Skip to main content

Acute Generalized Exanthematous Pustulosis: AGEP

  • Chapter
  • First Online:
Deadly Dermatologic Diseases

Abstract

Acute generalized exanthematous pustulosis (AGEP) is a rare clinical entity characterized by an acute onset of numerous sterile, nonfollicular, pinhead-sized pustules arising on an erythematous, edematous base accompanied by fever (>38 C), neutrophilia, and occasionally eosinophilia. In 1968, Baker and Ryan reported 104 cases of pustular psoriasis and specifically detailed five patients whom they diagnosed as having “exanthematic pustular psoriasis” most likely due to a drug or infectious etiology. These five patients did not have a history of psoriasis and were characterized as having pustular skin reactions of acute onset that quickly resolved and did not recur. Subsequently, in 1980, Beylot et al. reviewed the existing literature regarding pustuloses and reported four additional cases; they are responsible for coining the name of this distinct entity as “acute generalized exanthematic pustulosis.” Additional literature reports by Macmillan in 1973 and Staughton and Harper in 1984 denote pustular skin eruptions most likely representing AGEP which they termed as “generalized pustular drug rash” and “toxic pustuloderma,” respectively.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 109.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Baker H, Ryan TJ. Generalized pustular psoriasis. A clinical and epidemiological study of 104 cases. Br J Dermatol. 1968;80(12):771–93.

    Article  CAS  PubMed  Google Scholar 

  2. Beylot C, Bioulac P, Doutre MS. Acute generalized exanthematic pustuloses (four cases) (author’s transl). Ann Dermatol Venereol. 1980;107(1–2):37–48.

    CAS  PubMed  Google Scholar 

  3. Macmillan AL. Generalised pustular drug rash. Dermatologica. 1973;146(5):285–91.

    Article  CAS  PubMed  Google Scholar 

  4. Staughton RC, Payne CM, Harper JI, McMichen H. Toxic pustuloderma–a new entity? J R Soc Med. 1984;77 Suppl 4:6–8.

    PubMed  PubMed Central  Google Scholar 

  5. Sidoroff A, Dunant A, Viboud C, Halevy S, Bavinck JN, Naldi L, et al. Risk factors for acute generalized exanthematous pustulosis (AGEP)-results of a multinational case-control study (EuroSCAR). Br J Dermatol. 2007;157(5):989–96.

    Article  CAS  PubMed  Google Scholar 

  6. Kostopoulos TC, Krishna SM, Brinster NK, Ortega-Loayza AG. Acute generalized exanthematous pustulosis: atypical presentations and outcomes. J Eur Acad Dermatol Venereol. 2014;8:209–14.

    Google Scholar 

  7. Feio AB, Apetato M, Costa MM, Sa J, Alcantara J. Acute generalized exanthematous pustulosis due to Coxsackie B4 virus. Acta Med Port. 1997;10(6–7):487–91.

    CAS  PubMed  Google Scholar 

  8. Naides SJ, Piette W, Veach LA, Argenyi Z. Human parvovirus B19-induced vesiculopustular skin eruption. Am J Med. 1988;84(5):968–72.

    Article  CAS  PubMed  Google Scholar 

  9. Roujeau JC, Bioulac-Sage P, Bourseau C, Guillaume JC, Bernard P, Lok C, et al. Acute generalized exanthematous pustulosis. Analysis of 63 cases. Arch Dermatol. 1991;127(9):1333–8.

    Article  CAS  PubMed  Google Scholar 

  10. Klein N, Hartmann M, Helmbold P, Enk A. Acute generalized exanthematous pustulosis associated with recurrent urinary tract infections. Hautarzt. 2009;60(3):226–8.

    Article  CAS  PubMed  Google Scholar 

  11. Manzano S, Guggisberg D, Hammann C, Laubscher B. Acute generalized exanthematous pustulosis: first case associated with a Chlamydia pneumoniae infection. Arch Pediatr. 2006;13(9):1230–2.

    Article  CAS  PubMed  Google Scholar 

  12. Davidovici BB, Pavel D, Cagnano E, Rozenman D, Halevy S. EuroSCAR, et al. Acute generalized exanthematous pustulosis following a spider bite: report of 3 cases. J Am Acad Dermatol. 2006;55(3):525–9.

    Article  PubMed  Google Scholar 

  13. Cannistraci C, Parola ILL, RiganÒ R, Bassetti F, Ortona E, Santucci B, et al. Acute generalized exanthematous pustulosis in cystic echinococcosis: immunological characterization. Br J Dermatol. 2003;148(6):1245–9.

    Article  CAS  PubMed  Google Scholar 

  14. Schmid S, Kuechler PC, Britschgi M, Steiner UC, Yawalkar N, Limat A, et al. Acute generalized exanthematous pustulosis: role of cytotoxic T cells in pustule formation. Am J Pathol. 2002;161(6):2079–86.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Sidoroff A, Halevy S, Bavinck JN, Vaillant L, Roujeau JC. Acute generalized exanthematous pustulosis (AGEP)–a clinical reaction pattern. J Cutan Pathol. 2001;28(3):113–9.

    Article  CAS  PubMed  Google Scholar 

  16. Meadows KP, Egan CA, Vanderhooft S. Acute generalized exanthematous pustulosis (AGEP), an uncommon condition in children: case report and review of the literature. Pediatr Dermatol. 2000;17(5):399–402.

    Article  CAS  PubMed  Google Scholar 

  17. Hotz C, Valeyrie-Allanore L, Haddad C, Bouvresse S, Ortonne N, Duong TA, et al. Systemic involvement of acute generalized exanthematous pustulosis: a retrospective study on 58 patients. Br J Dermatol. 2013;169(6):1223–32.

    Article  CAS  PubMed  Google Scholar 

  18. Krishna S, Ortega-Loayza A, Malakouti N, Brinster N. A rapidly progressive and fatal case of atypical acute generalized exanthematous pustulosis. J Am Acad Dermatol. 2014;71(3):e89–90.

    Article  PubMed  Google Scholar 

  19. Halevy S, Kardaun SH, Davidovici B, Wechsler J, EuroSCAR and RegiSCAR Study Group. The spectrum of histopathological features in acute generalized exanthematous pustulosis: a study of 102 cases. Br J Dermatol. 2010;163(6):1245–52.

    Article  CAS  PubMed  Google Scholar 

  20. Kardaun SH, Kuiper H, Fidler V, Jonkman MF. The histopathological spectrum of acute generalized exanthematous pustulosis (AGEP) and its differentiation from generalized pustular psoriasis. J Cutan Pathol. 2010;37(12):1220–9.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Kara Trapp BA .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Trapp, K. (2016). Acute Generalized Exanthematous Pustulosis: AGEP. In: Crowe, D., Morgan, M., Somach, S., Trapp, K. (eds) Deadly Dermatologic Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-31566-9_28

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-31566-9_28

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-31564-5

  • Online ISBN: 978-3-319-31566-9

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics