Pemphigus vulgaris is a rare autoimmune disease (up to 3.2 cases per 100,000 population) that causes severe blistering of the skin and of the mucous membranes lining the mouth, nose, throat and genitals. Blisters are sacs with fluid that develop on the upper layer of the skin so their roofs are very thin and fragile and break easily to leave raw areas (erosions) that can be extensive and painful. Pemphigus does not go away by itself, and always needs treatment by a Dermatologist.
Pemphigus vulgaris affects both males and females equally. It can start at any age but is most common in adults usually between 50-60 years old. It affects people of all races but is more common in some than others, for example in people of Jewish origin.
In most patients, the blisters and erosions start first in the mouth, and appear later on the skin. In a few patients, the skin is affected first.
Other sites affected include the conjunctiva, oesophagus, labia, vagina, cervix, penis, urethra and anus.
The raw unhealing erosions of Pemphigus Vulgaris are painful and can disturb sleep. Those in the mouth can interfere with eating and drinking and can lead to weight loss. Most patients get erosions in their mouth at some time; but some never get blisters or erosions on their skin and an initial diagnosis often follows a trip to the Dentist. When it affects the conjunctiva of the eyes, an ophthalmologist should be consulted.
Sites often overlooked include around the nails (manifest as painful, red, and swollen), the pharynx and larynx (pain on swallowing and hoarseness), and the nasal cavity (nasal congestion and a bloody mucous discharge, particularly noticeable upon blowing the nose in the morning). As many as 49% of patients were shown to have laryngeal and nasal involvement.
50-70% of patients get oral lesions – common features of oral mucosal pemphigus include:
blistering superficial and often appears as erosions
widespread involvement in the mouth
painful and slow to heal
may spread to the larynx causing hoarseness when talking
may make it difficult to eat or drink
The skin lesions start as thin-walled blisters (collections of clear fluid within the skin), arising on a background of normal-looking skin. Because they are so fragile, pemphigus blisters break very easily, leaving raw areas known as erosions.
Erosions look raw and feel sore – like a burn. Erosions can join together to create larger areas of raw skin. Erosions can become crusty and scabbed. When they heal, those on the skin may leave discoloured marks.
Skin lesions appear as thin walled flaccid blisters filled with clear fluid that easily rupture causing painful erosions. Erosions in the skin folds occasionally develop into lesions which are granular and crusty looking (known as pemphigus vegetans).
Before the advent of corticosteroids, pemphigus had a high fatality rate, with approximately 70+% of patients dying within a year.
With treatment, lesions can heal normally without scarring and the hyperpigmentation associated with pemphigus often resolves after several months. Most patients treated for pemphigus will enter a partial remission within 2 to 5 years.
Pemphigus vulgaris (or foliaceus) may present or worsen during pregnancy, especially in the first and second trimesters, and is associated with an increased risk of premature birth and foetal death.
More information can be obtained from the British Association of Dermatologists Patient Leaflets and from the DermNetNZ.