REVIEW   Free access

Italian Journal of Dermatology and Venereology 2022 December;157(6):489-96

DOI: 10.23736/S2784-8671.22.07415-1

Copyright © 2022 EDIZIONI MINERVA MEDICA

language: English

Pustular psoriasis with a focus on generalized pustular psoriasis: classification and diagnostic criteria. An Italian expert consensus

Antonio COSTANZO ^{1, 2} ✉, Federico BARDAZZI ^{3, 4}, Clara DE SIMONE ^{5, 6}, Gabriella FABBROCINI ⁷, Caterina FOTI ⁸, Angelo V. MARZANO ^{9, 10}, Luigi NALDI ¹¹, Aurora PARODI ¹², Francesca PRIGNANO ¹³, Giampiero GIROLOMONI ¹⁴

¹ Unit of Dermatology, Department of Biomedical Sciences, Humanitas University, Milan, Italy; ² Skin Pathology Laboratory, IRCCS Humanitas Research Hospital, Rozzano, Milan, Italy; ³ Department of Dermatology, IRCCS S. Orsola-Malpighi Polyclinic, Bologna, Italy; ⁴ Department of Specialistic, Diagnostic and Experimental Medicine (DIMES), Alma Mater Studiorum, University of Bologna, Bologna, Italy; ⁵ Section of Dermatology, Sacred Heart Catholic University, Rome, Italy; ⁶ Department of Dermatology, IRCCS A. Gemelli University Polyclinic Foundation, Rome, Italy; ⁷ Section of Dermatology and Venereology, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy; ⁸ Section of Dermatology, Department of Biomedical Science and Human Oncology, University of Bari, Bari, Italy; ⁹ Unit of Dermatology, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy; ¹⁰ Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy; ¹¹ Department of Dermatology, AULSS8 - San Bortolo Hospital, Vicenza, Italy; ¹² DiSSal Sezione Dermatologia, IRCCS San Martino Polyclinic Hospital, University of Genoa, Genoa, Italy; ¹³ Section of Dermatology, Department of Health Sciences, University of Florence, Florence, Italy; ¹⁴ Section of Dermatology, Department of Medicine, University of Verona, Verona, Italy

Generalized pustular psoriasis (GPP) is a severe and life-threatening systemic disease associated with significant morbidity and mortality. Recent progress has been made in understanding the pathogenetic pathways involved in GPP and an intricate interaction between innate and adaptive immune mechanisms has been suggested. Despite formal consensus guidelines on pustular psoriasis currently available in the literature, the definitions and classifications of GPP used across studies were inconsistent. Consequently, there are no unified criteria that can be universally adopted for precise diagnosis, classification and effective treatment of GPP patients with new targeted drugs. The aim of this review was to collect all the main evidence on available diagnostic criteria for GPP and to establish recommendations in order to promote a better stratification and therapeutic management of this severe and heterogeneous disease.

KEY WORDS: Psoriasis; Diagnosis; Consensus