1 1 American Journal of Clinical Dermatology 2 Review Article 3 4 Generalized pustular psoriasis: Clinical management and update on autoinflammatory 5 aspects 6 7 Takuya Takeichi, MD, PhD and Masashi Akiyama, MD, PhD 8 9 Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan 10 11 Corresponding Author: 12 Takuya Takeichi, MD, PhD 13 Department of Dermatology, Nagoya University Graduate School of Medicine 14 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi 466-8550, Japan 15 Tel: +81-52-744-2314, Fax: +81-52-744-2318 16 E-mail:
[email protected] 17 Masashi Akiyama, MD, PhD 18 E-mail:
[email protected] 19 20 Running heading: Review of GPP as autoinflammatory disease 21 22 Compliance with Ethical Standards: 23 Author: Takuya Takeichi 24 Funding sources: JSID’s Fellowship Shiseido Research Grant 2019 to TT. 25 Conflicts of interest: None declared 1 2 26 27 Author: Masashi Akiyama 28 Funding sources: JSID’s Fellowship Shiseido Research Grant 2019 to TT. 29 Conflicts of interest: None declared 30 31 32 Word, table and figure counts: 3,371/6,000 words, 60 references, 4 tables, 1 figure 33 2 3 34 Abstract 35 Generalized pustular psoriasis (GPP) is a chronic, systemic inflammatory disease accompanied 36 by high fever and general malaise. Diffuse erythema and swelling of the extremities occur with 37 multiple sterile pustules all over the body in GPP patients. GPP often relapses over the lifetime 38 and can be life-threatening. Recent discoveries of the underlying molecular genetic basis of 39 many cases of this disorder have provided major advances to clinicians and researchers towards 40 an understanding of the pathomechanism of GPP.