than those seen with SJS, often exceeding
    39 degrees Celsius.
  • Mucous membranes are involved in nearly all
    cases.

6
TEN (continued)
  • The skin lesions are widely distributed
    erythematous macules and patches, although about
    50 percent of cases begin with diffuse erythema.
  • The skin lesions progress to full-thickness
    epidermal necrosis leads.
  • The ultimate appearance of the skin has been
    likened to that of extensive thermal injury.

7
SJS/TEN overlap syndrome
  •  SJS/TEN overlap syndrome describes patients with
    involvement of greater than 10 percent, but less
    than 30 percent of body surface area.

8
Etiology
  • Medications are the leading trigger of SJS and
    TEN in both adults and children.
  • In adults, medications cause 30 to 50 percent of
    cases of SJS and up to 80 percent of cases of
    TEN.
  • Infections are the next most common trigger of
    adult SJS (up to 15 percent).
  • Rare causes of SJS and TEN include vaccinations,
    systemic diseases, chemical exposure, herbal
    medicines, and foods.

9
Medications
  • The following groups of agents are most commonly
    implicated
  • Anti-gout agents (especially allopurinol)
  • Antibiotics (sulfonamides gtgt penicillins gt
    cephalosporins)
  • Antipsychotics and anti-epileptics
    (including carbamazepine, dilantin,lamotrigine,
    and phenobarbital)
  • Analgesics and non-steroidal anti-inflammatory
    agents (especially piroxicam)

10
HISTORY AND CLINICAL PRESENTATION
  • Drug exposure commonly precedes the onset of
    symptoms by one to three weeks (average 14 days)
    in medication-related cases.
  • Reexposure may result in onset of symptoms in as
    little as 48 hours.

11
Prodrome 
  • SJS and TEN typically have a prodrome of fever
    and influenza-like symptoms one to three days
    before the development of mucocutaneous lesions.
  • Fever is usually higher with TEN, and often
    exceeds 39 degrees Celsius.
  • Skin tenderness, photophobia, and conjunctival
    itching or burning may be early symptoms in both
    conditions.

12
Clinical presentation
  • The following signs and symptoms, when present
    early in the course of a drug reaction or
    illness, should alert clinicians to the
    possibility of SJS/TEN
  • Confluent erythema (erythroderma)
  • Facial edema or central facial involvement
  • Skin pain
  • Palpable purpura
  • Skin necrosis
  • Blisters and/or epidermal detachment
  • Mucous membrane erosions and crusting
  • Swelling of tongue

13
Clinical presentation
  • There may be multiorgan involvement.
  • In the absence of complications, the disorder
    generally resolves sufficiently that the patient
    can be discharged from the hospital in two to
    four weeks.

14
diagnosis
  • the diagnosis of SJS or TEN would be appropriate
    in a patient with
  • A suggestive history of antecedent drug exposure
    or illness
  • A prodrome of acute-onset febrile illness and
    malaise
  • Erythematous macules, targetoid lesions, or
    diffuse erythema progressing to vesicles and
    bullae
  • Necrosis and sloughing of the epidermis (of
    varying degrees)

15
diagnosis
  • The diagnosis of SJS or TEN is clinical.
    Histologic findings on skin biopsy are
    supportive, but not independently diagnostic.
  • The differential diagnosis includes
  • erythema multiforme
  • other types of severe medication reactions
  • severe reactions to bacterial toxins (eg, toxic
    shock syndrome
  • staphylococcal scalded skin syndrome)
  • Kawasaki disease.

16
Management
  • Early recognition and immediate withdrawal of any
    potentially causative agents are critical first
    steps in the management of SJS/TEN.
  • Multiple specialists should be involved in the
    care of patients with SJS/TEN when possible,
    including experts in
  • critical care
  • plastic surgery
  • Dermatology
  • infectious disease
  • Ophthalmology
  • nutrition

17
To prevent possible sepsis
  • Sepsis is the major cause of death. Sterile
    handling, infection control measures, topical
    antibiotic agents, and surveillance cultures of
    possible sites of superinfection are important
    components of prevention. 

18
Supportive care gt adjunctive therapies
  • Supportive care should be the primary focus of
    management of SJS/TEN.
  • Beyond this, there is insufficient evidence to
    establish the benefit of any adjunctive
    therapies.
  • Systemic glucocorticoids and intravenous
    gammaglobulin (IVIG) are commonly used at many
    centers, although not all.
  • Other treatments plasmapheresis, thalidomide.

19
Prognosis
  • The mortality of SJS is 1 to 3 percent, while the
    mortality of TEN ranges from 25 to 35 percent.
  • Predictors of mortality include older age at
    onset and greater extent of skin involvement.
  • Long-term sequelae of the skin and eyes are
    common among survivors.

20
End
  • Thank you.
Write a Comment
User Comments (0)
About PowerShow.com