Diagnostic criteria of ulcerative Pyoderma Gangrenosum a delphi consensus of international experts

Emanual Maverakis, Chelsea Ma, Kanade Shinkai, David Fiorentino, Jeffrey P. Callen, Uwe Wollina, Angelo Valerio Marzano, Daniel Wallach, Kyoungmi Kim, Courtney Schadt, Anthony Ormerod, Maxwell A. Fung, Andrea Steel, Forum Patel, Rosie Qin, Fiona Craig, Hywel C. Williams, Frank Powell, Alexander Merleev, Michelle Y. Cheng

Research output: Contribution to journalReview article

53 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)461-466
Number of pages6
JournalJAMA Dermatology
Volume154
Issue number4
DOIs
Publication statusPublished - Apr 1 2018

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Pyoderma Gangrenosum
Ulcer
Clinical Trials
Erythema
Immunosuppressive Agents
Diagnostic Errors
ROC Curve
Patient Selection
Arthritis
Cicatrix
Leg
Guidelines
Biopsy
Sensitivity and Specificity
Skin
Infection

ASJC Scopus subject areas

  • Dermatology

Cite this

Maverakis, E., Ma, C., Shinkai, K., Fiorentino, D., Callen, J. P., Wollina, U., ... Cheng, M. Y. (2018). Diagnostic criteria of ulcerative Pyoderma Gangrenosum a delphi consensus of international experts. JAMA Dermatology, 154(4), 461-466. https://doi.org/10.1001/jamadermatol.2017.5980

Diagnostic criteria of ulcerative Pyoderma Gangrenosum a delphi consensus of international experts. / Maverakis, Emanual; Ma, Chelsea; Shinkai, Kanade; Fiorentino, David; Callen, Jeffrey P.; Wollina, Uwe; Marzano, Angelo Valerio; Wallach, Daniel; Kim, Kyoungmi; Schadt, Courtney; Ormerod, Anthony; Fung, Maxwell A.; Steel, Andrea; Patel, Forum; Qin, Rosie; Craig, Fiona; Williams, Hywel C.; Powell, Frank; Merleev, Alexander; Cheng, Michelle Y.

In: JAMA Dermatology, Vol. 154, No. 4, 01.04.2018, p. 461-466.

Research output: Contribution to journalReview article

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 2018, 'Diagnostic criteria of ulcerative Pyoderma Gangrenosum a delphi consensus of international experts', JAMA Dermatology, vol. 154, no. 4, pp. 461-466. https://doi.org/10.1001/jamadermatol.2017.5980
Maverakis, Emanual ; Ma, Chelsea ; Shinkai, Kanade ; Fiorentino, David ; Callen, Jeffrey P. ; Wollina, Uwe ; Marzano, Angelo Valerio ; Wallach, Daniel ; Kim, Kyoungmi ; Schadt, Courtney ; Ormerod, Anthony ; Fung, Maxwell A. ; Steel, Andrea ; Patel, Forum ; Qin, Rosie ; Craig, Fiona ; Williams, Hywel C. ; Powell, Frank ; Merleev, Alexander ; Cheng, Michelle Y. / Diagnostic criteria of ulcerative Pyoderma Gangrenosum a delphi consensus of international experts. In: JAMA Dermatology. 2018 ; Vol. 154, No. 4. pp. 461-466.
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N2 - 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.

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