acute generalized exanthematous pustulosis (agep)
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DESCRIPTION
Acute generalized exanthematous pustulosisPresented by Planee Vatanasurkitt, MD.TRANSCRIPT
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Acute generalized exanthematous pustolosis
Planee Vatanasurkitt,MD
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Clinical feature Differential diagnosis histopathology in AGEP Etiology EuroSCAR study Pathophysiology Investigation treatment
outlines
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1-5 per million/year
incidence
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Widespread erythema with hundreds of small, flaccid, confluent, non-follicular pustules, especially along the groins and on the flexor surfaces of the legs.
Clinical feature
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(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
Clinical feature
Eur J Dermato 2010;20 (4):425-33
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Pustular psoriasis Subcorneal pustular dermatosis
Pustular vasculitis
•History of Psorisis•Longer duration
•Larger flaccid blister•Less acute clinical course
•Bullous and or pustular lesion in purpura of leucocytoclastic vasculitis•Localized mainly on dorsum of hands
Differential diagnosis
Sidoroff A et al. Acute generalized exanthematous pustulosis (AGEP) Clinical reaction pattern , J Cutan Pathol 2001: 28: 113–119
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Pustular psoriasis
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Subcorneal pustular dermatosis
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Pustular vasculitis
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AGEP
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spongiform subcorneal and/or intraepidermal pustules,
marked papillary edema polymorphous perivascular infiltrates with
neutrophils and exocytosis of some eosinophils.
Typical histopathology
Eur J Dermato 2010;20 (4):425-33
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Two subcorneal, intraepidermal pustules with papillary edema and a mixed inflammatory infiltrate of mainly neutrophils and some eosinophils.
Eur J Dermato 2010;20 (4):425-33
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J Clin Invest. 2001; 107(11):1433–1441
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J Clin Invest. 2001; 107(11):1433–1441
Healthy skin AGEP
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J Clin Invest. 2001; 107(11):1433–1441
Healthy skin AGEP
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J Clin Invest. 2001; 107(11):1433–1441
Healthy skin AGEP
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Patch test positive lesion in AGEP
J Clin Invest. 2001; 107(11):1433–1441
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Patch test positive lesion in AGEP
J Clin Invest. 2001; 107(11):1433–1441
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Patch test positive lesion in AGEP
J Clin Invest. 2001; 107(11):1433–1441
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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,
etiology
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Viral infection and pneumococcal vaccination are most common trigger in pediatric population
etiology
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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.
Genetic predisposing
S. Halevy. AGEP.Cur Opion Allergy and Clinical Im 2009, 9:322 – 328
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drug
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EuroSCAR study
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EuroSCAR study
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EuroSCAR study
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Highly suspected drug
Other suspected drug
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Not yet understood Suggest genetic hypersensitivity or type 4
hypersensitivity Can be divided to 3 phase
Pathophysiology
Eur J Dermato 2010;20 (4):425-33
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Eur J Dermato 2010;20 (4):425-33
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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
T-cell orchestration of neutrophil-mediated inflammatory processes via interleukin-17
Curr Opin Allergy Clin Immunol 2:325–331
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trigger Drug,infection
cell T cell/neutrophil
cytokine CXCL8
patho Sterile pustule filled with neutrophil
My conclusion in AGEP
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Patch test In vitro test: lymphocyte transformation test
investigation
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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.
treatment
Sidoroff A et al. Acute generalized exanthematous pustulosis (AGEP) Clinical reaction pattern , J Cutan Pathol 2001: 28: 113–119
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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
My conclusion
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Thank you