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Title of Guideline

The management of patients with


Toxic Epidermal Necrolysis
or Stevens Johnson Syndrome
within Adult Critical Care
Contact Name and Job Dr Martin Levitt,
Title (author)
Consultant in Intensive Care, Critical Care, NUH
Dr Martin Beed
Consultant in Intensive Care, Critical Care, NUH
Directorate & Speciality Specialist Support
Adult Critical Care
Date of submission 1/12/2013
Date on which 1/12/2018
guideline must be
reviewed
Explicit definition of Applies to all adult patients requiring Critical Care admission
patient group to which it
applies (e.g. inclusion for management of Toxic Epidermal Necrolysis or Stevens
and exclusion criteria, Johnson Syndrome
diagnosis)
Excludes: All paediatric cases
Abstract This guideline describes the procedures and management
plan associated with the Critical Care Management of the
above patients.
Management and care are shared between Burns Team
ICU Team, Dermatology Team
Key Words Toxic Epidermal Necrolysis, Stevens Johnson Syndrome,
Adult critical care
Evidence base of the guideline:
Peer reviewed by: NUH critical care consultants; Critical care cross-town
guidelines group
Evidence base: (1-5)
4 expert committee reports or opinions and / or clinical experiences of respected authorities
5 recommended best practice based on the clinical experience of the guideline developer
Consultation Process Cross-town guidelines group
Burns and dermatology consultants
Target audience Medical and nursing staff all adult critical care areas
This guideline has been registered with the trust. However, clinical guidelines are guidelines only. The
interpretation and application of clinical guidelines will remain the responsibility of the individual
clinician. If in doubt contact a senior colleague or expert. Caution is advised when using guidelines
after the review date.

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NOTTINGHAM UNIVERSITY HOSPITALS NHS TRUST

CRITICAL CARE GUIDELINES

Guidance for the management of patients with Toxic Epidermal Necrolysis

(or Stevens Johnson Syndrome) within Adult Critical Care

Introduction:

Toxic epidermal necrolysis (TEN) is a rare, potentially life threatening dermatological


condition. It is characterised by widespread detachment of the epidermis from the underlying
dermis as a result of immune mediated keratinocyte death.
TEN is regarded as part of a spectrum of conditions that include Stevens-Johnson syndrome
(SJS), the differentiation being the percentage skin involvement.
The condition is usually precipitated by an adverse reaction to a drug (see table 1) but may
also be associated with infections such as mycoplasma and HSV, and following bone marrow
transplantation.
The overall mortality for TEN is around 30% with the more severe cases having greater than
90% risk of death. For this reason TEN is managed on Critical Care in a similar manner to a
thermal burn injury.

Table 1. Common drug causes of TEN

Sulfonamides
Beta lactam antibiotics
Macrolides
NSAIDS
Allopurinol
Methotrexate
Antiretroviral drugs
Phenobarbitone
Carbamazepine
Sodium valproate
Corticosteroids

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Diagnosis

Diagnosis may be suspected from the clinical appearance and temporal association with drug
administration. It is usual for the diagnosis to be made or confirmed by the dermatologists.
Referral is then made to the burns team at Nottingham University Hospital, City Campus for
further management. Skin biopsy is not considered essential, but may be helpful in cases
where there is diagnostic uncertainty and in those uncommon cases that fail to re-
epithelialize.

Assessment of severity

Although thought of as a cutaneous disease, TEN can affect all mucous membranes.
Typically the eyes and mouth are involved, but involvement of the genitalia, gastro-intestinal
tract and the respiratory epithelium can occur. On admission to Critical Care, the patient
should be examined thoroughly and the extent and location of the skin involvement
documented. Note that the disease may progress following admission.

It is customary to assess the patient using the SCORTEN scoring system (table 2). This was
developed in the 1990’s and gives an approximate mortality prediction based upon the
presence or absence of 7 predictive factors at admission . It should be noted that the
confidence intervals are extremely wide and overlapping.

