Accepted Manuscript

Toxic Epidermal Necrolysis (TEN) – The Chelsea Westminster Hospital Wound

Management Algorithm

Christopher Abela , MBBS, FRCS(Plast) Christoph EA. Hartmann , BSc, MBBS,

MRCS A. De Leo , MD Anna de Sica Chapman , MRCP Hetul Shah , MBBS, AICSM,
BSc Mohammad Jawad , MBBS, FRCS Christopher B. Bunker , MA, MD, FRCP Greg
JP. Williams , MBBS FRCS FRCS(Plast) Jorge Leon-Villapalos , MBBS, MSc, DIC,
FRCS(Plast)

PII: S1748-6815(14)00165-X
DOI: 10.1016/j.bjps.2014.04.003
Reference: PRAS 4148

To appear in: Journal of Plastic, Reconstructive & Aesthetic Surgery

Received Date: 19 July 2013

Revised Date: 8 March 2014
Accepted Date: 16 April 2014

Please cite this article as: Abela C, Hartmann CE, De Leo A, de Sica Chapman A, Shah H, Jawad
M, Bunker CB, Williams GJ, Leon-Villapalos J, Toxic Epidermal Necrolysis (TEN) – The Chelsea
Westminster Hospital Wound Management Algorithm, British Journal of Plastic Surgery (2014), doi:
10.1016/j.bjps.2014.04.003.

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 ACCEPTED MANUSCRIPT
Title Page

Title of Article

Toxic Epidermal Necrolysis (TEN) – The Chelsea Westminster Hospital Wound

Management Algorithm

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Authors
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Christopher Abela MBBS, FRCS(Plast) & 1Christoph EA Hartmann BSc, MBBS,

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MRCS, 1A De Leo MD, 2Anna de Sica Chapman MRCP, 2Hetul Shah MBBS,

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AICSM, BSc, 1Mohammad Jawad MBBS, FRCS, 2Christopher B Bunker MA, MD,

FRCP, 1Greg JP Williams MBBS FRCS FRCS(Plast), 1Jorge Leon-Villapalos MBBS,

MSc, DIC, FRCS(Plast)

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Institution to Which the Work Should be Attributed

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Burns Service and 2Department of Dermatology, Chelsea and Westminster Hospital,
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369 Fulham Road, London.

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Author for Correspondence

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Correspondence: Christoph EA Hartmann BSc, MBBS, MRCS

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Burns Service, Department of Plastic Surgery

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Chelsea and Westminster Burns Unit, 369 Fulham Road, London, UK

Email: ceahartmann@gmail.com

Tel: (+44) 0208 237 2500

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SUMMARY

Toxic epidermal necrolysis syndrome (TEN) is a potentially catastrophic exfoliative

muco-cutaneous disorder first described by Lyell in 1956. It represents the most

extensive form of Steven-Johnson Syndrome. TEN is defined varyingly around the

globe, but in the United Kingdom the consensus opinion describes the process as

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involving >30% of the total body surface area. It can rapidly become more extensive

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and threatens life. The estimated annual incidence is approximately 1-2 cases per

million population. The risk of mortality increases with surface area involved and

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meta-analysis of the literature shows this risk to be between 16% and 55%.

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Over a six month period the Chelsea and Westminster Hospital Burns Service treated
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five consecutive patients with more than 80% total body surface area involvement or
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a more than 80% mortality risk, using the severity-of-illness score for toxic epidermal

necrolysis (SCORTEN). All patients were treated according to the Chelsea and
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Westminster Hospital wound management algorithm with excellent outcome and no

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mortalities.
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The aim of this paper is to propose a generic TEN wound management algorithm
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according to the severity of skin lesions, using a simple wound grading system.
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Keywords

