This document discusses acute generalized exanthematous pustulosis (AGEP). It describes the clinical features as widespread erythema and hundreds of small pustules, differential diagnoses, and histopathology showing subcorneal and intraepidermal pustules. Common triggers are drugs (90%) and infections. The pathophysiology is not fully understood but may involve a type IV hypersensitivity reaction with T cells and neutrophils secreting cytokines. Treatment involves discontinuing the culprit drug and treating any infections.

1. Planee Vatanasurkitt,MD

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

3.  1-5 per million/year

4.  Widespread
erythema with
hundreds of
small, flaccid, confl
uent, non-follicular
pustules, especially
along the groins
and on the flexor
surfaces of the
legs.

5.  (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

6. 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 hands
Sidoroff A et al. Acute generalized exanthematous pustulosis
(AGEP)

11.  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

12. Two subcorneal, intraepidermal pustules with
papillary edema
and a mixed inflammatory infiltrate of mainly
neutrophils and some eosinophils.
Eur J Dermato 2010;20 4 :425-3

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

14. Healthy skin AGEP
J Clin Invest. 2001; 107(11):1433–
1441

15. Healthy skin AGEP
J Clin Invest. 2001; 107(11):1433–
1441

16. Healthy skin AGEP
J Clin Invest. 2001; 107(11):1433–
1441

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

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

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

20.  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,

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

22.  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

27. Highly suspected drug
Other suspected drug

31.  Not yet understood
 Suggest genetic hypersensitivity or type 4
hypersensitivity
 Can be divided to 3 phase
Eur J Dermato 2010;20 4 :425-3

32. Eur J Dermato 2010;20 4 :425-3

36.  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

37. trigger Drug,infection
cell T cell/neutrophil
cytokine CXCL8
patho Sterile pustule filled with
neutrophil

38.  Patch test
 In vitro test: lymphocyte transformation test

39.  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

40.  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