Accurate diagnosis of bullous pemphigoid requires multiple health care visits

Päivi Leisti; Anna Pankakoski; Jari Jokelainen; Outi Varpuluoma; Laura Huilaja; Jaana Panelius; Kaisa Tasanen

doi:10.3389/fimmu.2023.1281302

Accurate diagnosis of bullous pemphigoid requires multiple health care visits

Front Immunol. 2023 Nov 27:14:1281302. doi: 10.3389/fimmu.2023.1281302. eCollection 2023.

Authors

Päivi Leisti^#¹, Anna Pankakoski^#², Jari Jokelainen³, Outi Varpuluoma¹, Laura Huilaja^#¹, Jaana Panelius^#², Kaisa Tasanen¹

Affiliations

¹ Department of Dermatology, Research Unit of Clinical Medicine, Medical Research Center Oulu, Oulu University Hospital and University of Oulu, Oulu, Finland.
² Department of Dermatology and Allergology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
³ Infrastructure for Population Studies, Faculty of Medicine, University of Oulu, Oulu, Finland.

^# Contributed equally.

Abstract

Introduction: Accurate use of diagnostic codes is crucial for epidemiological and genetic research based on electronic health record (EHR) data.

Methods: This retrospective study validated the International Classification of Diseases (ICD)-10 diagnostic code L12.0 for bullous pemphigoid (BP) using EHR data from two Finnish university hospitals. We found 1225 subjects with at least one EHR entry of L12.0 between 2009 and 2019. BP diagnosis was based on clinical findings characteristic of BP and positive findings on direct immunofluorescence (DIF), BP180-NC16A enzyme-linked immunosorbent assay (ELISA) or indirect immunofluorescence (IIF) assay.

Results: True BP was found in 901 patients; the positive predictive value (PPV) for L12.0 was 73.6% (95% CI 71.0-76.0). L12.0 was more accurately registered in dermatology units than any specialized health care units (p<0.001). Including patients with multiple L12.0 registrations (≥3), increased the accuracy of the L12.0 code in both dermatology units and other settings.

Discussion: One diagnostic code of L12.0 is not enough to recognize BP in a large epidemiological data set; including only L12.0 registered in dermatology units and excluding cases with <3 L12.0 record entries markedly increases the PPV of BP diagnosis.

Keywords: ICD-10 code; bullous pemphigoid; diagnosis validation; incidence; positive predictive value.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Autoantigens / analysis
Humans
Non-Fibrillar Collagens
Pemphigoid, Bullous* / diagnosis
Pemphigoid, Bullous* / epidemiology
Retrospective Studies
Sensitivity and Specificity

Substances

Autoantigens
Non-Fibrillar Collagens

Grants and funding

The author(s) declare financial support was received for the research, authorship, and/or publication of this article. This study was financially supported by the Academy of Finland (grant number 294738), the Sigrid Jusélius foundation, Inflammation Center of Helsinki University Hospital, Finska Läkaresällskapet, State Research Funding of OUH, University of Oulu Graduate School, Finnish Dermatological Society and Finnish Society of Dermatopathology