Toxic Epidermal Necrolysis Syndrome; TEN; Wound Care; Steven-Johnson

Syndrome; Wound Management; Grading

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 ACCEPTED MANUSCRIPT
TEXT

Toxic epidermal necrolysis syndrome (TEN) is a potentially catastrophic exfoliative

muco-cutaneous disorder first described by Lyell in 1956.1 It presents when the

process is involving >30% of the total body surface area (TBSA, Table 1).2 It can

rapidly become more extensive and threatens life. The estimated annual incidence is

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approximately 1-2 cases per million population.3, 4
The risk of mortality increases

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with surface area involved and meta-analysis of the literature shows this risk to be

between 16% and 55%.5

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TEN seems to be most often initiated by an adverse drug reaction, occasionally

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associated with concomitant viral infection (Table 1). It has also been seen in graft
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versus host disease, malignant disorders and following vaccinations.6 Diagnosis,
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differentials and management of TEN are a multidisciplinary endeavours and plastic

surgeons should be familiar with this. The pathogenesis and medical management of
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TEN in our unit has previously been discussed by de Sica-Chapman et al. in 2010 and
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will not be covered further.7

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Diagnosis and management are multidisciplinary endeavours. Plastic surgeons should

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be familiar with the differential diagnosis of TEN. It may be necessary to arrange

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expeditious patient transfer to a multidisciplinary facility, such as a burns service

where management by a team skilled in wound care, intensive care, pain control and

disease modification has shown improved survival.7 Aggressive medical treatment

and correct early wound management prior to transfer is essential.

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Many pathophysiological mechanisms for TEN have been postulated, but it is

generally accepted that the disease pathogenesis is a drug-induced immune mediated

reaction at the level of the dermo-epidermal junction.

Blister extension at the dermo-epidermal junction is demonstrated easily in this

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condition. when light pressure onto erythematous skin causes dermo-epidermal

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separation. The traction separation of the dermo-epidermal interface is termed a

positive Nikolsky sign.8 Blisters can extend through physical pressure of blister fluid

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on the weakened neighbouring dermo-epidermal interface. The weakened relationship

between the layers of the skin at this specific level is characteristic of TEN.

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The blisters can desquamate, to denude the dermis of its overlying epidermis at the
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disrupted dermo-epidermal junction. Involvement of the palms and soles is a poor

prognostic indicator and suggests extensive cutaneous involvement or a more

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aggressive evolution within the clinical picture. Loss of the physical barrier to
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colonising bacteria predisposes patients to invasive infection and septicaemia, which

translates into the most common cause of mortality. 7 9

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Other epithelial surfaces can be involved leading to gastrointestinal, genitourinary,

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respiratory and conjunctival ulceration. Mucosal membranes are affected to a varying

degree and commonly arise during the prodromal phase. Gross involvement of

respiratory epithelium causes failure of oxygenation, whilst mucosal pathology can

cause painful oral lesions and gastrointestinal/genitourinary haemorrhage.9 10

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Currently the most important considerations in managing TEN are prompt diagnosis,

early disease modification, good supportive care and effective wound management.

Worldwide data suggest that these are best delivered following early transfer to a

Burns service.5

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Over a six month period the Chelsea and Westminster Hospital Burns Service treated

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five consecutive patients with more than 80% total body surface area involvement or

a more than 80% mortality risk, using the severity-of-illness score for toxic epidermal

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necrolysis (SCORTEN). All patient were treated according to the Chelsea and

Westminster Hospital wound management algorithm with excellent outcome and no

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mortalities. The aim of this paper is to propose a generic TEN wound management
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algorithm, aided by a simple grading system for severity of TEN skin lesions.
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PATIENTS
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Five consecutive patients (4x female, 1x male) with a mean age of 50 years (range =
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31 to 61 years) with TEN involving a final mean TBSA of 75% (range =60 to 95%)

were treated at the Chelsea and Westminster Hospital Burns Service (Table 4) (Table
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2). See Figure 1 for the complete management algorithm. In all patients TEN was
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diagnosed by skin biopsy, all medications were stopped and immunosuppression

