<center><a href="https://commons.wikimedia.org/wiki/File:Changthangi_Goats_in_Jammu_and_Kashmir.jpg" rel="noopener" title="This link will take you away from steemit.com"><img src="https://steemitimages.com/640x0/https://cdn.steemitimages.com/DQmbJ4sxi3NETTc1zSJydHgKC3ihJAdUiC2MaUhpe5PUzhr/Cashmere%20goat%20Changthangi_Goats_in_Jammu_and_Kashmir%20%20Eatcha%204.0.jpg" alt="Cashmere goat Changthangi_Goats_in_Jammu_and_Kashmir  Eatcha 4.0.jpg"></a></center><br>
<center><em><a href="https://www.frontiersin.org/articles/10.3389/fgene.2018.00553/full" rel="noopener" title="This link will take you away from steemit.com">Goats in Tibet are susceptible to pemphigus.</a></em></center>
<hr>
<hr>
<p>About twenty years ago I showed my doctor a spot behind my left ear. 
 The doctor referred me to a dermatologist who performed a biopsy.  The 
report came back: pemphigus vulgaris.  I looked that up on the Internet 
and refused to accept the idea, so I went to a research hospital.  
Second biopsy: pemphigus vulgaris.</p>
<p>A powerful topical steroid erased the spot quickly and it never came 
back.  As you read this blog you will see that my fortune was good in 
the extreme.  Pemphigus can run a cruel course, and it can take a life. 
 Many, many people who receive the same pathology report I did, do not 
share my good fortune.</p>
<p>Here's some of what I learned about pemphigus in the ensuing years.  
The information might be useful someday, for you, your family or your 
pet. Pemphigus <a href="http://www.pemphigus.org/companion-animals-too-can-get-pemphigus-2/" rel="noopener" title="This link will take you away from steemit.com">affects animals, too.</a></p>
<hr>
<center><a href="https://commons.wikimedia.org/wiki/File:Akita-j.jpg" rel="noopener" title="This link will take you away from steemit.com"><img src="https://steemitimages.com/640x0/https://cdn.steemitimages.com/DQmT3XZDvNkt83KCDUhAGRLEPFACQgtNR1noyQHVep2eAVp/Akita%20Jonnydrh%20public.jpg" alt="Akita Jonnydrh public.jpg"></a></center>   <br>
<em>Certain dog breeds are more susceptible to pemphigus than others.  These breeds include <a href="https://vcahospitals.com/know-your-pet/pemphigus-in-dogs" rel="noopener" title="This link will take you away from steemit.com">Akita, Chow Chow, and Cocker Spaniels.</a></em>
<hr>
<hr>
<hr>
<h4><center>What is It?</center></h4>
<hr>
<p>According to <a href="https://www.merckmanuals.com/professional/dermatologic-disorders/bullous-diseases/pemphigus-vulgaris" rel="noopener" title="This link will take you away from steemit.com">Merk Manual</a>:<br>
"Pemphigus vulgaris is an uncommon, potentially fatal, autoimmune 
disorder characterized by intraepidermal blisters and extensive erosions
 on apparently healthy skin and mucous membranes". <sup><a href="https://steemit.com/submit.html#footnote1" rel="noopener" title="This link will take you away from steemit.com">1</a></sup></p>
<p>Translated into ordinary language: pemphigus is very rare, and it can
 be fatal.  Blisters form within the cells of the upper layer of skin.  
