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Paraneoplastic Pemphigus

Sometimes also known as paraneoplastic autoimmune multiorgan syndrome (PAMS).


This page covers the following:

Introduction

Paraneoplastic pemphigus (PNP) is almost always associated with an underlying malignancy, most often a lymphatic disorder such as non-Hodgkin Lymphoma or Chronic Lymphocytic Lymphoma. It is very rare, although the incidence of the disease isn’t known. It seems to affect men or women between 40 and 70 but can affect anyone at any age, including children.


PNP can indicate an undiagnosed malignancy or follow a diagnosis.


What are the symptoms of PNP?

PNP can cause very painful blistering, inflammation and erosions on the lips and in the mouth and throat, making it almost impossible to eat or swallow. This latter can result in dehydration and malnutrition. It can also affect other mucous membranes such as the genitals.


Blisters on the skin can be very painful, as with pemphigus generally and these can also be very itchy.


PNP is usually accompanied by muscle weakness and severe fatigue as well as the involvement of other organs and internal body linings such as the gut and lungs. Exposed lesions can also result in further dehydration and a predisposition to infections. If the eyes are affected, conjunctivitis and even ulceration of the cornea can occur.


There are five subtypes of PNP, probably connected to the different autoantibodies created, and these will all have variations on the symptoms which appear.


Subtypes are:


What are the further complications of PNP?

A serious complication of PNP can be Bronchiolitis Obliterans, which is very hard to treat and can result in respiratory failure.


What caused my PNP?

The causes and processes at play in the development of PNP are not well understood but it is believed that the underlying cancer disrupts normal immune functions. The immune system appears to react in the same way as that involved in pemphigus, generating autoantibodies which damage proteins (desmosones) in the upper layers of the skin and, particularly, in the mucous membranes. These desmosones are the “glue” that hold layers of the skin together. The autoantibodies may also attack proteins (hemidesmasones) which occur at deeper levels of the skin, in the same way as occurs in bullous pemphigoid.


There may be a genetic factor involved in a predisposition to PNP.

 

How is PNP diagnosed?

PNP is diagnosed based on a combination of clinical findings, biopsies and blood samples. In the laboratory, these are tested using histopathology, direct and indirect immunofluorescence and immune serology.


Once PNP is diagnosed, a thorough search for any underlying malignancy if undetected previously, is crucial.


How is PNP treated?

You are likely to have a multi-disciplinary medical team who manage your treatment. The first part of the treatment plan will be to manage the underlying malignancy whilst keeping the symptoms of the PNP under control.


There will be an extensive and complex care management plan as well as the probable use of corticosteroids and immunosuppressive medicines.


Rituximab, which is a biological treatment, is often used to reduce your immune system activity.


What are the risks of PNP?

Treatment with a broad range of therapies may improve the outcomes for people with PNP. However, only a small percentage of patients survive PNP.


Where can I find more information about PNP?

The Primary Care Dermatology Society have information about PNP. You can also read more about PNP on DermNetNZ.

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