What are the signs and symptoms of PV?

PV blisters typically start in the mouth and may also spread to the skin after several months

The first sign of PV is typically when the mouth and lips become hot and swollen due to blisters developing in the mouth. These blisters are very fragile and easily burst causing painful sores (lesions). The lesions can take weeks or months to heal.1,2 As the mouth becomes sore, eating and drinking may become uncomfortable and you may become hoarse if the blisters spread to the larynx (voice box).

If you have PV, you may only get blisters and lesions in the mouth or blisters may also start to develop on the skin after a few months.1 Blisters on the skin are fragile and often painful, but are not usually itchy.1 Skin blisters typically occur on the face, scalp, chest and between the shoulder blades.1 The areas of raw exposed skin caused by the lesions will eventually scab over leaving skin dry and crusted.2 This can sometimes leave the skin discolored once the lesions have healed. 

pemphigus vulgaris symptoms

Images reproduced with permission from Medscape Drugs & Diseases (https://emedicine.medscape.com/), Pemphigus Vulgaris, 2018, available at: https://emedicine.medscape.com/article/1064187-overview.

Other areas of the body that may be affected include the lining of the nose, throat and genitals and also the inner eyelid, known as the conjunctiva.2 The symptoms of PV can be upsetting and the lesions on exposed body parts may have a negative impact on your self-esteem.  

Typically affected areas

pv skin disease

As a chronic condition, PV has periods when the disease is active (flares) or inactive (remission), when the affected areas of the body heal.1 PV blisters are an infection risk and if PV is not treated it can become more severe resulting in multiple lesions, further increasing the risk of infection.1 It is important to diagnose PV early so that appropriate treatment can be provided, which will minimize the impact of PV symptoms.

What does it feel like to live with PV?

PV Patient Interview Part 1: From first symptoms to diagnosis

PV Patient Interview Part 2: Burden of disease and treatment

References

  1. Hertl M, et al. J Eur Acad Dermatol Venereol. 2015; 29:405-414.
  2. Kasperkiewicz M, et al. Nat Rev Dis Primers. 2017; 3:17026.