Epidemiology and Factors Associated with Remission of Pemphigus Vulgaris and Foliaceus in Singapore

Abstract

Background: Pemphigus is a chronic, relapsing immunobullous disease. There is limited data on the clinical course and prognostic factors of pemphigus in Asian patients. Methods: We conducted a retrospective cohort study of all newly diagnosed pemphigus vulgaris (PV) and pemphigus foliaceus (PF) patients seen at the National Skin Centre from 1 January 2004 to 31 December 2009. Demographic and clinical data on co-morbidities, treatment and remission were recorded. Mortality information was obtained from the National Registry of Diseases. Prognostic endpoint was overall remission at last visit. Results: Sixty- one patients (36 PV and 25 PF) were recruited. Among PV patients, higher initial prednisolone dose (P = 0.017) and the use of azathioprine (P = 0.028) were significantly associated with overall remission at last visit. However, higher desmoglein 1 antibody titres at diagnosis (P = 0.024) and the use of dapsone (P = 0.008) were negatively associated with overall remission at last visit. Among PF patients, only higher desmoglein 1 antibody titre at diagnosis (P = 0.041) was found to be associated with lower overall remission at last visit. There was no mortality during the 3-year follow-up period in both PV and PF. Conclusions: Higher initial prednisolone dose and the use of azathioprine in PV desmoglein 1 antibody titre at diagnosis in PV and PF might be prognostic markers for achieving remission. Use of dapsone was associated with lower overall remission in PV, but this might be confounded because dapsone was used as an adjuvant therapy in recalcitrant cases. Owing to study methodology and limitations, further evaluation is needed for better prognostication of pemphigus. Ann Acad Med Singapore 2020;49:367–76 Key words: Autoimmune blistering disease, Dermatology, Pemphigus foliaceus, Pemphigus vulgaris, Remission

Worldwide epidemiologic factors in pemphigus vulgaris and bullous pemphigoid

“…Twenty-five papers were reviewed that reported on the gender ratio of females to males in PV ( 10 , 23 – 26 , 31 , 34 , 36 , 40 , 42 , 43 , 58 – 72 ). The lowest ratio, less than one, indicating there were more males affected than females, was found in Saudi Arabia with a ratio of 0.46 ( 25 ).…”

“…Fourteen papers were reviewed that presented information on the mean age of onset in PV patients enrolled in their study ( 8 , 23 – 26 , 31 , 36 , 38 , 40 , 42 , 43 , 58 , 64 , 71 ). The ages ranged from 36.5 years in Kuwait ( 38 ) to 71 years in the United Kingdom ( 36 ), with the average age across these studies being 49.8 years ( Figure 4A ).…”

“…26, 31, 36, 38, 40,42, 43,58,64,71). The ages ranged from 36.5 years in Kuwait (38) to 71 years in the United Kingdom (36), with the average age across these studies being 49.8 years (Figure…”