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Pemphigus in South Africa
J Aboobaker1, N Morar, P K Ramdial
1Dermatology Department and Anatomical Pathology, Faculty of Medicine, University of Natal and King Edward VIII Hospital, Congella, South Africa.
International Journal of Dermatology
|May 1, 2001
Summary
Pemphigus foliaceus is more common in Black individuals, while Pemphigus vulgaris predominantly affects Indians in South Africa. Genetic factors may influence pemphigus presentation and severity.
Area of Science:
- Dermatology
- Autoimmune Diseases
- Genetics
Background:
- Pemphigus is a severe autoimmune blistering disease affecting the epidermis.
- Understanding pemphigus epidemiology and characteristics is crucial for patient management.
- Kwa-Zulu Natal, South Africa, presents a unique demographic for studying pemphigus.
Purpose of the Study:
- To characterize the demography, clinical presentation, and human leukocyte antigen (HLA) associations of pemphigus in Kwa-Zulu Natal.
- To compare features of pemphigus foliaceus (PF) and pemphigus vulgaris (PV) in this population.
- To investigate potential genetic links in pemphigus subtypes.
Main Methods:
- Prospective recruitment of 112 pemphigus patients over 12 years (1987-1999).
- Collection of demographic, clinical, histological, and immunofluorescence data.
- HLA typing performed on a subset of patients.
Main Results:
- Pemphigus foliaceus (PF) was the most common type (62 patients), predominantly affecting Black individuals (80%).
- Pemphigus vulgaris (PV) affected 50 patients, primarily of Indian descent (82%), with oral lesions.
- Mortality was 14%; HLA-B8 was significantly associated with PF (41%, P < 0.001).
Conclusions:
- PF is more prevalent in Black populations, and PV in Indian populations in this region.
- Pemphigus patients exhibited severe, extensive disease.
- Clinical similarities in PV patients to those in India suggest a genetic predisposition.