With a particular type of pemphigus, pemphigus vulgaris, blisters also 
form in the mucous membranes.</p>
<hr>
<p>Pemphigus is categorized as an <a href="https://www.fda.gov/drugs/drug-information-consumers/orphan-products-hope-people-rare-diseases" rel="noopener" title="This link will take you away from steemit.com">orphan disease</a>
 by the U.S. Food and Drug Administration. The occurrence of pemphigus 
varies by region across the globe, and also by ethnicity. It ranges 
between <a href="https://rarediseases.org/rare-diseases/pemphigus/" rel="noopener" title="This link will take you away from steemit.com">0.7 and 5</a>
 people, per million. Populations with a Mediterranean or Ashkenazi 
Jewish background are at higher risk. The incidence in these populations
 may be as high as <a href="http://www.pemphigus.org/research/clinically-speaking/pemphigus/" rel="noopener" title="This link will take you away from steemit.com">6-32 cases per million.</a>  A particular subset of pemphigus (endemic pemphigus) occurs with greater frequency in the <a href="http://www.pemphigus.org/research/clinically-speaking/pemphigus/" rel="noopener" title="This link will take you away from steemit.com">Brazilian rainforest and in Colombia.</a></p>
<p>There are photos available on the Internet, of people afflicted with 
pemphigus.  I'd feel I was violating those people if I used such 
distressing, personal photos.  However I did find an illustration that 
is less intrusive and yet reflects how extensively pemphigus can affect 
the skin.  This picture shows a patient (in 1896) with pemphigus 
foliaceus, PF,  a less fatal type of the disease than pemphigus 
vulgaris, PV.</p>
<p>The severe condition of this patient is one that was suffered by most before the introduction of modern treatments.</p>
<hr>
<center><a href="https://commons.wikimedia.org/wiki/File:Plate_XVIII,_Pemphigus_foliaceus,_Crocker_1896_Wellcome_L0074328.jpg" rel="noopener" title="This link will take you away from steemit.com"><img src="https://steemitimages.com/640x0/https://cdn.steemitimages.com/DQmNU5JTLa9WoVA1xushnTgbMAwdC7F2iUprfmNq2s6uku6/Pemphigus%20foliaceus,%20Crocker2%201896%20Wellcome%20L0074328%20CC%204.0%20Wellcome%20Trust.jpg" alt="Pemphigus foliaceus, Crocker2 1896 Wellcome L0074328 CC 4.0 Wellcome Trust.jpg">  </a></center>
<hr>
<hr>
<h4><center>Types of Pemphigus</center></h4>
<hr>
<p>As I poured through the literature, the words pemphigus and 
pemphigoid appeared in different contexts.  A variety of blistering 
diseases can be characterized by the words pemphigus or pemphigoid.  I 
found some categorical clarity in a 2016 article, <em>Mechanisms of Disease: Pemphigus and Bullous Pemphigoid</em>, published in the <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5560122/" rel="noopener" title="This link will take you away from steemit.com"><em>Annual Review of Pathology</em></a>.
  The authors separate the histological character (tissue analysis, 
available from a biopsy) of the disease from its clinical manifestations
 (what does it look like, what are the symptoms).</p>
<p>According to this article (and also according to the <a href="http://www.pemphigus.org/research/clinically-speaking/pemphigus/" rel="noopener" title="This link will take you away from steemit.com">International Pemphigus and Pemphigoid Foundation</a>),
 histologically there are two types: pemphigus vulgaris--PV-- and 
pemphigus foliaceus--PF .  In tissue samples of both types there will be
 evidence of <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2703183/" rel="noopener" title="This link will take you away from steemit.com">autoantibodies.</a>.
 It is the type of autoantibody and its specific target tissue that 
become diagnostic in pemphigus.  A pathologist assesses this in a 
biopsy.</p>
<p>In pemphigus, autoantibodies target a specific substance found in the
 epidermis: desmoglein.   With the destruction of desmoglein, essential 
cells, called keratinocytes, cannot hold together.  Blisters form in 
those areas of failure and the skin essentially falls apart.</p>
<hr>
<h5><center>Keratinocytes</center></h5>
<center><a href="https://commons.wikimedia.org/wiki/File:Human_keratinocytes.jpg" rel="noopener" title="This link will take you away from steemit.com"><img src="https://steemitimages.com/640x0/https://cdn.steemitimages.com/DQmY6yKt3P8tNZE6HUrdJRiA5cFbKudA3gQGodSkjjx5bzY/Human%20keratinocytes3%20ManatYesbol,%20Zhansaia,%20Gaziza%20Aiatbekovna%204.0.jpg" alt="Human keratinocytes3 ManatYesbol, Zhansaia, Gaziza Aiatbekovna 4.0.jpg"></a></center>
<hr>
<hr>
<p><em>Keratinocytes make up about <a href="http://library.open.oregonstate.edu/aandp/chapter/5-1-layers-of-the-skin/" rel="noopener" title="This link will take you away from steemit.com">95% of the epidermis.</a>  Without treatment, a pemphigus patient may actually lose most of the epidermis.  This would lead to <a href="https://rarediseases.org/rare-diseases/pemphigus/" rel="noopener" title="This link will take you away from steemit.com">fluid loss, widespread infection, and likely sepsis.  Most patients would  die in about two years.</a></em></p>
<hr>
