Lyell's syndrome or Toxic epidermal necrolysis

Lyell's syndrome or Toxic epidermal necrolysis

1. Name : Francis Sharon Jessy Group : 12 Topic : Lyell’s Syndrome Teacher: Pazylova Baktigul Osh State University - Medical Faculty

2. DEFINITION Definition: Toxic epidermal necrolysis  ( TEN ), also known as  Lyell's syndrome , is a rare, life-threatening  skin  condition that is usually caused by a reaction to drugs .  The disease causes the top layer of skin (the  epidermis ) to detach from the lower layers of the skin (the dermis), all over the body, leaving the body susceptible to severe infection. 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

3. Mucocutaneous reaction widespread erythema necrosis bullous detachment of epidermis & mucous membranes GI haemorrhage respiratory failure genitourinary complications

4. Causes Adverse drug reaction over 100 drugs implicated Commonest Causative Drugs Sulphonamide antibiotics Anticonvulsants NSAIDs Allopurinol Corticosteroids Newest Causative Drugs Nevirapine (antiretroviral) Lamatrogine Other causes: immunisations; bone marrow transplants; solid organ transplants

5. Clinical Features 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 . Discomfort Pain Fever Sore throat Cough Malaise

6. Differential Diagnosis 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.

7. Investigations 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

8. Management 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

9. Complications Ocular conjunctivitis vesiculation corneal ulceration/scarring uveitis synchiae pseudomembrane formation blindness Cutaneous scarring hypopigmentation hyperpigmentation Mucous membranes scarring oesophageal, bronchial, anal and vaginal strictures

10. TREATMENT  Intravenous immunoglobulin (IVIG ) Physical examination   corticosteroids cyclosporin cyclophosphamide plasmapheresis

11. Prognosis SCORTEN assessment; first 24 hours after admission Factors 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 Rate 0-1 3% 2 12% 3 35% 4 58% >5 90% Overall mortality around 30%