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.
Click here for an example image of this condition
Management principles:
- a review suggests systemic glucocorticoids as first line-treatment for all forms of pemphigus vulvaris (PV), with or without adjuvant therapies (4):
- for mild PF, topical steroids, systemic steroids, dapsone, and rituximab are possible first-line treatments
- in a secondary analysis of the Ritux 3 trail, first-line treatment of patients with pemphigus with the Ritux 3 regimen (rituximab) was associated with long-term sustained complete remission without corticosteroid therapy without any additional maintenance infusion of rituximab (5)
Reference:
- 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
- 4. Malik AM, Tupchong S, Huang S, Are A, Hsu S, Motaparthi K. An Updated Review of Pemphigus Diseases. Medicina (Kaunas). 2021 Oct 9;57(10):1080.
- 5. Tedbirt B, Maho-Vaillant M, Houivet E, et al. Sustained Remission Without Corticosteroids Among Patients With Pemphigus Who Had Rituximab as First-Line Therapy: Follow-Up of the Ritux 3 Trial. JAMA Dermatol. Published online January 24, 2024. doi:10.1001/jamadermatol.2023.5679