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 the connections between cells of the epidermis.
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 are part of the spot desmosomes (intercellular junctions that connect cells of the stratum spinosum with each other). The antibodies mess up the desmosomes, disrupting connections between the squamous cells of the epidermis and causing 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! In pemphigus, there are autoantibodies against the connections between epidermal cells. But in bullous pemphigoid, the are directed against hemidesmosomes (specialized intercellular junctions that attach epithelial cells to the basement membrane).
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).
Bottom line: 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
Wow thank you so much! I could not figure it out at all until I read this!
Thank u so so so much. ive been a dentist for 4 years now and this is the first time i can really say that i will not get confused anymore… the best and easiest explanation
I’m so glad you found it helpful!!
Nice and instructive information.Please do add about significance of DSG3 antibodies ELISA titre values in diagnosis and monitoring course-prognosis and predicting relapses or permanent remmission.
Thank you! Thanks for mentioning the DSG3 antibody – can you elaborate on how you use that?
I was diagnosed with Mucous Membrane Pemphigoid about two years ago. The diagnosis took over a year and visits to several different specialists. My physician has treated me with steroids, and my dosage has been decreased as my occurrences have decreased. No one seems to know much about this autoimmune disorder, least of all what causes it. I’ve been having flare ups over the last month, which is upsetting. Does anyone know of any medications that may be effective in the treatment of this painful disorder?
I’m sorry to hear about your diagnosis! It sounds very difficult. I don’t know of any drugs except steroids – but I’m just a pathologist – I’m sure a good clinician would know what your options are…
Cool post on Bullous disorders!!!!!!!! Thanks a lot for sharing 🙂
Staphylococcal Scalded Skin Syndrome, has similar pathology to Pemphigus, the difference being in DSG3 is destroyed by auto-antibodies in pemphigus, while Staph’s exotoxins (SSST) destroy DSG3 in SSSS
I remember that itchiness is part of the symptomatology of Pemphigus & Bullous Pemphigoid,
I wonder why you have itching in these bullous disorders.
hmmm, itching could be related to histamine release,…
Wonder how is histamine released when DSG3 is destroyed by antibodies
Whew…great question!
After 7 months of taking my very young 70 yr old mom to specialists everywhere for a large “wound” on her head……& after a hospitalization where she was dosed with major IV antibiotics……my Mom’s eyes swelled. I took her to several doctors…..the stories and diagnosis from Dr. To Dr. were torchering & worrisome. Scar tissue developed in her eyes. Meanwhile, I kept having to take my mom to have her esophagus stretched (dilated), as strictures kept coming back. Finally, a Corneal Specialist here in Syracuse, NY recognized that the wound on her head…..& the 5 esophageal stricture dilitation procedures in the past 15 months might be related….he did a biopsy of scar tissue in Mom’s right eye; he agreed there is strong evidence for MMP. He then sent us to Boston, MA, where she consulted with Dr. Stephen Foster, founder of MERSI (Massachusetts Eye Research Surgery Institute); Dr. Foster diagnosed mom within a 2-hour period with MMP.
Though glad we understand what has been happening to her health is now diagnosed, many doctors did not tie together many details—-she is rapidly losing her vision; surgery to remove scar tissue is risky because the Pemphagoid is active.
Dr. Foster was very articulate regarding how aggressive the MMP activity is at this time, in my Mom’s case. To not seek aggressive therapy, will only lead to blindness in a very short time. She is soon to start infusion therapy in Rochester, NY, starting with 2 infusion sessions, 2 weeks apart to attempt to get the MMP into remission. The infusion medication is Retuxemab.
Other than the drug website side effects info., I am not finding much research on the effectiveness of this drug for MMP. Can anyone share information or experiences??
Looking forward to shared information. Thank you for your time.
— Valerie
Such a great explanation!
I have continued to forget what the difference is between pemphigoid and pemphigus. Things are so much easier to remember if you actually understand WHY or how they work and you explain it all in such a straightforward way,
Thanks : )
Thanks Lucy!! So glad it helped. I totally agree: if you understand WHY then you don’t have to memorize seemingly random stuff.
