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Maverakis E, Ma C, Shinkai K, Fiorentino D, Callen JP, Wollina U, Marzano AV, Wallach D, Kim K, Schadt C, Ormerod A, Fung MA, Steel A, Patel F, Qin R, Craig F, Williams HC, Powell F, Merleev A, Cheng MY, et al.
JAMA dermatology. Date of publication 2018 Apr 1;volume 154(4):461-466.
1. JAMA Dermatol. 2018 Apr 1;154(4):461-466. doi: 10.1001/jamadermatol.2017.5980. Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum: A Delphi Consensus of International Experts. Maverakis E(1), Ma C(1), Shinkai K(2), Fiorentino D(3)(4), Callen JP(5)(6), Wollina U(7), Marzano AV(8), Wallach D(9), Kim K(10), Schadt C(5), Ormerod A(11), Fung MA(1), Steel A(1), Patel F(1), Qin R(1), Craig F(11), Williams HC(12), Powell F(13), Merleev A(1), Cheng MY(1). Author information: (1)Department of Dermatology, University of California, Davis, Sacramento. (2)Department of Dermatology, University of California, San Francisco. (3)Division of Immunology and Rheumatology, Department of Dermatology, Stanford University, Stanford, California. (4)Division of Immunology and Rheumatology, Department of Internal Medicine, Stanford University, Stanford, California. (5)Division of Dermatology, Department of Medicine, University of Louisville, Louisville, Kentucky. (6)Associate Editor. (7)Department of Dermatology and Allergology, Academic Teaching Hospital Dresden, Dresden, Germany. (8)UOC di Dermatologia, IRCCS Fondazione Ca' Granda Ospedale Maggiore Policlinico, Milano-Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Milano, Italy. (9)Department of Dermatology, Paris Hospitals, Paris, France. (10)Division of Biostatistics, Department of Public Health Sciences, University of California, Davis. (11)Department of Dermatology, School of Medicine and Dentistry, University of Aberdeen, Aberdeen, United Kingdom. (12)Centre of Evidence Based Dermatology, King's Meadow Campus, University of Nottingham, United Kingdom. (13)Charles Institute of Dermatology, University College, Dublin, Ireland. Importance: Pyoderma gangrenosum is a rare inflammatory skin condition that is difficult to diagnose. Currently, it is a "diagnosis of exclusion," a definition not compatible with clinical decision making or inclusion for clinical trials. Objective: To propose and validate diagnostic criteria for ulcerative pyoderma gangrenosum. Evidence Review: Diagnostic criteria were created following a Delphi consensus exercise using the RAND/UCLA Appropriateness Method. The criteria were validated against peer-reviewed established cases of pyoderma gangrenosum and mimickers using k-fold cross-validation with methods of multiple imputation. Findings: Delphi exercise yielded 1 major criterion-biopsy of ulcer edge demonstrating neutrophilic infiltrate-and 8 minor criteria: (1) exclusion of infection; (2) pathergy; (3) history of inflammatory bowel disease or inflammatory arthritis; (4) history of papule, pustule, or vesicle ulcerating within 4 days of appearing; (5) peripheral erythema, undermining border, and tenderness at ulceration site; (6) multiple ulcerations, at least 1 on an anterior lower leg; (7) cribriform or "wrinkled paper" scar(s) at healed ulcer sites; and (8) decreased ulcer size within 1 month of initiating immunosuppressive medication(s). Receiver operating characteristic analysis revealed that 4 of 8 minor criteria maximized discrimination, yielding sensitivity and specificity of 86% and 90%, respectively. Conclusions and Relevance: This Delphi exercise produced 1 major criterion and 8 minor criteria for the diagnosis of ulcerative pyoderma gangrenosum. The criteria may serve as a guideline for clinicians, allowing for fewer misdiagnoses and improved patient selection for clinical trials. DOI: 10.1001/jamadermatol.2017.5980 PMID: 29450466
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Pyoderma Gangrenosum - Introduction and Assessment