Abstract

Background: 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.

Objectives: 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).

Methods: 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.

Results: 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).

Conclusions: Ethnic group, age at presentation, initial intercellular antibody titre and initial Dsg 3 antibody levels all had a significant impact on prognosis of pemphigus.

Where can I read this paper?

Full article on Wiley Online Library.

Published: 2014 Jan;170(1):116-22. doi: 10.1111/bjd.12630.