Table 2. SCORTEN Severity of illness score for TEN

Age >40 years

Malignancy

Heart rate >120

Initial percentage of epidermal detachment >10%

Serum Urea >10 mmol/L

Serum glucose level >14 mmol/L

Bicarbonate level <20 mmol/L

SCORTEN Score Mortality %


0-1 3
2 12
3 35
4 58
5+ 90+

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Critical Care Management.
With the obvious exception of stopping all potential causative agents, there is no definitive
treatment for TEN. Management is therefore resuscitative and supportive in the anticipation
that the epidermis will regenerate allowing re-epithelialisation. The management of TEN
patients is complex and requires a multidisciplinary team approach involving Critical Care,
burns team, dermatology and the ophthalmology / corneal team.

1. Ensure that all possible causative agents have been stopped.

2. If possible, the patient should be admitted to a single room to facilitate infection control
and control of the thermal environment.

3. Perform an initial ABC assessment of the patient. Immediate intubation is seldom


required, but the majority of patients require ventilation at some stage of their
admission.

4. Unless resuscitation has been commenced, the patient should be assumed to be


hypovolaemic. Obvious gross volume depletion should be corrected using a colloid of
choice. Following this, initial volume requirements can be calculated using a
modification of the Baxter-Parklands formula. Because skin involvement can be
patchy, it is difficult to accurately assess the area of skin loss.

Volume requirements are given by:

Skin area affected x body weight x 3 ml.

This is approximately 2/3 of the thermal burn requirement and is given over the first 24
hour period. There is no evidence of benefit for colloids over crystalloids and it is
usual to use Hartmann’s solution. Fluid requirements beyond the first 24 hours should
be managed according to the patient’s condition.

5. Patients with TEN are likely to have protracted ICU stay. The loss of skin precludes
non-invasive BP monitoring. Patients should have arterial and central venous access
achieved through unaffected skin. It is usual to utilise non-invasive cardiac output
monitoring to guide volume requirements and the need for inotropic or vasopressor
agents.

6. Measures to promote skin healing may be used: keeping the patient in a warmed
environment; warming peripheral skin to avoid a core-peripheral gradient of >2ºC;
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avoidance of vasopressors unless severe hypotension occurs which is intractable to
fluids (or there is evidence that the patient’s fluid balance is too positive / tissue
oedema is problematic)

7. Nasogastric or naso-jejunal feeding should be established as soon as practical


according to the ICU protocol.

8. TEN is painful. Non-intubated patients will require opiate analgesics. Ventilated


patients will require sedation with morphine and midazolam. Dressing changes will
require additional analgesia e.g. ketamine, propofol, remifentanil.

9. The Burns team will be aware of the patient’s admission and will attend to carry out
dressings. The routine de-roofing of blisters is not recommended. Patients with TEN
are at risk of wound infection. There is no evidence for the use of one type of dressing
over another, but current opinion favours the use of nano-crystaline silver dressings on
the basis of stronger antimicrobial action and decreased frequency of dressing
changes. Dressing changes are at the discretion of the Burns Consultants, but should
always form part of the screening in patients who develop sepsis.

10. Ophthalmology assessment. Ophthalmic involvement in TEN can lead to blindness


and the development of adhesions. The corneal team, based at QMC, should be
notified of the admission and should assess the patient within 24 hours of admission.
The current recommendations are:

a. Topical eye lubricants 1-2 hourly


b. Topical antibiotics (preservative free)
c. The application of amniotic membrane to the corneas to prevent the
development of symblepharon formation guided by corneal team.

11. Therapeutics. Patients with TEN should receive routine thromboprophylaxis and
stress ulcer prophylaxis. Intubated patients should receive oral decontamination.
 There is no indication for prophylactic antibiotic administration.

 There is no evidence for benefit in the use of plasmapheresis,

corticosteroids, cyclophosphamide, cyclosporin, or TNF-alpha inhibitors.

 Thalidomide was associated with an increase in mortality.

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12. Intravenous Immunoglobulin: The role of IVIG remains controversial, with little
evidence for benefit and some for potential harm. There is no consensus, so the use
of IVIG remains at the discretion of the dermatology or the burns teams. TEN is listed
in the DOH guidelines for use of IVIG.