<p>There are different types of desmoglein.  A diagnosis of PV or PF is based on which kind of <a href="http://www.jofs.in/article.asp?issn=0975-8844;year=2014;volume=6;issue=2;spage=104;epage=107;aulast=Gandhi" rel="noopener" title="This link will take you away from steemit.com">desmoglein is under attack.</a>
  A biopsy will reveal this. If it turns out that desmoglein 1 is the 
target, then the finding is for PF.  If desmoglein 3 is under attack, 
then the diagnosis is PV.  There are other diagnostic tests that are 
used to come up with a definitive diagnosis, but separating the 
histology into attacks on desmoglein 1 and 3 is a basic step.</p>
<hr>
<h5><center>IgG, an Autoantibody Found in Pemphigus</center></h5>
<center><a href="https://upload.wikimedia.org/wikipedia/commons/2/27/Anatomy_of_an_IgG.png" rel="noopener" title="This link will take you away from steemit.com"><img src="https://steemitimages.com/640x0/https://cdn.steemitimages.com/DQmUcqFiSCSEMwUVoojE15Z9yyy6FLrU8WRXUFtGmEPUceZ/IgG%20Anatomy%20wikipedia%20user%20AJVincelli%20public%20domain.jpg" alt="IgG Anatomy wikipedia user AJVincelli public domain.jpg"></a></center>
<hr>
<hr>
<p><em>The presence of <a href="https://www.ncbi.nlm.nih.gov/pubmed/19963079" rel="noopener" title="This link will take you away from steemit.com">IgG autoantibodies is a hallmark</a> of PF and PV.</em></p>
<p><em>IgG is an immunoglobulin.  It is the <a href="https://www.sciencedirect.com/topics/neuroscience/immunoglobulin-g" rel="noopener" title="This link will take you away from steemit.com">most abundant</a>
 immunoglobulin in the body and is necessary for proper function of the 
immune system.  As a matter of fact, it is the only antibody class that 
can <a href="http://www.jimmunol.org/content/jimmunol/194/1/13.full.pdf" rel="noopener" title="This link will take you away from steemit.com">cross the placental barrier.</a> This allows the mother to bestow some of her immunity on the fetus.</em></p>
<p><em>IgG is not the only autoantibody involved in the development of 
pemphigus, but it is the most predominant.  It is also implicated in a 
number of other <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4602543/" rel="noopener" title="This link will take you away from steemit.com">autoimmune diseases.</a></em></p>
<hr>
<hr>
<h5><center>Desmoglein 1 and Desmoglein 3</center></h5>
<hr>
<p>Most of us know that keratin is contained in hair, nails and skin. Keratinocytes <a href="http://library.open.oregonstate.edu/aandp/chapter/5-1-layers-of-the-skin/" rel="noopener" title="This link will take you away from steemit.com">manufacture and store keratin, </a> and are the <a href="https://www.ncbi.nlm.nih.gov/pubmed/28223740" rel="noopener" title="This link will take you away from steemit.com">basic building blocks</a>
 of the epidermis.  Keratinocytes are held together by extracellular 
structures called desmosomes. These structures are like the mortar 
between bricks in a wall.  Desmosomes are comprised of two 
glycoproteins, <a href="http://jcs.biologists.org/content/127/10/e1001" rel="noopener" title="This link will take you away from steemit.com">desmoglein and desmocollin.</a></p>
<p>Remember, in PF and PV, it is desmoglein that is destroyed by 
autoantibodies.  So, if the desmoglein is  destroyed, desmosome, the 
intracellular mortar between keratinocytesis is destroyed.  The 
keratinocytes are no longer held together.   The basic building blocks 
of the skin separate, and the skin literally falls apart.  Blisters form
 in the gaps that open up.</p>
<hr>
<hr>
<hr>
<p><em>I made a GIF to demonstrate how the structure of the skin is 
undermined by the destruction of desmoglein.  Here the mortar between 
the bricks in a wall slowly erodes and the bricks fall away.  Compare 
this GIF to the image of tissue undergoing the process of acantholysis, a
 little further down in the blog.</em></p>
<hr>
<center><img src="https://steemitimages.com/0x0/https://cdn.steemitimages.com/DQmP116hVwdgm8PyhqPYoPB5C9iTrVcPt8m1AXi3GoUFWpA/brick%20wall%204.gif" alt="brick wall 4.gif"></center>
<center><em>Brick wall from Paint 3D</em></center>
<hr>
<hr>
<hr>
<hr>
<hr>
<h5><center>Illustration of Desmosome Cell Adhesion</center></h5>
<center><a href="https://commons.wikimedia.org/wiki/File:Desmosome_cell_junction_en.svg" rel="noopener" title="This link will take you away from steemit.com"><img src="https://steemitimages.com/640x0/https://cdn.steemitimages.com/DQmQq8iie1g2rRuN5xfzDKHSr5KMR9ooAwLXqRnYDugQQi9/Desmosome2.jpg" alt="Desmosome2.jpg"></a></center>
<hr>
<p>Desmosomes are only one type of intracellular juncture in animal 
tissue.  Most of us are more familiar with another kind: cartilage.  
Both desmosomes and cartilage are designed to bear weight and to allow 
for flexibility.   You can see in the illustration above that fibers 
connect one cell to another at an adhesion point called a cadherin.  It 
is the cadherin that is <a href="http://jcs.biologists.org/content/127/10/e1001" rel="noopener" title="This link will take you away from steemit.com">is made up of desmoglein.</a>  This is precisely the area of vulnerability in pemphigus.</p>
<hr>
<p>Below is a microscopic image of tissue taken from a patient who has 
PV.  Even with the untrained eye, we can see gaps in the tissue. 
