Pemphigus in South Africa

Background Pemphigus is an autoimmune disease characterized by intraepiderm al blistering. We describe the demography, prevalence, clinical features, r esponse to treatment, and human leukocyte antigen (HLA) characteristics of pemphigus in Kwa-Zulu Natal, South Africa. Methods All patients with pemphigus were prospectively recruited over 12 ye ars from January 1987 to December 1999. The demography, clinical features, histology, and immunofluorescence (IF) were recorded. In a subset of patien ts, HLA tests were performed. Results One hundred and twelve patients had pemphigus. Pemphigus foliaceus (PF) was the commonest variant seen (62 patients) and 80% of these patients were black. The mean age was 43 years (12-93 years) and the male to female ratio was 1 : 1.4. Fifty patients had pemphigus vulgaris (PV), of whom 82% were Indian. The mean age of presentation of PV was 48 years (21-82 years) . The male to female ratio was 1 :1.7. There was no mucosal involvement in PF. PV patients had painful oral lesions. The mortality rate was 14% in the total sample (six in PV and two in PF). HLA-B8 was positive in 41% of pati ents with PF (P<0.001). Conclusions PF occurs more commonly in black people, while most cases in In dians present with the PV subtype. Pemphigus patients present with severe a nd extensive disease, and PV patients share features in common with patient s from their land of origin (India), suggesting a genetic link.