Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum
- 1 April 2018
- journal article
- research article
- Published by American Medical Association (AMA) in JAMA Dermatology
- Vol. 154 (4), 461-466
- https://doi.org/10.1001/jamadermatol.2017.5980
Abstract
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.Keywords
This publication has 46 references indexed in Scilit:
- Idiopathic Pyoderma Gangrenosum: Successful Resolution with Infliximab Therapy and Pro-inflammatory Cytokines AssessmentActa Dermato-Venereologica, 2012
- Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patientsBritish Journal of Dermatology, 2011
- Pyoderma gangrenosum associated with renal carcinomaJournal of the American Academy of Dermatology, 2011
- Role of inflammatory cells, cytokines and matrix metalloproteinases in neutrophil-mediated skin diseasesClinical and Experimental Immunology, 2010
- Successful treatment of severe pyoderma gangrenosum with pimecrolimus cream 1%Journal of the European Academy of Dermatology and Venereology, 2007
- Extranodal NK/T-Cell Lymphoma Nasal Type Mimicking Pyoderma GangrenosumActa Dermato-Venereologica, 2007
- Pyoderma gangrenosum with spleen involvement and monoclonal IgA gammopathyJournal of the European Academy of Dermatology and Venereology, 2004
- Efficacy and systemic absorption of topical tacrolimus used in pyoderma gangrenosumBritish Journal of Dermatology, 2004
- Multiple lesions of pyoderma gangrenosum in association with hyper-reactive malarial splenomegalyBritish Journal of Dermatology, 2004
- Pyoderma gangrenosum: Classification and managementJournal of the American Academy of Dermatology, 1996