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commenced – Intravenous immunoglobulin (IVIG) 2g/Kg bolus over first 24 hours

and 1g/Kg twice a day over 48 hours, with intravenous Cyclosporin (IVC) at 2-

5mg/kg once daily (dependent on renal function). As previously discussed by de

Sicca-Chapman and collegues, the Chelsea and Westminster Hospital protocol is that

all patients get granulocyte-cell stimulating factor (G-CSF) unless contraindicated.7

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Four patients with significant mucosal involvement were treated with topical steroids

to prevent synechiae (Table 5) (Table 3): All patients were regularly review by

gastroenterologists, gynaecologists and ophthalmologists. In significant

oropharyngeal involvement nasogastric feeding was instituted throughout with

glucose control on an insulin sliding scale, clinical observation of gastrointestinal

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absorption and review by gatroenterologists. Three out of five patient developed chest

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sepsis, which were treated with culture specific antibiotics.

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Skin dressing management was dependent on the Stage of denudation and skin loss

(Table 6) (Table 4): Stage 0 - Normal skin was protected with emollient; Stage 1 -

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Erythematous skin was then treated with topical steroids. Erythematous skin was then
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treated with topical steroids every 4-6 hours from the time of admission until clinical

resolution (Table 3). All areas where then covered with MepitelTM (Molnlycke Health
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Care, US, LLC, Norcross, GA) and betadine soaked gauze; Stage 2 - Blisters were
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aspirated to prevent extension and the overlying epidermis reapplied and dressed with
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MepitelTM and betadine soaked gauze. The outer gauze dressings were carefully

changed every day to prevent bacterial strike through, leaving the MepitelTM layer
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intact; Only the outer gauze dressings were carefully changed every day to prevent

bacterial strike through, leaving the MepitelTM layer intact and thus not striping the
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reapplied epidermis; Stage 3 - Areas with denuded skin / epidermal loss were covered

with BiobraneTM (Smith & Nephew Healthcare Ltd, Hull, UK), cryopreserved

cadaveric allografts and/or E-Z dermTM (Molnlycke Health Care, US, LLC, Norcross,

GA), a porcine derived xenograft. Pre-fabricated BiobraneTM gloves were used for

hands. Air mattresses were used in patients with involvement of back, buttock and the

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posterior surfaces of the limbs. Thermo regulated environments were used during

dressing changes to prevent hypothermia.

All patients made a timely recovery with a mean stay in the specialist burns unit of

22 days (range = 12 to 32 days) and a mean out-patient follow up of 14.8 month

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(range = 12 to 18 months).

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DISCUSSION

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DIAGNOSIS: Stevens Johnson syndrome and TEN fall within the same spectrum.10,
11, 12 11, 12, 13
Bastuji-Garin et al in 1993 define the diagnostic criteria based on the

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TBSA of epidermal detachment and the morphology of the skin lesions (Table 7)
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(Table 1).2, 11, 13 2, 12, 14
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Chave and colleagues proposed in 2005 that the surface area to be included when
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diagnosing TEN should be limited to those areas that demonstrate epidermal

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detachment: ‘either blistered or denuded, or in danger of this (i.e. Nikolsky positive)

but not that involved with either an erythematous or petechial rash.6 This blistered
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area can be calculated in a similar manner to burn area assessment, by a variety of

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methods and expressed as a percentage of TBSA. This measurement is disease

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defining (over 30% TBSA is TEN).

Whilst it may not be essential for diagnosis, we recommend that the TBSA of skin

affected by early lesions (suggestive TBSA) such as a rash, erythema or petechiae

should be calculated separately from the area clearly blistered or Nikolsky positive

(diagnostic TBSA). This measurement of ‘threatened TBSA’ is useful as an objective

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serial measure of disease progression and when communicating with tertiary referral

centres. Photography as a method of documentation and monitoring of clinical

progression can help.

