► What is T.E.N.?

► Causes

► Clinical Features

► Differential Diagnosis

► Investigations

► Management

► Complications

► Prognosis

► Definition:► Toxic = “pertaining to, due to or the nature of a poison or toxin, manifesting the symptoms of severe infection”► Epidermal = “pertaining to or resembling the epidermis”► Necrolysis = “separation or exfoliation of tissue due to necrosis”

► Rare► Life threatening► Drug induced

► adverse drug reaction: 5-15% drug treatments► cutaneous reactions: most common

► morbilliform type: most common► symmetrical, erythematous rash, macules & papules, lasts few days

► can proceed to serious cutaneous reactions:► serum sickness► hypersensitivity syndrome► T.E.N.

► Mucocutaneous reaction► widespread erythema► necrosis► bullous detachment of epidermis & mucous membranes

► GI haemorrhage► respiratory failure► genitourinary complications

► Incidence: 0.4-1.2 cases/million population/year► Affects all ages► Mortality rate: 30-40%

► Adverse drug reaction► over 100 drugs implicated

Commonest Causative DrugsSulphonamide antibiotics

Anticonvulsants

NSAIDs

Allopurinol

Corticosteroids

Newest Causative DrugsNevirapine (antiretroviral)

Lamatrogine

► Other causes:► immunisations; bone marrow transplants; solid organ transplants

► Prodromal phase: 1 – 14 days► flu-like symptoms

► Inflammation:► eyelids► conjunctiva

► Tenderness:► oral mucosa► general cutaneous

► Generalised macular erythma:► progresses to flaccid blisters and bullae► join to form large bullae► large areas of epidermis are “sloughed off”

► Mucous membranes often involved► usually 1-3 days earlier then skin lesions► eyes; oropharynx; respiratory tract; GI tract; genital tract; anus

Rapid progression over days. 10 – 100% of body’s surface area involved.

DiscomfortPain

FeverSore throat

CoughMalaise

Differential Diagnosis of T.E.N.

Burns

Conjunctivitis

Ulcerative keratitis

Staphylococal Scalded Skin Syndrome

Stevens-Johnson Syndrome (S.J.S.)

Toxic Shock Syndrome

Exfoliative dermatitis

Erythema multiforme

Pemphigus

S.J.S. and T.E.N. Are very similar in cause, severity, clinical features and variability. They only really differ in the extend of skin detachment and mortality – both being larger in T.E.N.

► MICROBIOLOGY► blood culture► MSU► swabs; including MRSA screening swabs

► GENERAL► FBC► ESR► U&E and creatinine► LFTs► Albumin► Glucose► Calcium► CRP► Urine dipstick (protein & blood)

► IF INDICATED► coagulation studies► CXR

► MAINLY SUPPORTIVE

► discontinue causative drug

► burns unit► skin care; protect skin from infection► monitor fluid and electrolyte balance► nutritional support► analgesics (may need oral/iv morphine)► eye care; lubrication with chloramphenicol

► SCORTEN assessment

► Ocular► conjunctivitis► vesiculation► corneal ulceration/scarring► uveitis► synchiae► pseudomembrane formation► blindness

► Cutaneous► scarring► hypopigmentation► hyperpigmentation

► Mucous membranes► scarring

► oesophageal, bronchial, anal and vaginal strictures

► SCORTEN assessment; first 24 hours after admissionFactors associated with poor prognosis

Age > 40 years

Heart rate > 120 bpm

Malignancy

Day 1 blistering affecting > 10% body surface area

Urea > 10 mmol/l

Bicarbonate < 20 mmol/l

Glucose > 14 mmol/l

SCORTEN score Mortality Rate0-1 3%

2 12%

3 35%

4 58%

>5 90%

Overall mortalityaround

30%

► Bastuji-Garin, S., Fouchard, N., Bertocchi, M., Roujeau, J-C., Revuz, J. & Wolkenstein, P. (2000). SCORTEN: A severity-of-illness scor for toxic epidermal necrolysis, J. Invest. Dermatol., 115(2): 149-153.

► Dictionary of Cell and Molecular Biology

► Devkaran, A. & Gupta, S. (2008) Toxic Epidermal Necrolysis, Student BMJ; 16:168-170.

► www.wsh.nhs.uk/dermatology

► www.medscape.com

► www.adr.org.uk

► www.pathconsultddx.com