“Pemphigus is serious, but pemphigoid you can’t avoid” – Pemphigus is linked to high mortality rates but pemphigoid can cause blindness and is linked to significant morbidity (not mortality though)
This is excellent! Thank you so much!
Very nice explanation…. Thxxx
Very nice explanation ,it mas usefull
I have had symptoms off and on for about 12 years starting when I was 24. I have been to many different physicians (GP, dermatologists and a dermatologist/immunologist) and despite cultures and biopsies no one could make a positive diagnosis.
I currently have a very painful flare up so I went to a new dermatologist in my area – he took one look at my blisters and thought BP or PV immediately. I will have the immunoflorescence (sp?) test done by the end of the week.
After reading up on the disorder, and the experiences of others already diagnosed, for the first time I think I may understand what is happening to me.
The treatment and the prognosis of BP and PV are a real concern but I am more than ready to move forward should I receive the BP or PV diagnosis. Thank you to all who contributed to this forum.
I was diagnosed with BP from my dermatologist when my biopsy came back. I’m now on 40 mg a day of prednisone for 30 days and a tropical crème called something starting with a “T”. Its a long name. and the itching has stopped. I’m on my 12th day. Its clearing just a little but most of it is on my arms, buttocks, and stomach with a little on the legs. My question is will the ugly redness and blotches ever go away and my skin regain its normal coloring again. Its very ugly and being so hot outside its very hard this time of the year to always wear long sleeves to work to cover it up. I have not told anyone at work about this because we don’t have close relationships outside of just work related. Nobody has asked me any questions about my arms reddish blotches that are VERY noticeable. Please! anyone with any advice or experiencing the same issues please write me back and let me know if this will go away and my normal skin color will return. It has been a horrible experience. I’m 57 years old and have never had any skin issues before this occurred. HELP!
Thank you for a great explanation of PV vs BP. I was diagnosed with PV in 2011 after a brief hospitalization. I have been able to maintain at 10 mg prednisine and 3000 mg micophenylate but have had to do prednisone “surges” every three or 4 months. The disease seems to be creeping up…I have new areas of involvement but so far limited to mucous membrane. It has definitely been life-changing for me!
I am so sorry to hear about your diagnosis – sounds like a really horrible thing 🙁 Maybe someone who has BP will write in with some helpful information. I don’t have it – and am not that knowledgeable about the clinical course – so I hope you can get more information. Is your doctor helpful in this regard? Sending you good thoughts and wishes. -Kristine.
Hi Jennifer – so sorry to hear about your disease 🙁 Seems there are quite a few patients with either disease that have left comments here. I hope you are able to get it under control with drugs – what a difficult thing it must be. My thoughts are with you! -Kristine
Got PV at the tail end of 2011, had thrush and couldn’t even swallow water, hair loss, had to brush my teeth with a Q-Tip, and many other awful things, like a bad science fiction movie. Had to keep working thru it all, had people answering my business calls because i had no voice, bloodshot ruby red eyes, and children screamed when they saw me. My lips lost their “glue” and were coming apart at the seams, all oozy and sticky, lipstick would literally fall off. had to sleep on cotton, had so many oozing bloody burn-like leasions, SO started 60 mg of pred, and 3000 mg of celcept in the morn and 2000 in the evening. now July 24, 2014, like 2 yrs and 8 mos into it, i am on 5mg pred and same celcept, but just have tiny leasions in my mouth, nothing else. Don’t know if that helps anyone, hope it does.
i have been diagnosed for bp since i was 19.now im 23yo.at earlier, the symptoms are more active,my blister can came out more frequently, very painful and more to my lower limb n joint part.its more like blister with ?water and very large.so i was given steroid in the beginning for treatment and luckly it’s resolving.
i have no more follow up for now, latest is on early 2014 because i actually discover my disease can be worsen when i got stress and very cold since now i work at hospital as a nurse (full aircnd). i really have a hard time to adjust my stress and body in new environment.
now its only occur when i finish my night shift but only 1 or 2 small2 haemoragic bullae which can heal fast if i have my rest properly.not on any medication. my doctor said bp actually can cure after 5 or 6 years.i meant it went to passive phase.
amazing explanation. well put