The main differential diagnoses of TEN include staphylococcal scalded skin

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syndrome, the severe bullous skin disorders including erythema multiforme.12 13 Thus

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skin biopsy is essential in diagnosis of TEN and should be taken across the border of

affected and normal skin. Thus, as previously described, skin biopsy is essential in

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diagnosis of TEN.7

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MANAGEMENT: Although our treatment rationale is systematic and similar to the
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management of severe burns, it must be remembered that the pathological insult
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occurs between viable layers of skin. Burn injuries involve necrosis of the epidermis

(and the dermis to varying degrees) and no benefit is derived from utilising epidermal
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remnants. The epidermal cells in TEN are not directly injured in the
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immunopathogenesis of this inflammatory dermatosis and are initially viable and

potentially salvageable. The epidermis ‘takes’ like epidermal-only split thickness skin
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graft would and therefore not only can this layer be used as a biologically active
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dressing, protecting the dermis (obviating more complex wound management

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strategies,) but it may allow complete early wound healing. We therefore advocate a

strategy that aims to preserve this potentially viable epidermal layer.

Early on in the necrolysis process, petechiae and erythema are suggestive of an

early/mild immunological process. Upper respiratory symptoms (cough, sore throat,

hoarseness) are evident, but the mucosa is intact. As the disease matures, more

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keratinocytes apoptose and the accumulation of inflammatory infiltrate in response, at

the dermo-epidermal junction, produces clinical evidence of blistering.

Desquamation or sloughing of these blisters creates an epidermal/mucosal defect or

ulcer. If the process is left unchecked, the skin separates in larger confluent areas as

cytokine rich fluid weakens cell-cell interactions and the pressure of blister fluid

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cleaves the tissue plains to extend and lift neighbouring epidermis.16 15

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As more cells within the dermo-epidermal junction undergo cell death, the

immunological cytokines drive the pathology exponentially. TNF, Perforin,

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Granzyme B and specifically Fas-ligand, analysed from blister fluid are powerful
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inducers of epidermolysis.14 FAS-ligand activity drives apoptosis through binding

with a cell-surface receptor.15, 16 If the process is left unchecked, the skin separates in
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larger confluent areas as cytokine rich fluid weakens cell-cell interactions and the
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pressure of blister fluid cleaves the tissue plains to extend and lift neighbouring
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epidermis.16
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By employing treatment strategies conventionally used to manage burn injuries, we

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have treated five patients with high predicted mortality or >80% TBSA involvement
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and achieved complete healing within 32 days at the latest.

Increasing age, significant co-morbidity and TBSA correlate with a worse

prognosis.17 16
Additional important negative prognostic factors include persistent

neutropaenia (lasting >5 days) and hypoalbuminaemia (<20g/L). SCORTEN has been

devised as an estimate of mortality risk (Table 2&3).18 17

However, its value in

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prediction of true mortality is controversial and is suggested to be an overestimate of

true mortality.19, 20 18, 19 Traditional validated methods of risk assessment such as the

‘Acute Physiology and Chronic Health Evaluation’ (APACHE) score and Multi

Organ Dysfunction Score (MODS) may prove more useful in series collected

prospectively.21, 22 20, 21
Studies have shown that early transport to a Burns Service

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significantly reduces the mortality rate.23, 24, 25 22, 23, 24

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A multidisciplinary approach draws on the specialist skills of plastic surgeons,

dermatologists, intensivists, ophthalmologists, gynaecologists, gastroenterologists,

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paediatricians, haematologists, nurses, physiotherapists, nutritionists, pharmacists,
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occupational therapists, pain specialists and others. Studies have shown that early

transport to a Burns Service significantly reduces the mortality rate.23, 24, 25

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The evidence for a predominant immunopathogenic driver to the TEN process is

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overwhelming.26, 20, 27 7, 19, 25

The intensive care and medical management is outside

the scope of this paper, but should proceed along normal resuscitation protocols with
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special provision of a thermoregulated environment to reduce insensible losses. The

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Chelsea and Westminster Hospital medical treatment regimen has previously been
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published and will not be discussed here, needless to say we believe that early

immunosupression and cessation of all non-vital medication arrests evolution of the

disease process and reduces the necessity for ancillary management strategies such as

complex wound care and total parenteral nutrition and/or ventilatory support.26 7 It is

prudent to be wary of medications with long half-lives, as they may have persistent

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action. New medication should be introduced/re-introduced with extreme caution and

by the patient’s clinical course.

