Pemphigus vulgaris is an autoimmune condition that causes blistering of the skin and mucous membranes.1 Pemphigus vulgaris can be life-threatening.
Pemphigus vulgaris has an increased incidence in Ashkenazi Jews and those of Mediterranean origin, but otherwise has an estimated incidence of 0.5-3.2 cases per 100,000 people.2
Pemphigus vulgaris can affect any age group, with the average age of onset 50-60, however it has appeared in children and the elderly.3
Symptoms of pemphigus vulgaris4
- Soft blister with clear fluid inside
- Affects the mouth
- Slow healing erosions in the mouth
- May affect the vulva, labia, vagina, cervix, oesophagus, conjunctiva, urethra, and anus
Causes of pemphigus vulgaris
The cause is unknown, however the circulating antibodies are directed at the cell surface of keratinocytes. There are several forms of pemphigus – vulgaris, foliaceus and paraneoplastic. Over 70 per cent of pemphigus cases are vulgaris. It can be triggered by some medication.5
Treatment of pemphigus vulgaris
Typical treatment is steroids and immunosuppressants. The point of treatment is to reduce the inflammatory response and autoantibody production.6
References
- 1.Cholera M, Chainani-Wu N. Management of Pemphigus Vulgaris. Adv Ther. Published online June 2016:910-958. doi:10.1007/s12325-016-0343-4
- 2.Vodo D, Sarig O, Sprecher E. The Genetics of Pemphigus Vulgaris. Front Med. Published online August 14, 2018. doi:10.3389/fmed.2018.00226
- 3.Hübner F, Recke A, Zillikens D, Linder R, Schmidt E. Prevalence and Age Distribution of Pemphigus and Pemphigoid Diseases in Germany. Journal of Investigative Dermatology. Published online December 2016:2495-2498. doi:10.1016/j.jid.2016.07.013
- 4.Robati RM, Rahmati-Roodsari M, Dabir-Moghaddam P, et al. Mucosal manifestations of pemphigus vulgaris in ear, nose, and throat; before and after treatment. Journal of the American Academy of Dermatology. Published online December 2012:e249-e252. doi:10.1016/j.jaad.2011.06.022
- 5.Tavakolpour S. Pemphigus trigger factors: special focus on pemphigus vulgaris and pemphigus foliaceus. Arch Dermatol Res. Published online November 6, 2017:95-106. doi:10.1007/s00403-017-1790-8
- 6.Gregoriou S, Efthymiou O, Stefanaki C, Rigopoulos D. Management of pemphigus vulgaris: challenges and solutions. CCID. Published online October 2015:521. doi:10.2147/ccid.s75908
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