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Pemphigus Vulgaris

Background

Pemphigus vulgaris (PV) is a condition which causes blistering of the outer layer of the skin (epidermis) and mucosal membranes, including the nose and throat. It is a very rare condition and occurs most commonly in middle-aged or older people of all races and ethnic groups. Because PV is very rare, diagnosis may be difficult or delayed. It is one of the three main types of pemphigus, the other two forms being pemphigus foliaceus and pemphigus paraneoplastic.

Pemphigus foliaceus (PF)
In Pemphigus Foliaceus, blisters and sores do not occur in the mouth. Crusted sores or fragile blisters usually first appear on the face and scalp and later involve the chest and back. The blisters are superficial and often itchy, but are not usually as painful as PV. In PF, disfiguring skin lesions can occur, but the mortality (death) rate from the disease is lower than in PV.

Paraneoplastic pemphigus (PNP)
PNP is potentially the most serious form of pemphigus. It occurs most often in someone who has already been diagnosed with cancer. Fortunately, it is also the least common.

Painful sores of the mouth, lips and oesophagus are almost always present; and skin lesions of different types occur. In some cases, the diagnosis of the disease will prompt doctors to search for a hidden tumour. In some cases the tumour will be benign and the disease will improve if the tumour is surgically removed. It is important to know that this condition is rare and looks different than the other forms of pemphigus. The antibodies in the blood in somebody with PNP are also different from other forms of pemphigus and the difference can be determined by laboratory tests.

Credits

Medical text written September 2002 written by Contact a Family. Approved September 2002 by Professor M Black, Consultant Dermatologist, St John's Institute of Dermatology, Guy's, King's and St. Thomas' School of Medicine, London, UK. Last updated June 2010 by Dr Richard Groves, Consultant Dermatologist, St John's Institute of Dermatology, Kings College London, London, UK.

What are the symptoms?

PV causes the skin to separate easily and peel easily. For many individuals, PV usually begins with blistering in the mouth and throat. This may be followed by blistering or erosions of the skin, including the groin, underarm, face, scalp and chest areas. PV lesions may cover extensive portions of the body. In some affected people the lesions are relatively asymptomatic. However, in the healing stage following treatment, the lesions often crust over but they no longer itch or burn and leak fluid. In some people the skin lesions may itch and burn continuously and rupture which may leave red erosions of the skin surrounded by a crust and scaling. Affected areas usually heal without scarring, unless the lesions become infected. Blisters in the mouth may make it difficult to eat and drink, leading to problems with weight loss and dehydration.

What are the causes?

PV is one of a group of chronic, relapsing conditions in which the immune system produces antibodies against specific proteins in the skin and mucous membrane, leading to the inability of the skin cells to bind together. It is thought that PV may be triggered by a range of factors. A few cases of PV have occurred following reactions to medications, including penicillamine and captopril. Individuals are probably genetically predisposed to PV. Specific information about a gene change(s) will not, however, indicate the certainty with which an individual will become affected with PV in the future.

How is it diagnosed?

Diagnosis of these diseases is made by skin biopsy (where a small amount of skin is removed for study in a laboratory) and immunopathology (analysis of body fliuds to detect if there are any antibodies attaking the proteins in the skin and mucous membranes).

How is it treated?

There is no cure available for PV but in most cases available treatments are highly successful in reducing symptoms and preventing complications. PV is often initially controlled with high-dose steroids together with one of a number of so-called 'steroid sparing' immunosuppressive drugs. Response to medication varies from individual to individual and, for some people, lesions may not heal for extended periods of time. The main risk from PV is infection and complications resulting from medication aimed at suppressing the immune system. Consequently doses are kept as low as possible, consistent with controlling symptoms.

Inheritance patterns and prenatal diagnosis

Inheritance patterns
None detectable but familial cases occur very rarely.

Prenatal diagnosis
None available and not appropriate.

Is there support?

Pemphigus Vulgaris Network

Helpline: 0208 863 3735
www.pemphigus.org.uk

The Network is a support group for people living with Pemphigus and Mucous Membrane Pemphigoid. The helpline offers information and support to affected individuals, their families and carers, and medical professionals. 

Group details last updated October 2015.

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