The evidence for a predominant immunopathogenic driver to the TEN process is

overwhelming. It is conceded that there is insufficient evidence to ratify any single

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specific immunosuppressive treatment, but the therapeutic necessity to give systemic

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immunosupression is compelling, particularly following our experience and that from

other centres.20, 27

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We advocate use of steroid preparation creams and ointments as part of this

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immunosupressive regimen (Table 5 & 6) (Table 3 & 4). Concerns regarding the
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inadvertent use of potentially absorbable topical steroid preparations adjunctively
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should be allayed. From our experience outside TEN, steroid levels are unlikely to

achieve the systemic levels of intravenous administration and have been widely and
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successfully used in the treatment of endogenous and exogenous inflammatory

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dermatoses.28 26
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SKIN LESIONS: We present our thoughts on the armamentarium available to the

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plastic surgeon in the management of the epithelial lesions found in TENS. This is by
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no means a strict decision tree, but a general algorithm when faced with

heterogeneous cases. In order to simplify the treatment algorithm in respect to disease

progression, skin pathology is classified in stages 0 to 3 (Table 6) (Table 4):

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Stage 0: The application of emollient and a protective dressing to unaffected areas is

advocated. An alternative to occlusive dressings is barrier nursing under a cradle in a

thermo-regulated environment.

Stage 1: These areas are at greatest risk of disease progression and should be

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protected against bacterial invasion. Avoiding shear force during patient transfer is

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therefore imperative. The steroid preparations are used alternately with 50:50 liquid /

white soft paraffin emollient and the latter is applied as often as necessary to keep the

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skin moist. For example, TrimovateTM (Glaxo Smith Kline, London, UK) is a topical

steroid cream that also contains Nystatin and a Tetracycline, useful in intertriginous

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sites to prevent candidal and mycobacterial superinfection. When exposed, skin
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should be assessed regarding the need for emollient every 2 hours. Steroid should be
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reapplied every 4-6 hours where practicable.

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Stage 2: Inflammatory exudate that separates epidermis from dermis forms blisters
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(<10mm) and bullae (collections >10mm diameter). Minor blisters can be aspirated to

prevent physical extension of dermo-epidermal separation as the intercellular

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junctions weaken. Aspiration of cytokines theoretically reduces the immunological

drive.15, 22 27, 21 Nikolsky positive skin is therefore included in this group.

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Laying back separated epidermis can prevent dermal death through exposure

desiccation and provides a sterile biological dressing, acting as a barrier to infection.

This can either be left to adhere through exposure nursing, or held in place and

protected from shear forces using Mepitel™ held in place with betadine soaked

gauze. To prevent further sheering during dressing changes, only the superficial layer

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is changed, leaving MepitelTM in place. Daily inspection of wounds should be

instigated for serial aspiration of re-accumulated blister fluid, close monitoring of

disease progression and re-application of sheared epidermis. Daily inspection of

wounds through the transparent/holed Mepitel layer should be instigated for serial

aspiration of re-accumulated blister fluid, close monitoring of disease progression and

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re-application of sheared epidermis. If the epidermis remains intact, dressing changes

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can be ward based. This rationale is more practical, cheaper and logistically easier

than multiple general anaesthetics to apply allograft/xenograft to exposed dermis.

