acute generalized exanthematous pustulosis (agep)

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Acute generalized exanthematous pustolosis Planee Vatanasurkitt,MD

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Acute generalized exanthematous pustulosisPresented by Planee Vatanasurkitt, MD.

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Page 1: Acute generalized exanthematous pustulosis (AGEP)

Acute generalized exanthematous pustolosis

Planee Vatanasurkitt,MD

Page 2: Acute generalized exanthematous pustulosis (AGEP)

Clinical feature Differential diagnosis histopathology in AGEP Etiology EuroSCAR study Pathophysiology Investigation treatment

outlines

Page 3: Acute generalized exanthematous pustulosis (AGEP)

1-5 per million/year

incidence

Page 4: Acute generalized exanthematous pustulosis (AGEP)

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

Page 5: Acute generalized exanthematous pustulosis (AGEP)

(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

Page 6: Acute generalized exanthematous pustulosis (AGEP)

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

Page 7: Acute generalized exanthematous pustulosis (AGEP)

Pustular psoriasis

Page 8: Acute generalized exanthematous pustulosis (AGEP)

Subcorneal pustular dermatosis

Page 9: Acute generalized exanthematous pustulosis (AGEP)

Pustular vasculitis

Page 10: Acute generalized exanthematous pustulosis (AGEP)

AGEP

Page 11: Acute generalized exanthematous pustulosis (AGEP)

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

Page 12: Acute generalized exanthematous pustulosis (AGEP)

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

Page 13: Acute generalized exanthematous pustulosis (AGEP)

J Clin Invest. 2001; 107(11):1433–1441

Page 14: Acute generalized exanthematous pustulosis (AGEP)

J Clin Invest. 2001; 107(11):1433–1441

Healthy skin AGEP

Page 15: Acute generalized exanthematous pustulosis (AGEP)

J Clin Invest. 2001; 107(11):1433–1441

Healthy skin AGEP

Page 16: Acute generalized exanthematous pustulosis (AGEP)

J Clin Invest. 2001; 107(11):1433–1441

Healthy skin AGEP

Page 17: Acute generalized exanthematous pustulosis (AGEP)

Patch test positive lesion in AGEP

J Clin Invest. 2001; 107(11):1433–1441

Page 18: Acute generalized exanthematous pustulosis (AGEP)

Patch test positive lesion in AGEP

J Clin Invest. 2001; 107(11):1433–1441

Page 19: Acute generalized exanthematous pustulosis (AGEP)

Patch test positive lesion in AGEP

J Clin Invest. 2001; 107(11):1433–1441

Page 20: Acute generalized exanthematous pustulosis (AGEP)

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

Page 21: Acute generalized exanthematous pustulosis (AGEP)

Viral infection and pneumococcal vaccination are most common trigger in pediatric population

etiology

Page 22: Acute generalized exanthematous pustulosis (AGEP)

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

Page 23: Acute generalized exanthematous pustulosis (AGEP)

drug

Page 24: Acute generalized exanthematous pustulosis (AGEP)

EuroSCAR study

Page 25: Acute generalized exanthematous pustulosis (AGEP)

EuroSCAR study

Page 26: Acute generalized exanthematous pustulosis (AGEP)

EuroSCAR study

Page 27: Acute generalized exanthematous pustulosis (AGEP)

Highly suspected drug

Other suspected drug

Page 28: Acute generalized exanthematous pustulosis (AGEP)
Page 29: Acute generalized exanthematous pustulosis (AGEP)
Page 30: Acute generalized exanthematous pustulosis (AGEP)
Page 31: Acute generalized exanthematous pustulosis (AGEP)

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

Page 32: Acute generalized exanthematous pustulosis (AGEP)

Eur J Dermato 2010;20 (4):425-33

Page 33: Acute generalized exanthematous pustulosis (AGEP)
Page 34: Acute generalized exanthematous pustulosis (AGEP)
Page 35: Acute generalized exanthematous pustulosis (AGEP)
Page 36: Acute generalized exanthematous pustulosis (AGEP)

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

Page 37: Acute generalized exanthematous pustulosis (AGEP)

trigger Drug,infection

cell T cell/neutrophil

cytokine CXCL8

patho Sterile pustule filled with neutrophil

My conclusion in AGEP

Page 38: Acute generalized exanthematous pustulosis (AGEP)

Patch test In vitro test: lymphocyte transformation test

investigation

Page 39: Acute generalized exanthematous pustulosis (AGEP)

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

Page 40: Acute generalized exanthematous pustulosis (AGEP)

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

Page 41: Acute generalized exanthematous pustulosis (AGEP)

Thank you