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Toxic Epidermal Necrolysis
Kristine Scruggs, MD
AM Report
July 28, 2009
Definition
 SJS/TEN:
 Lesions: Small blisters on dusky purpuric macules or
atypical targets
 Mucosal involvement common...
Presentation
 Fever (often >39) and flu-like illness 1-3 days
before mucocutaneous lesions appear
 Confluent erythema
 ...
Epidemiology
 2-7/million people/year
 SJS: age 25-47, TEN: age
46-63
 Women: >60%
 Poor prognosis:
 Intestinal/Pulmo...
Pathogenesis
 Secondary to cytotoxicity and delayed hypersensitivity reaction
to the offending agent.
 Antigen is either...
Etiologies
 Medications (Odds Ratio for exposure in
hospitalized pts):
 Sulfonamide antibiotics (172)
 Allopurinol (52)...
Differential Diagnosis for Vesicular or
Bullous Rash
Bullous
Pemphigoid
Often affects
the elderly
Dermatitis Herpetiformis...
Differential Diagnosis, cont.
Linear IgA Disease
Itchy, ring-shaped, no internal disease
Contact Dermatitis
Varicella/Zost...
Differential Diagnosis, cont.
 Erythema Multiforme
 Staphylococcal Scalded Skin Syndrome
 Bullous Impetigo
 Toxic Shoc...
Treatment
 Early diagnosis - biopsy
 Immediate discontinuation of offending agent
 Supportive care – pay close attentio...
Prognosis
Independent Prognosis Factors Weight
Age >/= 40 years 1
Malignancy Yes 1
BSA Detached >/= 10% 1
Tachycardia >/= ...
Resources:
 Cooper, et al. The Washington Manual of Medical
Therapeutics, 32nd
Edition. 2007.
 High, et al. Stevens-John...
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Transcript of "7.28.09 scruggs ten sjs"

  1. 1. Toxic Epidermal Necrolysis Kristine Scruggs, MD AM Report July 28, 2009
  2. 2. Definition  SJS/TEN:  Lesions: Small blisters on dusky purpuric macules or atypical targets  Mucosal involvement common  Prodrome of fever and malaise common  Stevens-Johnson Syndrome:  Rare areas of confluence.  Detachment </= 10% BSA  Toxic Epidermal Necrolysis:  Confluent erythema is common.  Outer layer of epidermis separates easily from basal layer with lateral pressure.  Large sheet of necrotic epidermis often present.  >30% BSA involved.
  3. 3. Presentation  Fever (often >39) and flu-like illness 1-3 days before mucocutaneous lesions appear  Confluent erythema  Facial edema or central facial involvement  Lesions are painful  Palpable purpura  Skin necrosis, blisters and/or epidermal detachment  Mucous membrane erosions/crusting, sore throat  Visual Impairment (secondary to ocular involvement)  Rash 1-3 weeks after exposure, or days after 2nd exposure
  4. 4. Epidemiology  2-7/million people/year  SJS: age 25-47, TEN: age 46-63  Women: >60%  Poor prognosis:  Intestinal/Pulmonary involvement  Greater extent of detachment  Older age  Mortality:  SJS: 5%  TEN: 30%  Risk Factors:  HIV infection  Genetic factors  Certain HLA types  “Slow acetylators”  Polymorphisms in IL4 receptor gene  Concomitant viral infections  Underlying immunologic diseases  Physical factors  UV light, radiation therapy  Malignancy  Higher doses of known offenders
  5. 5. Pathogenesis  Secondary to cytotoxicity and delayed hypersensitivity reaction to the offending agent.  Antigen is either the implicated drug or a metabolite.  Histopathology:  Granulysin (cytolytic protein produced by cytotoxic T cells and NK cells)  Expression of HLA-DR and intracellular adhesion molecule (ICAM)-1 by  Keratinocytes  CD4 cells (in dermis)  CD8 T cells (in epidermis)  Apoptosis of keratinocytes facilitated by  TNF-alpha, perforin and granzyme secretion  fas-ligand expression (cell death receptor) Subepidermal split with cell-poor bullous. Epidermis shows full thickness necrosis.
  6. 6. Etiologies  Medications (Odds Ratio for exposure in hospitalized pts):  Sulfonamide antibiotics (172)  Allopurinol (52)  Amine antiepileptics  Phenytoin (53)  Carbamazepine (90)  Lamotrigine  NSAIDs (72)  Infections (e.g. Mycoplasma pneumonia)  Other: Vaccinations, Systemic diseases, Chemical exposure, Herbal medicines, Foods
  7. 7. Differential Diagnosis for Vesicular or Bullous Rash Bullous Pemphigoid Often affects the elderly Dermatitis Herpetiformis Associated with gluten intolerance Cicatricial Pemphigoid Mucosal involvement, sometimes cutaneous Pemphigus Affects middle-aged or elderly
  8. 8. Differential Diagnosis, cont. Linear IgA Disease Itchy, ring-shaped, no internal disease Contact Dermatitis Varicella/Zoster Virus Herpes Simplex Virus Hand-Foot-Mouth Disease (Enteroviruses)
  9. 9. Differential Diagnosis, cont.  Erythema Multiforme  Staphylococcal Scalded Skin Syndrome  Bullous Impetigo  Toxic Shock Syndrome  Paraneoplastic Pemphigus  Cutaneous emboli  Diabetic Bullae  Porphyria Cutanea Tarda  Porphyria Variegata  Pseudoporphyria  Bullous dermatosis of Hemodialysis  Coma Bulloae  Epidermolysis Bullosa Acquisita
  10. 10. Treatment  Early diagnosis - biopsy  Immediate discontinuation of offending agent  Supportive care – pay close attention to ocular complications  IV hydration (e.g. Parkland formula)  Antihistamines  Analgesics  Local v. systemic corticosteroids  Think about nursing requirements!  Possible treatment in burn unit, wound care  IVIg?
  11. 11. Prognosis Independent Prognosis Factors Weight Age >/= 40 years 1 Malignancy Yes 1 BSA Detached >/= 10% 1 Tachycardia >/= 120/min 1 Serum urea >10 mmol/l 1 Serum glucose >14 mmol/l 1 Serum bicarbonate <20 mmol/l 1 SCORTEN # 7
  12. 12. Resources:  Cooper, et al. The Washington Manual of Medical Therapeutics, 32nd Edition. 2007.  High, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis: Management, prognosis, and long-term sequelae. Up To Date. 2009.  Kasper, et al. Harrison’s Principles of Internal Medicine, 16th Edition. 2005.  Nirken, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis: Clinical manifestations, pathogenesis, and diagnosis. Up To Date. 2009.
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