title: The histopathological spectrum of acute generalized exanthematous pustulosis (AGEP) and its differentiation from generalized pustular psoriasis.
abstract: BACKGROUND
  Acute generalized exanthematous pustulosis (AGEP) represents a severe, acute, pustular skin reaction that is most often induced by drugs. AGEP can be difficult to differentiate from generalized pustular psoriasis (GPP) both clinically and histopathologically. We present a systematic description of the histopathological spectrum of AGEP and GPP with a focus on discriminating features.
  
  
  METHODS
  A retrospective, descriptive, comparative histopathological study was completed utilizing step sections of 43 biopsies of 29 cases with a validated diagnosis of probable or definite AGEP and 24 biopsies of 19 cases with an established diagnosis of GPP.
  
  
  RESULTS
  In AGEP, biopsies from erythema and pustules showed minor differences, whereas histopathology of the acute stage of GPP showed major differences compared to the chronic stage. Comparing AGEP and GPP, the presence of eosinophils, necrotic keratinocytes, a mixed interstitial and mid-dermal perivascular infiltrate and absence of tortuous or dilated blood vessels were in favor of AGEP. Moreover, chronic GPP was characterized by prominent epidermal psoriatic changes. The frequency of a psoriatic background of AGEP patients in our study was higher than that of psoriasis in the general population. However, histopathology of a subgroup of AGEP patients with a personal history of psoriasis revealed no significant differences from the other AGEP patients.
  
  
  CONCLUSIONS
  The spectrum of histopathological features of both AGEP and GPP is presented. Despite considerable overlap, subtle consistent histopathological differences and the grade of severity of specific features can help in differentiation. We could neither substantiate earlier reports that follicular pustules exclude AGEP nor did we see vasculitis as a specific feature in AGEP. Our study also supports the concept that AGEP is a separate entity that is distinct from GPP.
authors.affiliation: Department of Dermatology, Reference Center for Cutaneous Adverse Drug Reactions, University Medical Center Groningen, University of Groningen, Hanzeplein 1, Groningen, The Netherlands. s.h.kardaun@derm.umcg.nl
authors.forename: Sylvia H
authors.initials: SH
authors.lastname: Kardaun
authors.forename: Hilde
authors.initials: H
authors.lastname: Kuiper
authors.forename: Vaclav
authors.initials: V
authors.lastname: Fidler
authors.forename: Marcel F
authors.initials: MF
authors.lastname: Jonkman
mesh_terms: D000208:Acute Disease; D056150:Acute Generalized Exanthematous Pustulosis / Q000473:pathology*; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011565:Psoriasis / Q000473:pathology; D012189:Retrospective Studies