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
