What are Pemphigus and Pemphigoid?

Pemphigus and pemphigoid are neither contagious nor inherited. They are diagnostic terms that include a range of rare and specific autoimmune diseases that affect people in different ways, but the common thread is that they result in blisters somewhere in or on the body. Pemphigus vulgaris (PV) which is the more common of the Pemphigus diseases usually affects both the skin and mucous membranes for example.

Our immune system is vital to protect us from infections, but in an autoimmune disease, the immune system malfunctions and attacks our own body instead. The mistake made by the immune system in these blistering diseases is that it regards the cells in our skin and mucous membranes as ‘foreign’ and creates antibodies, called ‘auto-antibodies’ to fight them.

Blistering is often accompanied by pain, itching, burning, and stinging. In one form of pemphigoid, mucous membrane pemphigoid (MMP) it may also result in scarring. In extreme cases of PV, the blistering can lead to life-threatening fluid loss and infection, or damage or blockage to important mucosa such as eyes or airways. PV can also cause significant damage to the skin, including nail loss and changes in pigmentation. Timely interventions are essential, although early diagnosis is often a problem due to the lack of experience of the medical professional treating the symptoms. The site of the initial disease means that patients may be treated by Dentists, Ear Nose and Throat specialists or Ophthamologists. Correct diagnosis requires a biopsy.

Pemphigus and Pemphigoid are chronic illnesses that rarely improve without active treatment, and, although they cannot be cured, they can by brought into remission. Treatment usually involves an initial phase to bring the blistering under control, followed by maintenance using the most effective and least toxic treatment regime. Complete remission or future flare ups are both possible.

With patience and effective treatment, people suffering with Pemphigus and Pemphigoid can lead normal and pain free lives.

 

What is the difference between Pemphigus and Pemphigoid?

This is a good explanation of the difference between Pemphigus and Pemphigoid.

This information is courtesy of Dr. Kristine Krafts at the University of Minnesota School of Medicine. It is re-produced from the International Pemphigus and Pemphigoid Federation at http://www.pemphigus.org

Pemphigus

Pemphigus

“Pemphigus” is used in a very specific way to describe blistering disorders caused by autoantibodies against some part of the epidermis, which lead to disruption of the proteins that hold the cells together (intercellular junctions) and hence bullae or blisters develop. Pemphigus vulgaris, not surprisingly, is the most frequent type of pemphigus (“vulgar-” comes from the Latin vulgaris, meaning common). It occurs primarily in adults between the ages of 30 and 60, and is characterized by, flaccid bullae that burst easily (in most patients, you’ll see more ruptured, scab-covered bullae than intact ones). Patients often present first with oral bullae and ulcerations, and later develop bullae on the skin.

In this disease, patients have autoantibodies against desmogleins, which disrupt the connections between the squamous cells of the epidermis and cause very superficial, intraepidermal, fragile blisters (or bullae). If you do immunofluorescence on the skin, you’ll see a kind of outlining of each individual epidermal cell (because there are autoantibodies bound to the junctions between the cells). Treatment consists of immunosuppressive agents; prognosis is variable, but many patients have a higher than normal mortality rate.

Pemphigoid

Pemphigoid

In pemphigoid, patients have autoantibodies – but they are against the basement membrane of the epidermis, not against epidermal cell junctions. This means that the bullae are actually subepidermal (that is between the outer layer (epidermis) and next layer of the skin (the dermis), so they are less fragile than those of pemphigus vulgaris (if you see a patient with bullous pemphigoid, you’ll see lots of intact, tense bullae, rather than a bunch of ruptured bullae covered with scabs). The immunofluorescence pattern is correspondingly different – you’ll see just a line at the base of the epidermis (rather than the lace-like outlining of epidermal cells you see in pemphigus vulgaris).