Re-produced in part, from the British Association of Dermatologists Guidelines http://www.bad.org.uk/for-the-public/patient-information-leaflets/pemphigus-vulgaris#.VDKkdRbfITA (these will be rewritten in 2014) and from the IPPF. Further information can be obtained from these and other sources.
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.
What causes pemphigus vulgaris?
- The auto antibodies in pemphigus vulgaris target proteins called desmogleins, which are present on the cells in the outer layer of the skin (the epidermis). Desmogleins act as a glue to stick these cells together.
- When the auto antibodies formed in pemphigus vulgaris combine with the desmogleins, the cells in the skin and mucous membranes no longer stick together properly and fall apart. This causes the blisters and erosions that are typical of pemphigus vulgaris.
- Although it is known that antibodies to desmoglein cause pemphigus vulgaris, it is still not clear why some people develop these auto antibodies in the first place.
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. Pemphigus vulgaris is not an infection: it cannot be caught or passed on to anyone else.
Is pemphigus vulgaris hereditary?
Strictly speaking it is not, as it does not pass from generation to generation. Genetic factors are important, but other factors are needed to trigger pemphigus vulgaris into activity.
What are the symptoms of pemphigus vulgaris?
The raw unhealing erosions are painful and can disturb sleep. Those in the mouth can interfere with eating and drinking and can lead to weight loss, hence the advice of the dentist would help especially those with dentures. It can also affect other mucous membranes such as the genitalia (causing painful intercourse) and the conjunctiva of the eyes (in which case the help of an ophthalmologist would be useful).
What does pemphigus vulgaris look like?
In most patients, the blisters and erosions start first in the mouth, and appear later on the skin. In a few, the skin is affected first.
Other sites affected include the conjunctiva, oesophagus, labia, vagina, cervix, penis, urethra and anus. Most patients get erosions in their mouth at some time; but some never get blisters or erosions on their skin.
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 may develop into vegetative lesions which are granular and crusty looking (known as pemphigus vegetans).
Will pemphigus vulgaris go away?
No, it will not clear without treatment. There will be times when it flares up, and others when it gets better. There is no way of knowing when these flare-ups will happen or how bad they will be. However, pemphigus vulgaris can be controlled by long-term treatment which may eventually result in no further flare ups.
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 fetal death.