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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.

What are the symptoms? View What are the symptoms?

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.

 

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