Last reviewed 05/2021
This is a group of conditions that are characterized by the formation of blisters within the epidermis of both skin and mucous membranes. Current evidence suggests that there is an autoimmune basis (1). Blisters are formed when autoantibodies target the cell surface antigens (desmoglein 3 & 1) on keratinocytes which results in loss of cell to cell adhesion properties and separation from one another (2).
The annual incidence worldwide is 0.1 - 0.5 per 100 000. It is slightly more higher in women, and in Asians and Jews. Peak onset is between 60 and 70 years of age.
The disease diagnosis is done on the basis of ¢ clinical manifestations - flaccid blisters and erosions on skin and oral mucosa
- histology - epidermal acantholysis
- immunological abnormalities - keratinocyte surface antigens antibodies (3)
In comparison, bullous pemphigoid is characterised by blister formation at the level of the basement membrane and not within the epidermis.
- 1. Langan S.M.et al. Bullous pemphigoid and pemphigus vulgaris - incidence and mortality in the UK: population based cohort study. BMJ 2008; 337:a180
- 2. Stanley J.R, Amagai M. Pemphigus, Bullous Impetigo, and the Staphylococcal Scalded-Skin Syndrome. NEJM 2006; 355:1800-1810
- 3. Bystryn J, Rudolph J.L. Pemphigus. The Lancet 2005; 336(9479): 61-73