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Similar to Lyell's syndrome or Toxic epidermal necrolysis
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Lyell's syndrome or Toxic epidermal necrolysis
- 1. Name : Francis Sharon Jessy Group : 12 Topic : Lyell’s Syndrome Teacher: Pazylova Baktigul
- 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. ► 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. ► 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 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. ► 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. ► 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. ► 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. ► 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%