Skip to main content

Pemphigus Vulgaris

Pemphigus vulgaris is a blistering autoimmune skin lesion mostly seen in elderly patients.The disorder is characterised by a process called Acanthoysis (loss of cohesion between epidermal cells).Lesions are mostly flaccid blisters over the skin.These blisters burst out and the surounding skin gets denuded.In severe cases, substantial portions of the body get denuded.Manual pressue over these blisters can cause separation of epidermis.This is known as Nikolsky's sign.This finding is a characteristic feature of Pemphigus vulgaris but not the specific feature. Nikolsky's sign is also seen in Stevens Johnson Syndrome, Toxic epidermal necrolysis and few other skin conditions.

Clinical Features:
Sometimes the disorder starts with pruritis(itching).Lesions in Pemphigus vulgaris are usually seen over the oral mucosa, scalp, axilla, trunk, face, neck.Blisters are usually seen in the mouth first and then on other mucosa (pharyngeal, laryngeal, oesophageal, conjunctival, vulval or rectal) in severe cases.Severe pain may be seen associated with extensive denudation of body surface area. Lesions usually heal without scarring except at sites complicated by secondary infections or with mechanical dermal wounds.Post inflammatory hyper pigmentation is usaually seen at sites of healed lesions for some time.

Diagnosis:
On biopsy, the lesions show acantholytic epidermal cells and intra epithelial vesicles.Basal keratocytes are attached to the basement membrane, hence blister formation takes place in suprabasal portion of epidermis.Eosinophil predominant leukocytosis is seen in these regions.
Direct immuofluoresence microscopy shows - IgG antibodies both in the lesional and normal skin.But in the lesional skin, there are compliment components which are absent in the normal skin.These IgGs are derived from autoantibodies directed against cell surface antigens on the epidermal cells.These autoantibodies are targeted against desmogleins (Dsgs, these are transmembrane desmosomal glycoprotiens that belong to cadherin supergene family of calcium dependent adhesion molecules).These autoantibodies can be demonstrated by indirect imunofluoresence microscopy or by ELISA.Anti Dsgs titers relate with the activity of the disease.Mostly anti Dsgs3 is seen in these patients but in severe cases, antiDsgs3 and anti Dsgs1 autoantibodies are seen.Anti Dsg antibodies are pathogenic for blister formation and their titers correspond with the disease activity.

Treatment:
Prior to availability of Glucocorticoids, the mortality rate was 60 to 90%. Now it has been reduced to 5%.Prednisolone 60 to 80 mg/day is given. If new blister appear after 1 or 2 weeks of treatment, the dose must be increased. Immunosuppressants are given along with glucocorticoids if needed. Azathioprine (1 to 2 mg/day), Cycliphosphamine (1 to 2 mg/day), Mycophenolate mofetil (20 to 35 mg/day).

Comments

Popular posts from this blog

Magnetism in the Unvaccinated: The 'Magneto'-Spike Protein and Magnetogenetics.

Reports of covid19 unvaccinated people in close contact with the vaccinated people becoming 'magnetized' has been puzzling lot of independent researchers, doctors and scientists. We know that the vaccinated people shed spike proteins . But Fe3O4 based Nanoparticles do not shed, neither do they replicate and infect/transmit other people, like the spike proteins do. These Nanoparticles get deposited into brain and neural tissue as we discussed earlier . Looking at this strange phenomenon, it appears like something 'magnetic' is  Replicating - Some vaccinated people are having their whole body 'magnetized' starting from the injection site. And Transmitting : from the vaccinated people to unvaccinated people who are in close contact with them. It looks like something 'Magnetic' is being added to the Covid19 viral Spike Protein genome, which is being injected through the DNA Vector and mRNA experimental Covid19 vaccines. What if I tell you, that may be very m

The unvaccinated need not worry about the Covid-19 variants.

  Nobody needs to worry about these variants : Dr. Mark McDonald.   First of all lets shrug off the burden the main stream media has been dumping on our shoulders. The unvaccinated do not cause mutations or variants. The vaccinated people create variants. Not me, Nobel Laureate Scientist Luc Montagneir and many other scientists and doctors have said that. Vaccinations cause variants.  Curve of vaccination is followed by curve of deaths: Luc Montagneir.  Reports are coming in from Britain and Israel (among the highest vaccinated countries in the world) that vaccinated people are suffering more Deaths and Hospitalizations with 'new variants' than the unvaccinated. Click Here .  Click Here .  Click Here .  Click Here .  Click Here .  Click Here .  Click Here .  Green: Vaccinated. Red: Unvaccinated.  Remember the SAGE report  had predicted that majority of casualties of 'third wave' (Deaths and Hospitalizations) would be amongst the vaccinated people.  Also we have seen D

Deaths Occurring After Experimental Covid19 Vaccine Shots: A Small List.

 In the previous post on covid19 vaccine safety issue, I had emphasized how the deaths after vaccines may not be co-incidental but might have something to do with the experimental vaccines. Recently South Indian actor and comedian, Mr, Vivek had taken covid19 vaccine and urged everyone to take the vaccine. After taking the vaccine he went on to say " The vaccine is  the only thing that can save you. If you ask me if people who get vaccinated don't get Covid19, its not like that. Even if Covid19 hits you, there wont be death." The next day Mr. Vivek died of cardiac arrest causing a major panic among the people of Tamil Nadu. Many villagers refused to take vaccine . In fact people in India, especially the rural population are so scared of the experimental vaccines that in the state of UP, 200 villagers jumped into the river to avoid vaccinations . VAERS data: Not so safe after all.  Immediately the media swung into action and started discrediting anyone who questions the