Acute generalized exanthematous pustulosis (AGEP)
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Acute generalized exanthematous pustulosis (AGEP)

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Acute generalized exanthematous pustulosis

Acute generalized exanthematous pustulosis

Presented by Planee Vatanasurkitt, MD.

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Acute generalized exanthematous pustulosis (AGEP) Acute generalized exanthematous pustulosis (AGEP) Presentation Transcript

  • Planee Vatanasurkitt,MD
  •  Clinical feature Differential diagnosis histopathology in AGEP Etiology EuroSCAR study Pathophysiology Investigation treatment
  •  1-5 per million/year
  •  Widespread erythema with hundreds of small, flaccid, confl uent, non-follicular pustules, especially along the groins and on the flexor surfaces of the legs.
  •  (1) numerous, small non-follicular, intraepidermal or subcorneal pustules (< 5 mm) on an erythematous background, (2) typical histopathological changes, (3) fever (> 38 °C), (4) blood neutrophil counts > 7x106 cell/l (5) an acute evolution with spontaneous resolution of pustules in less than 15 days Eur J Dermato 2010;20 4 :425-3
  • Pustular psoriasis Subcorneal pustular Pustular vasculitis dermatosis•History of Psorisis •Larger flaccid blister •Bullous and or•Longer duration •Less acute clinical pustular lesion in course purpura of leucocytoclastic vasculitis •Localized mainly on dorsum of handsSidoroff A et al. Acute generalized exanthematous pustulosis(AGEP)
  •  spongiform subcorneal and/or intraepidermal pustules, marked papillary edema polymorphous perivascular infiltrates with neutrophils and exocytosis of some eosinophils. Eur J Dermato 2010;20 4 :425-3
  • Two subcorneal, intraepidermal pustules withpapillary edemaand a mixed inflammatory infiltrate of mainlyneutrophils and some eosinophils. Eur J Dermato 2010;20 4 :425-3
  • J Clin Invest. 2001; 107(11):1433–1441
  • Healthy skin AGEP J Clin Invest. 2001; 107(11):1433– 1441
  • Healthy skin AGEP J Clin Invest. 2001; 107(11):1433– 1441
  • Healthy skin AGEP J Clin Invest. 2001; 107(11):1433– 1441
  • J Clin Invest. 2001; 107(11):1433–1441
  • J Clin Invest. 2001; 107(11):1433–1441
  • J Clin Invest. 2001; 107(11):1433–1441
  •  90% are drug Other : acute viral infection , entero virus coxakie virus, echo virus, CMV, EBV, hepatitis virus,parvo virus, E.coli,Chalmydia pneumoniae,Mycoplasma pneumoniae Spider bite,mercury,chemotherapy,
  •  Viral infection and pneumococcal vaccination are most common trigger in pediatric population
  •  In one study , human leukocyte antigen haplotypes B51 , DR 11, and DQ3 were more common in AGEP patients than in the general population However, further studies are required to elucidate the genetic background of AGEP. S. Halevy. AGEP.Cur Opion Allergy and Clinical Im 2009, 9
  • Highly suspected drugOther suspected drug
  •  Not yet understood Suggest genetic hypersensitivity or type 4 hypersensitivity Can be divided to 3 phase Eur J Dermato 2010;20 4 :425-3
  • Eur J Dermato 2010;20 4 :425-3
  •  IL-17 secreted by CD4+ and CD8+ CD45RO induced cytokine and protaglandins from stromal cell and macrophage And increase CXCL8 and IL-6 from keratinocytes Curr Opin Allergy Clin Immunol 2:325–3
  • trigger Drug,infectioncell T cell/neutrophilcytokine CXCL8patho Sterile pustule filled with neutrophil
  •  Patch test In vitro test: lymphocyte transformation test
  •  Discontinue culprit drug Treat infection if indicate Systemic steroid are not necessary Symptomatically systemic antipyretics can be given if not suspected as causative drug for the disease. Sidoroff A et al. Acute generalized exanthematous pustulos Clinical reaction pattern , J Cutan Pathol 2001: 28: 113–11
  •  AGEP had typical clinical feature Etiology : drug, infection In children infection in predominant Pathophysiology : unknown but may be Type 4 hypersensitivity involve T cell/neutrophil, CXCL8 Treatment : discontinued causative drug, treat infection