Description

Sidoroff et al reported a score for the diagnosis of Acute Generalized Exanthematous Pustulosis (AGEP). The authors are from multiple institutions in Europe and Israel.


Parameters:

(1) pustules

(2) erythema

(3) distribution/pattern

(4) postpustular desquamation

(5) mucosal involvement

(6) acute onset (<= 10 days)

(7) resolution in days

(8) body temperature in °C

(9) absolute neutrophil count per microliter

(10) evidence of another disease on histology

(11) other histologic features

 

Parameter

Finding

Points

pustules

typical

2

 

compatible

1

 

insufficient

0

erythema

typical

2

 

compatible

1

 

insufficient

0

distribution/pattern

typical

2

 

compatible

1

 

insufficient

0

postpustular desquamation

yes

1

 

no

0

 

insufficient

0

mucosal involvement

no

0

 

yes

-2

acute onset

yes

0

 

no

-2

resolution

<= 15 days

0

 

> 15 days

-4

body temperature

< 38°C

0

 

>= 38°C

1

ANC

< 7,000 per µL

0

 

>= 7,000 per µL

1

other disease

no

0

 

yes

-10

other histology

spongiform subcorneal and/or intraepidermal pustules with papillary edema

3

 

2

 

exocystosis of neutrophils

1

 

none of these

0

 

one of the following:

(1) non-spongiform subcorneal and/or intraepidermal pustules

(2) NOS pustules with papillary edema

(3) (subcorneal and/or epidermal spongiform or NOS pustules) without papillary edema

 

total score =

= SUM(points for all of the parameters)

 

Interpretation:

• minimum score: -18

• maximum score: 12

 

Score

AGEP

<= 0

no

1 to 4

possible

5 to 7

probable

8 to 12

definite

 


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