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Stage 3: A few studies in TEN patients have shown that collagen dressings such as

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Biobrane™ decreases fluid loss by 90% compared with open wounds.29, 30 28, 29 This
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allows rapid re-epithelialisation through the application of a once only dressing.31, 32,
33 30, 31, 32
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To maximise adherence, it should be applied within 72h from the onset of

blistering to a clean wound. Biobrane™ can be left for 24 to 48 hours before

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reassessment, at which point the clinician must exclude fluid accumulation and non-
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adherence clinically. This management strategy should only be employed when the

whole area underneath is affected and does not need serial clinical examination. It is
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easily applied in large sheets to the back as this provides a single non-shear dressing,
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or on complex surfaces such as the hands and forearms glove forms exist for ease of
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application. Its obvious potential disadvantage includes sepsis. Betadine soaked gauze

is then applied prophylactically and as a secondary dressing that prevents shear.

Cadaveric allograft or porcine xenograft are well described in the management of

burns.34, 35 33, 34
We found their use just as valuable during the early-uncontrolled

phase of the TEN disease process, when disease progression is on-going and

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sequential areas of epidermis are lost despite immunosuppression. It is only a useful

dressing if it adheres (prevented by exudate, bacterial colonisation and physical shear

forces). This modality is inherently more expensive and requires general anaesthetic

procedures for re-application but it reduces the inflammatory response and the

physiological burden of the injury.35 34

Autologous cultured cells used in the

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management of burns had no role in our management algorithm as the philosophy of

epidermal salvage was paramount. Another alternative, E-Z dermTM is a porcine

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derived xenograft in which the collagen has been chemically cross-linked with an

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aldehyde to provide strength, durability and convenient storage at room temperature.

E-Z derm is a cheap dressing and we believe effective when skin injury is superficial

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and the dermis only needs protection for a few days to allow healing.
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Our preference is to use BiobraneTM as a first line dressing particularly on the hands,
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forearms and back for ease of application, reserving allograft for the face and EZ
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dermTM on large areas. Occasionally, E-Z derm, BiobraneTM and allograft fail to stick
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to the exposed dermis and in these cases mepitel in conjuction with betadine-soaked

gauze is used.
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Metachronous lesions are lesions of varying severity co-existing at similar times,

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whilst synchronous lesions relate to islands of similarly involved skin separated by

normal skin. If metachronous lesions exist in severe TEN it is often difficult to apply

differing management plans to the same area. The clinician must rationalise the

situation by deciding on the most suitable modality for that limb or surface and utilise

dressings appropriately (e.g. application of steroid preparations to normal skin

intervening two lesions or de-roofing blisters for the application of Biobrane™.

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MUCOSAL LESIONS: Mucosal involvement is a spectrum also ranging from

erythema and oedema to blistering, sloughing and ulceration. Affected mucosa

includes the gastrointestinal, genitourinary, respiratory tracts as well as ocular

surfaces.

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Mucosal ulceration may cause synechiae to form between adjacent epithelial defects.

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As these mature they can constrict to affect function. Ocular involvement occurs in

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around 85% of patients and can result in keratitis and corneal erosions. This can vary

from hyperaemia to extensive pseudo-membrane formation. Synechiae commonly

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form between the eyelids and conjunctiva. Genitourinary involvement can be painful
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and can result in urinary retention, phimosis in uncircumcised men and vaginal

synechiae.36 35
Prophylaxis with conjunctival irrigation and vaginal pessaries and
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creams containing steroids are examples of methods that can be effective if performed
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early and frequently in aggressive disease (Table 3). These methods should be
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employed under careful specialist supervision.

Most commonly the mouth is affected by painful ulceration and can be treated with
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topical lignocaine gel or cocaine mouthwash, whilst regular CorsodylTM

(Chlorhexidine by Glaxo Smith Kline, London, UK) or DifflamTM (benzydamine

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hydrochloride by Meda Pharmaceuticals, Glasgow, UK) mouthwash can prevent

against bacterial invasion. Gastrointestinal haemorrhage may result from intestinal

inflammation. Long term, stenosis occurs if the gastrointestinal tract becomes

circumferentially scarred during healing.

