Here are two diseases that are easy to confuse: pemphigus vulgaris and bullous pemphigoid. Both diseases are characterized by bullae (big, blister-like skin lesions) and both have “pemphig” in their names (“pemphig-” comes from the Greek pemphix, meaning blister, so that makes sense). So what are the differences between the two?
Pemphigus vulgaris
Here’s the key to differentiating between the two disorders. “Pemphigus” is used in a very specific way (you’d think it would be used to describe any blistering disorder, but not so!). It is used to describe blistering disorders caused by autoantibodies against some part of the epidermis, which lead to disruption of the intercellular junctions (and hence bullae). Pemphigus vulgaris, not surprisingly, is the most common type of pemphigus (“vulgar-” comes from the Latin vulgaris, meaning the general public). It occurs primarily in adults between the ages of 30 and 60, and is characterized by big, 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 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.
Bullous pemphigoid
This disease is called pemphigoid rather than pemphigus, because it looks like pemphigus but really isn’t! Pemphigus is characterized by autoantibodies against the connections between epidermal cells. In bullous 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, 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).
Patients with bullous pemphigoid are generally elderly, and the clinical presentation varies a lot (but usually it doesn’t start in the mouth, like pemphigus vulgaris). It’s a less serious disease, usually, since the bullae often don’t rupture (so there’s less chance of infection and scarring).
So if you can remember that pemphigus is a disease that has intraepidermal antibodies, then you can keep the clinical presentation and immunofluorescence pattern of the two diseases straight.
fantastic explanation
AT LAST! At last someone has helped me to get it through my head properly. Many thanks!
Awesome explanation. You made it all make sense. Thanks
was in your dental student class 2 years ago, loved it and still seem to find my way back to the blog!
Excellent explanation in simple words.
My mother was diagnosed after mutiple trips back and forth to th E.D. and trips to the dermatologist. It started in July and she was not diagnosed until Sept. I had to request a biopsy, because they kept treating it as a generalized rash. Her diagnosis was bullous pemphigiod, but after reading your article, she had all the symptoms of vulgaris pemphigoid.
Thanks for the explanation.
Thank you very much, finally a clear explanation!!
My partner has been diagnosed : BP
Is it ehh… Do I have to worry, can I get it too ?
No…it’s not contagious.
simplified and enlightening
Thank verymuch for clear explanation
I spent 12 days in ICU at the Chessapeake General hosp in VA. I was bombarded with antibiotics, biopsys,etc. and many side effects due to the meds, including low heart beat(medicated for that),they could not determine what my condition was.Several Drs involved ,(Dermatologist, Cardiologists etc). I finally insisted on being discharged because the condition had greatly improved, I had to sign a release. At no time during my stay was I told my condition and everytime I asked ,I was told by the Dermatologist “we dont know”.It was upon my outpatient visit to the Dermatologist that I said to him “you never did find out what was wrong with me!” Then he told me what it was, “Bullous Pemphigoid”.This was in April 2005. Thank God I have never had a relapse.Your article makes it clear, Thank you
Oh no! So sorry to hear about your ordeal. I’m glad you’re better and for 7 years have not had any relapse – that is great news. Best wishes to you!
Great explanation, didn’t know the pemphigOID was because it was like pemphigus.
A tip from a great registrar to try and keep them straight in my head was PemphiguS – Superficial, and PemphigoiD – Deep
I was biopsied with BP in 2007 at age 67. After many bad tries, UofMDerm.put me on Niacinimide,doxycycline w/ clobetasol and triamcinolone, went into remission after about 4 months. Minor toe surgery seemed to activate it again
on March 10,2012. This time it is severe on the thick pads of
my hands and feet to the point of poor sleep. Using the same treatment as poor.
I’m so sorry to hear of your disease recurrence. I hope that the same treatment will bring it under control, and quickly.
thx so much for such A POWERFUL EXPLANATION… I AM SO CLEAR OF ALL DOUBTS
I have just ben diagnosed with BP, after suffering on and off for about 7 years. Have been to a dermatologist and was treated with anti-biotics against a staph infection – to no avail of course.
I am now using a corticosteroid ointment – seems to help at present.
My mom is 86 yrs old, in very good health, but 4-5 yrs ago, after a year plus of biopsy, trying prednizone, antibiotics, not knowing what to do, a dermatologist found that prescribing Imeron, an immunosuppressant, helps her to cope with the blisters and it takes away the itch/sting! they try to lower her dose, but each time, she feels the itch back again.so far Imeron daily has kept her in a normal lifestyle!
I was diagnosed in 2001 with PV. It took several trips to the , Eye, Nose, Ear & Throat Dr. at UPMC in Pittsburgh, PA..Finally, I requested a biopsy. After finding out, I was referred to a Dermatologist at the same Hospital. I was on Prednisone 60mg a day, cellcept,tetracycline & finally Imuran. I suffered until 2009–finally went in remission but it only lasted 3 yrs. My best friends husband passed away unexpectantly 2 mths ago. After attending the funeral & burial, the following day in the same week, My PV started coming back & this time it is back with the vengence!! Wow, how easily I had forgotten about the pain in my mouth & blisters on my skin. Now back to square 1. This is a terrible disease. You just can’t take a vacation from PV—it’s around as long as it wants to be!!
Oh WOW. Thanks so much for sharing your story. It is always helpful to hear about a disease from a patient’s perspective – it gives a much better understanding of what patients have to go through. I’m so sorry to hear about your recurrence. Hopefully with medications you can get it back under control. Best wishes to you!
am69yearsold ! bp comesand goes ! Sometimes very paynfull! gums bleed alot steroid gell on gums helps , also use predzoneabout 30mg a day ! not a lot of fun.
So sorry to hear that! I hope you feel better soon.
the condition is hard to understand, but you have shed some light, am astudentTT
I was diagnosed in 2011