PV is rare and most general practitioners (GPs) have never seen it. So it is essential that the diagnosis of PV is made by a dermatologist (a hospital doctor specialising in skin conditions) or another medical professional who is a specialist in dealing with PV.

Getting an early diagnosis can increase your chances of getting successful treatment before it becomes too severe. Early diagnosis is important because the sooner you get treated the sooner you can start to feel more comfortable.

If the doctor thinks you might have PV, there are several tests that can help them decide:

• A visual examination. This is where a dermatologist will have a look at your skin and mucous membranes and give you an overall examination.
• A biopsy. This is where a sample of skin is taken from an erosion or blister after first making the skin numb with an injection of local anaesthetic. Two tests will be done on this skin sample:
  • The sample will be looked at under the microscope in a laboratory to see if the skin cells are separated from each other (see ‘What does pemphigus vulgaris look like?’ and ‘What causes pemphigus vulgaris?’).
  • Direct immunofluorescence. The sample is treated in the laboratory to see if the PV autoantibodies are present (see‘ What does pemphigus vulgaris look like?’).
• Another test called indirect immunofluorescence is usually done (sometimes called an antibody titre test). This test measures PV autoantibody levels in the blood (see ‘What causes pemphigus vulgaris?’). This test is useful for monitoring how active the PV is. It helps the doctor to work out if changes in your treatment and therapy are needed.
• The most accurate test to measure PV autoantibodies in the blood is called the ELISA test, but this is not available everywhere ¹⁵.

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