title: Development of a disease registry for autoimmune bullous diseases: initial analysis of the pemphigus vulgaris subset.
abstract: Pemphigus vulgaris (PV) is a rare, potentially life threatening, autoimmune blistering skin disease. The International Pemphigus and Pemphigoid Foundation (IPPF) has recently developed a disease registry with the aim to enhance our understanding of autoimmune bullous diseases with the long-term goal of acquiring information to improve patient care. Patients were recruited to the IPPF disease registry through direct mail, e-mail, advertisements, and articles in the IPPF-quarterly, -website, -Facebook webpage, and IPPF Peer Health Coaches to complete a 38-question survey. We present here the initial analysis of detailed clinical information collected on 393 PV patients. We report previously unrecognized gender differences in terms of lesion location, autoimmune comorbidity, and delay in diagnosis. The IPPF disease registry serves as a useful resource and guide for future clinical investigation.
authors.affiliation: Department of Dermatology, University at Buffalo and Roswell Park Cancer Institute, Buffalo, USA.
authors.forename: Amit Aakash
authors.initials: AA
authors.lastname: Shah
authors.forename: Kristina
authors.initials: K
authors.lastname: Seiffert-Sinha
authors.forename: David
authors.initials: D
authors.lastname: Sirois
authors.forename: Victoria P
authors.initials: VP
authors.lastname: Werth
authors.forename: Badri
authors.initials: B
authors.lastname: Rengarajan
authors.forename: William
authors.initials: W
authors.lastname: Zrnchik
authors.forename: Kristopher
authors.initials: K
authors.lastname: Attwood
authors.forename: Animesh A
authors.initials: AA
authors.lastname: Sinha
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015551:Autoimmunity; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D057210:Delayed Diagnosis; D018450:Disease Progression; D005260:Female; D006306:Health Surveys; D006801:Humans; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000628:therapy; D011237:Predictive Value of Tests; D016730:Program Development; D012008:Recurrence; D012042:Registries; D012074:Remission Induction; D012307:Risk Factors; D012737:Sex Factors; D011795:Surveys and Questionnaires; D013997:Time Factors; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology; D055815:Young Adult