Funding sources This work was generously supported by the British Skin Foundation, The Immunodermatology Fund of the Guy's and St Thomas' Charity and the National Institute for Health Research Comprehensive Biomedical Research Centre at Guy's and St Thomas' NHS Foundation Trust/King's College London.
Clinical and Laboratory Investigations
Prognostic factors in pemphigus vulgaris and pemphigus foliaceus
Article first published online: 13 JAN 2014
© 2013 British Association of Dermatologists
British Journal of Dermatology
Volume 170, Issue 1, pages 116–122, January 2014
How to Cite
Saha, M., Bhogal, B., Black, M.M., Cooper, D., Vaughan, R.W. and Groves, R.W. (2014), Prognostic factors in pemphigus vulgaris and pemphigus foliaceus. British Journal of Dermatology, 170: 116–122. doi: 10.1111/bjd.12630
Conflicts of interest None.
- Issue published online: 13 JAN 2014
- Article first published online: 13 JAN 2014
- Accepted manuscript online: 14 SEP 2013 03:55AM EST
- Manuscript Accepted: 31 AUG 2013
- British Skin Foundation
- Guy's and St Thomas' Charity
- National Institute for Health Research Comprehensive Biomedical Research Centre at Guy's
- NHS Foundation Trust/King's College London
Pemphigus typically has a chronic course, although there is great variability in disease duration (DD) and time taken to disease remission (DR) between individuals with the disease. The reasons for this are unclear.
To explore the prognostic influence of epidemiological, clinical, immunological and genetic factors on disease course and remission in pemphigus vulgaris (PV) and pemphigus foliaceus (PF).
This was a retrospective study of patients with PV and PF, recruited from a single UK centre. Direct and indirect immunofluorescence and enzyme-linked immunosorbent assay studies for antidesmoglein (Dsg) antibodies were used to assess immunological factors. Polymerase chain reaction with sequence specific primers (PCR-SSP) was used to assess the Class II human leukocyte antigen status of patients. Prognostic endpoints investigated were time to initial first DR and total DD.
Ninety-five patients were recruited (79 PV and 16 PF). Patients of Indo-Asian origin were significantly associated with longer DD than White-British patients (P = 0·029). In addition, younger age at onset was associated with a worse prognosis in terms of DD: the mean age at presentation of patients with DD of < 5 years was 49 years (SEM = 3·4) compared with 40 years (SEM = 1·9) in those with DD > 5 years (P = 0·039). A higher initial intercellular antibody titre on normal human skin substrate was associated with a greater time to initial DR (P = 0·007) and high anti-Dsg 3 levels at baseline were associated with a longer total DD (P = 0·03).
Ethnic group, age at presentation, initial intercellular antibody titre and initial Dsg 3 antibody levels all had a significant impact on prognosis of pemphigus.