Pemphigus Vulgaris

Dr. Wendy Levinbook, MD discusses Pemphigus Vulgaris. See more at http://www.dermnet.com PLEASE RATE AND COMMENT!!!
Pemphigus vulgaris (PV) is the most common form of pemphigus in North America and Europe. The mean age of onset is 50 to 60 years of age and it affects both sexes equally. It is more common in

Jews and in people of Mediterranean descent.




Clinically, mucous membranes are always involved. Patients typically present with painful oral mucous membrane erosions although other mucous

membranes such as the pharynx, larynx, esophagus, conjunctiva, anus, penis, vagina, and labia may be involved. Cutaneous involvement is variable. The primary lesion is a non-inflamed, flaccid

bullae. However, intact bullae are found infrequently because the vesicle roof consists of only a thin portion of epidermis that breaks easily. Therefore, the most common mucocutaneous lesions

are erosions subsequent to ruptured bullae. These lesions are typically painful, not pruritic and they are often large, due to their tendency to spread peripherally. Lesions are positive for

Nikolsky's sign (lateral pressure of unblistered skin at the periphery of active lesions causes the epidermis to shear off) and for Asboe Hansen's sign (pressure to the top of a bullae causes

extension of the blister to adjacent unblistered skin).




PV is an intraepidermal autoimmune blistering disorder that occurs secondary to the formation of antibodies that interact with desmoglein

3. This is a 130 kDa transmembrane glycoprotein of the desmosomal junction. Binding of IgG autoantibodies to desmoglein 3 causes acantholysis by interfering with the adhesion of adjacent

desmogleins, without the participation of other inflammatory events. Histopathologic exam demonstrates suprabasilar bullae with acantholysis. The basal cells remain fixed to the basement

membrane but may lose contact with adjacent cells thus giving the appearance of a row of tombstones. The upper epidermal cells usually remain intact.




The hallmark of PV is the finding of IgG

autoantibodies in the epidermis. Direct immunofluorescence of perilesional skin is positive for intercellular IgG in virtually all patients with active PV. Indirect immunofluorescence is

positive in about 75% of patients and titers tend to correlate well, although imprecisely, with disease activity. Therefore, it is more important to follow clinical disease activity than antibody

titers in the daily management of patients.

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• Pemphigus
• Vulgaris

[hotathlete2]

@5o5atoon Your mother's had this disease for 6 years? I was diagnosed with this disease in june 2011. I began at 80mgs of prednisone and am currently down to 20mgs. I also went thru chemotherapy with rituxamab for 2 sessions. Most of my symptoms are gone. I live in los angeles, I've been getting treated at Harbor UCLA hospital in torrance california. I've come a long way from my initial diagnoses. Let me know if there's anything else I can do.

[5o5atoon]

@hotathlete2 My mother infected with the disease .. more than six years and is still.. What was the method of treatment? .. and what's the name of the hospital where you received treatment?

I would be grateful if you gave me all information about the treatment of this disease 

[Lostlove777]

I have this disease for over 1 year, I took Prednione for first 3 month from 60mg then gradually down to 10mg along with 1000mg Cellcept., the condition seem went away. Then just 1000mg Cellcept after that to keep it from coming back. My doctor told me that Cellcept is much safer and less side effects. Very important, Cellcept must be taken on empty stomach, and one must avoid prolong sunlight.

[hotathlete2]

I've been diagnosed with this disease, although my worst symptoms are the mouth ulcers and tongue lesions. Thank God, the nikolsky sign sores are almost all gone. I've been under treatment with prednisone for the past 2 months and it appears to be working. I'm working on re-establishing my body's coordination and my muscles again. If anyone else has this rare autoimmuned disease, I'd appreciate some feedback. Perhaps we can share our treatment information.

[anseryt]

GREAT presentation.Thanks

[wellnessrealm]

Thank you for this video.