Japanese Guidelines for the Management and Treatment of Generalized Pustular Psoriasis: The New Pathogenesis and Treatment of GPP

J Dermatol. 2018 Nov;45(11):1235-1270. doi: 10.1111/1346-8138.14523. Epub 2018 Sep 19.


Generalized pustular psoriasis (GPP) is a rare disease characterized by recurrent fever and systemic flushing accompanied by extensive sterile pustules. The committee of the guidelines was founded as a collaborative project between the Japanese Dermatological Association and the Study Group for Rare Intractable Skin Diseases under the Ministry of Health, Labour, and Welfare Research Project on Overcoming Intractable Diseases. The aim of the guidelines was to provide current information to aid in the treatment of patients with GPP in Japan. Its contents include the diagnostic and severity classification criteria for GPP, its pathogenesis, and recommendations for the treatment of GPP. Since there are few clinical trial data with high levels of evidence for this rare disease, recommendations by the committee are described in the present guidelines.

Keywords: diagnostic criteria; generalized pustular psoriasis; severity criteria; treatment.

Publication types

  • Guideline
  • Letter

MeSH terms

  • Clinical Trials as Topic
  • Combined Modality Therapy / methods
  • Combined Modality Therapy / standards
  • Dermatologic Agents / therapeutic use*
  • Drug Therapy, Combination / methods
  • Drug Therapy, Combination / standards
  • Humans
  • Japan
  • Keratolytic Agents / therapeutic use
  • Psoriasis / diagnosis
  • Psoriasis / pathology
  • Psoriasis / therapy*
  • Severity of Illness Index
  • Skin / pathology
  • Treatment Outcome
  • Ultraviolet Therapy / methods
  • Ultraviolet Therapy / standards*


  • Dermatologic Agents
  • Keratolytic Agents