TY - JOUR
T1 - Generalized pustular psoriasis
T2 - overview of the status quo and results of a panel discussion
AU - Reich, Kristian
AU - Augustin, Matthias
AU - Gerdes, Sascha
AU - Ghoreschi, Kamran
AU - Kokolakis, Georgios
AU - Mößner, Rotraut
AU - Mrowietz, Ulrich
AU - Navarini, Alexander A.
AU - Pinter, Andreas
AU - Schäkel, Knut
AU - Staubach, Petra
AU - Sticherling, Michael
AU - Thaçi, Diamant
AU - Wilsmann-Theis, Dagmar
N1 - Publisher Copyright:
© 2022 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.
PY - 2022/6
Y1 - 2022/6
N2 - Generalized pustular psoriasis (GPP) is a rare, severe, potentially life-threatening, autoinflammatory, neutrophilic skin disease that may be accompanied by fever and leukocytosis. This paper describes the current state of knowledge on GPP in terms of classification, (differential) diagnosis and prevalence. We present a comparison of the genetics and pathoimmunology of GPP and psoriasis vulgaris with the central mechanisms of autoimmunology and autoinflammation. The currently available therapeutic options, expert recommendations for therapy, and data from early clinical trials investigating targeted therapies will be summarized. We present the results of our discussion with 13 experts for psoriasis vulgaris and GPP and give an integrated overview of indication and therapy based on our personal experience and present an outlook on further research questions. Collectively, this article highlights the high unmet need in GPP, as there exists no satisfactory method of diagnosis or treatment to date and new treatment options will be of great therapeutic benefit to those affected.
AB - Generalized pustular psoriasis (GPP) is a rare, severe, potentially life-threatening, autoinflammatory, neutrophilic skin disease that may be accompanied by fever and leukocytosis. This paper describes the current state of knowledge on GPP in terms of classification, (differential) diagnosis and prevalence. We present a comparison of the genetics and pathoimmunology of GPP and psoriasis vulgaris with the central mechanisms of autoimmunology and autoinflammation. The currently available therapeutic options, expert recommendations for therapy, and data from early clinical trials investigating targeted therapies will be summarized. We present the results of our discussion with 13 experts for psoriasis vulgaris and GPP and give an integrated overview of indication and therapy based on our personal experience and present an outlook on further research questions. Collectively, this article highlights the high unmet need in GPP, as there exists no satisfactory method of diagnosis or treatment to date and new treatment options will be of great therapeutic benefit to those affected.
UR - http://www.scopus.com/inward/record.url?scp=85131378118&partnerID=8YFLogxK
U2 - 10.1111/ddg.14764
DO - 10.1111/ddg.14764
M3 - Scientific review articles
C2 - 35674482
AN - SCOPUS:85131378118
SN - 1610-0379
VL - 20
SP - 753
EP - 771
JO - JDDG - Journal of the German Society of Dermatology
JF - JDDG - Journal of the German Society of Dermatology
IS - 6
ER -