13. Infection issues: TEN patients are at high risk of developing infection. Wound
colonization is usual and wound infection common. The use of nano-crystaline silver
dressings may reduce this. Regular surveillance cultures should be performed at each
dressings change. Other sources of infection include lines and VAP.

Persistent fever is common in patients with TEN, and does not always indicate the
presence of infection. The following indices may be used to aid the decision-making
process:
 New fever (>38ºC, when the patient was previously apyrexial)
 Fever >39ºC
 Hypothermia (<36.5ºC)
Especially where any of the above occurs in combination with any of:
 New tachycardia (>110 beats per minute)
 New tachypnoea (>25 breaths per minute); or increasing O2 requirement
 New thrombocytopaenia (<100x109)
 New hyperglycaemia
 New diarrhoea
 New failure to absorb enteral feed

Antimicrobials should be prescribed in the presence of suspected or proven infection


and on the advice of microbiology. In case of suspected sepsis, a full dressing change
must be performed. Blood and sputum cultures should be taken and consideration
given to requirement for resiting invasive lines. Empirical antimicrobial cover should
include vancomycin, an antipseudomal such as piperacillin-tazobactam and an
aminoglycoside. Discuss the addition of an antifungal with the duty microbiologist

14. Pyrexia: the presence of a core body temperature >39ºC should prompt measures to
cool the patient (cooled IV fluids, peripheral cooling, paracetamol).

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NSAIDs as antipyretics may only be prescribed at the direction a consultant and must
not be used if there is the possibility that they may have been the initial trigger

15. Patients who recover from TEN must be aware of the potential for re-exposure to the
causative drug and of the risks of cross reactivity, especially with anticonvulsants and
NSAIDS. The potential for a genetic basis for TEN suggests that blood relatives
should avoid exposure to trigger agents.

References:

Toxic epidermal necrolysis: current evidence, practical management and future directions
Chave TA, et al British Journal of Dermatology 2005;153:241–253

Toxic Epidermal Necrolysis and Stevens Johnson Syndrome: Our Current Understanding.
French LE Allergology International. 2006;55:9-16

SCORTEN: A Severity-of-Illness Score for Toxic Epidermal Necrolysis


Bastuji-Garin S, et al Journal of Investigative Dermatology 2000;115:149-153

Toxic Epidermal Necrolysis: Does Immunoglobulin Make a Difference?


Brown KM, et al Journal of Burn Care & Rehabilitation 2004;25(1):81-88

Treatment of Toxic Epidermal Necrolysis With High-Dose Intravenous Immunoglobulins


Multicenter Retrospective Analysis of 48 Consecutive Cases
Prins C, et al; for the TEN-IVIG Study Group Arch Dermatol 2003;139:26-32.

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Equality Impact Assessment Report

1. Name of Policy or Service


Response to external best practice policy

2. Responsible Manager
Owen Bennett (Clinical Quality, Risk and Safety Manager)

3. Name of person Completing EIA


Dr Martin Beed (Consultant in Intensive Care and
Anaesthesia)

4. Date EIA Completed


1/4/2011

5. Description and Aims of Policy/Service

This clinical guideline has been written to inform adult critical care
staff of how to safely manage critically ill patients requiring a
tracheostomy.

6. Brief Summary of Research and Relevant Data


There is no research or relevant data at the present time.

7. Methods and Outcome of Consultation


Consultations have been carried out with the following:

Adult critical care consultants and senior nurses

Comments from the above consultations have been received


and incorporated where appropriate.

8. Results of Initial Screening or Full Equality Impact


Assessment:

Equality Group Assessment of Impact

Age No Impact Identified

Gender No Impact Identified

8
Race No Impact Identified

Sexual Orientation No Impact Identified

Religion or belief No Impact Identified

Disability No Impact Identified

Dignity and Human No Impact Identified


Rights

Working Patterns No Impact Identified

Social Deprivation No Impact Identified

9. Decisions and/or Recommendations (including


supporting rationale)

From the information contained in the procedure, and


following the initial screening, it is my decision that a full
assessment is not required at the present time.

10. Equality Action Plan (if required)

N/A

11. Monitoring and Review Arrangements

1/12/2018