acute generalized exanthematous pustulosis (agep)

Acute generalized exanthematous pustolosis

Planee Vatanasurkitt,MD

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

outlines

1-5 per million/year

incidence

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

(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

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

Pustular psoriasis

Subcorneal pustular dermatosis

Pustular vasculitis

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

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

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

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

Patch test positive lesion in AGEP

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

Patch test positive lesion in AGEP

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

Patch test positive lesion in AGEP

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,

etiology

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

etiology

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

drug

EuroSCAR study

EuroSCAR study

EuroSCAR study

Highly suspected drug

Other suspected drug

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

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

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

trigger Drug,infection

cell T cell/neutrophil

cytokine CXCL8

patho Sterile pustule filled with neutrophil

My conclusion in AGEP

Patch test In vitro test: lymphocyte transformation test

investigation

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

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

Thank you