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Mucosal sloughing into the respiratory tract can lead to plugging, respiratory

embarrassment and ultimately oxygenation failure. This must be investigated by

bronchoscopy and managed by a skilled team of physicians and physiotherapists.

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CONCLUSIONS

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The TEN disease process is a multidisciplinary management challenge. Treatment of

synchronous and metachronous lesions over an extensive surface area is difficult and

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the process can evolve rapidly. We believe that the regenerative potential of the skin

is determined by the rapidity of disease abatement and a structured approach to

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wound care preventing the life-threatening sequelae of TEN.
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CONFLICT OF INTEREST STATEMENT

None

FUNDING

None

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DECLARATION

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Please note that the principles outlined in the Declaration of Helsinki have been

followed and conformed with.

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FIGURES

Figure 1. Summary management algorithm

DIAGNOSIS: TBSA >30% & skin biopsy

STOP ALL MEDICATIONS
Start IV immunoglobulin +/- IV cyclosporin + get
granulocyte-cell stimulating factor and analgesia.7

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Transfer to HDU

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INVESTIGATIONS OBSERVATIONS WOUND CARE
Including FBC and Urea and Chart progression of TBSA by stage of skin pathology
Electrolytes, Glucose, CRP
Bicarbonate and Albumin

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Monitor for neutropenia and Speak to local Burns Service early STAGE 0:
Renal Failure in case of deterioration 50:50 white soft paraffin/ liquid paraffin as

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emollient



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Involvement of Dermatology, Intensive care, Ophthalmology STAGE 1:
and Plastic Surgery. Inform Gynaecology, Gastroenterology and Apply topical steroid preparations suitable to the
Paediatric Consultants as indicated. Liaison with Dietician, Pharmacist site of application alternating with 50:50 white
and Haematologist early. soft paraffin/ liquid paraffin as emollient.
M

STAGE 2:
Aspirate accumulated fluid from blisters and
bullae. Re-apply viable epidermis. Protect with
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Mepitel™ and antimicrobial soaked gauze

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STAGE 3:
Consider Biobrane™ , Allograft or Xenograft with
Mepitel TM or Jelonet™ and betadine soaked
gauze
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TBSA: total body surface area, IV: intravenous, HDU: High Dependency Unit, FBC:
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full blood count, CRP: C-reactive protein,

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Figure 2. Stages of TEN skin, A = Stage 1. Early lesions such as poorly defined

erythematous macules with darker purpuric rash; B = Stage 2. Blister and bullae

formation by accumulation of inflammatory exudate between ‘loosened’ dermo-

epidermal junction; Stage 3. Skin denuded of epidermis and needs definitive surgical

management.

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Table 1. List of frequent drug-induced causes of TEN

Medication group Medications

Sulphonamides, Trimethoprim, Chloramphenicol,

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Antibiotics Macrolides (erythromycin), Penicillins, Quinolones

(ciprofloxacin, trovafloxacin)

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Phenylbutazone, Oxybutazone, Oxicams (piroxicam,
Non-steroidal anti-inflammatories
tenoxicam), Ibuprofen, Indomethacin

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Phenobarbitone, Phenytoin, Carbamazepine, Sodium
Anti-epileptic medication
valproate, Lamotrigine

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AN
Miscellaneous Allopurinol, Corticosteroids
M

Table 2. SCORTEN Diagnostic Criteria

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Age >40
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Heart Rate >120 per minute

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Blood Glucose >2.52g/l

Involved TBSA >10%

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Blood Urea Nitrogen >10mMol/L

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Serum Bicarbonate <20mMol/L

Cancer/Haemopathy ++

Each criterion achieves one point

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Table 3. SCORTEN score-related percentage mortality

Score ICU Mortality

0-1 3.2%

2 12.1%

3 35.3%

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4 58.3%

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5-7 90%

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Clinical classification of Epidermolysis Spectrum19

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Table 7. Table 1 AN
Erythema Multiforme Stevens Johnson Syndrome SJS/TEN Cross over TEN

% TBSA <10 <10 10-30 >30

M

TBSA: total body surface area, SJS: Stevens Johnson Syndrome

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Table 4. Table 2. Patient demographics

Patient Age Comorbidities Sex TBSA Medication IVIG IVC G-CSF Skin Mucosal SCORTEN / Complica Days in Follow

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/ initial (days) (days) (days) stage involvement Apache II / tions hospital up

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presentation MOD / (months)

predicted

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mortality

1 61 Epilepsy, / F 95% Phenytoin, 30 17 2 3 Oral, eyes 5 / / 14 / 9 - 32 24

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phenobarbitone,
Addisonian- / 90%

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teichoplanin
like crisis

2 47 Arthritis, M 50% Sulfasalazine 9 9 3 1&2 5 / 11 / 6 / Chest 14 24

M
hypertensio Oral 90% sepsis

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n, gout /

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rash

3 50 Single F 60% Teicoplanin, 27 7 2 2 Eyes, 5 / 13 / 7 / Chest 32 18

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gentamycin,
kidney / (renal genitalia 90% sepsis
acyclovir
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rash impai
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rment

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4 35 Epilepsy, F 95% carbamazapine 3 10 1 1&2 Oral, 2/6/5/ Chest 12 16

psoriasis / genitalia 12.1% sepsis

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rash

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5 55 SLE F 80% Prednisolone, 11 11 - 1, 2 & Oral, eyes, 2 / 13 / 0 / - 20 12
hydroxychloroq
3 genitalia 12.1

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uine

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TBSA: total body surface area, IVIG: intravenous immunoglobulin, IVC: intravenous cyclosporine, G-CSF: Granulocyte-Cell Stimulating

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Factor, SCORTEN: severity-of-illness SCORe for Toxic Epidermal Necrolysis, Apache II: Acute Physiology and Chronic Health Evaluation

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score, MOD: Multi Organ Dysfunction score, M: male, F: female.

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Table 5. Table 3 Summary of topical steroid management

Body part Management

Eumovate™ ointment (Glaxo Smith Kline, London, UK)

Face - Clobetasone butyrate 0.05% with white soft paraffin and liquid

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paraffin

CorsodylTM mouthwash (Glaxo Smith Kline, London, UK)

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- Chlorhexidine mouthwash
Mouth

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DifflamTM (Meda Pharmaceuticals, Glasgow, UK)

- Benzydamine hydrochloride

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Eyes Dexamethasone / lacrilube / viscotears eye drops
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Dermovate™ cream (Glaxo Smith Kline, London, UK)
Body
- Clobetasone propionate 0.05%
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Trimovate™ cream (Glaxo Smith Kline, London, UK)

- Clobetasone butyrate 0.05% with glycerol, white soft paraffin,

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Groin and perineum

an antibacterial agent (calcium oxytetracycline 3%) and
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antifungal agent (nystatin 100.000 unit/gr)

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Table 6. Table 4 Summary of cutaneous management by wound stage

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TEN skin stage Clinical signs Management

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50:50 white soft paraffin/ liquid paraffin as

Stage 0 Normal Skin
emollient

Poorly defined
Steroid preparation appropriate to site of
erythematous
Stage 1 application alternating with 50:50 white soft
macules with darker
paraffin/ liquid paraffin as emollient
purpuric rash

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Flaccid blisters and
Aspirate fluid in blisters and bullae
bullae with
Stage 2 Re-apply viable epidermis
surrounding
Mepitel™ and antimicrobial soaked gauze
oedema/rash/macules

Biobrane™, E-Z dermTM, Allograft, Xenograft™

Stage 3 Denuded skin

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or MepitelTM and betadine soaked gauze

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AN
M
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AC

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AN
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