According to the description that appears under this photo, "an early 
intraepidermal vesicle" is evident in this tissue.<sup><a href="https://steemit.com/submit.html#footnote2" rel="noopener" title="This link will take you away from steemit.com">2</a></sup>  That is, blisters are already forming, and are evident.  Compare this gap to the gap in my brick wall GIF.</p>
<p>The process by which desmosomes are destroyed and epithelial tissue separates is called <a href="https://www.sciencedirect.com/topics/medicine-and-dentistry/acantholysis" rel="noopener" title="This link will take you away from steemit.com">acantholysis.</a></p>
<center><a href="https://commons.wikimedia.org/wiki/File:Pemphigus_immunofluorescence.jpg" rel="noopener" title="This link will take you away from steemit.com"><img src="https://steemitimages.com/640x0/https://cdn.steemitimages.com/DQmZxm7ytyKYfRFZfuqHXQ8xNQm5ESm6j1HZ1PF69eycvtx/Pemphigus_immunofluorescence2%20%20attr%20Emmanuelm%20at%20english%20wikipedia%203.0.jpg" alt="Pemphigus_immunofluorescence2  attr Emmanuelm at english wikipedia 3.0.jpg"></a></center>
<hr>
<hr>
<h5><center>A Personal Digression</center></h5>
<hr>
<p>In the title of this blog I refer to a personal perspective. Here it 
is: my experience does not resemble in any way what I have just 
described.  Despite the diagnosis of pemphigus vulgaris, based on two 
biopsies, a blister never formed. Both pathologists did find that IgG 
autoantibodies were attacking desmoglein 3 in the biopsied tissue.  By 
the time I showed my doctor the spot, almost two years had passed (!).</p>
<p>The spot had grown a bit, but it was still under 2 cm.  I thought I might have a <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2872290/" rel="noopener" title="This link will take you away from steemit.com">modest squamous cell carcinoma.</a></p>
<p>I was wrong.</p>
<hr>
<hr>
<center><a href="https://commons.wikimedia.org/wiki/File:Diagram_of_human_skin.jpg" rel="noopener" title="This link will take you away from steemit.com"><img src="https://steemitimages.com/640x0/https://cdn.steemitimages.com/DQmXAT83RojGA2ZwygXt7k66GnwiuQEUjKE1RRCEHU8xHc9/Skin2%20Diagram%20of%20human%20skin%20Crystal%20Mason%203.0.jpg" alt="Skin2 Diagram of human skin Crystal Mason 3.0.jpg"></a></center>
<p><em>I selected this diagram out of many because it indicates that squamous cells are near the surface of the skin.  <a href="https://opentextbc.ca/anatomyandphysiology/chapter/5-1-layers-of-the-skin/" rel="noopener" title="This link will take you away from steemit.com">Stratified squamous epithelium</a>
 comprise the epidermis.  It is here, in the squamous cells, that 
pemphigus (PV and PF) develops. These cells are flat (wider than they 
are high) and are arranged in layers.</em></p>
<hr>
<hr>
<p>The image below shows the lining of a normal esophagus.  Stratified 
squamous cells are visible.  The esophagus is lined with a mucous 
membrane, which means it is vulnerable to the development of PV, but not
 PF.</p>
<hr>
<center><a href="https://commons.wikimedia.org/wiki/File:Tinci%C3%B3n_hematoxilina-eosina.jpg" rel="noopener" title="This link will take you away from steemit.com"><img src="https://steemitimages.com/640x0/https://cdn.steemitimages.com/DQmSB31261uWkaVPD1v7zCBLA5sgJ9pgcKVDSzvQbjXGLrN/squamous2%20cells%20in%20the%20esophagus,normal%20stain%20Samir@enwiki%203.0.jpg" alt="squamous2 cells in the esophagus,normal stain Samir@enwiki 3.0.jpg"></a></center>
<hr>
<hr>
<p>In PV,  mucous membranes are often the first sites affected.  Typically, oral lesions appear.  Because of this, <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3505431/" rel="noopener" title="This link will take you away from steemit.com">dentists can play an important role</a>
 in detecting the disease. Mucosal involvement may extend to the 
esophagus, the nose and even inside the eyelids. (Both pemphigus 
vulgaris and pemphigus foliaceus <a href="http://www.pemphigus.org/when-the-eyes-have-it-2/" rel="noopener" title="This link will take you away from steemit.com">may effect the eyelids.</a>)</p>
<p>Before the advent of modern treatment, oral and esophageal lesions inhibited eating, and this contributed to mortality.</p>
<hr>
<hr>
<h3><center>Clinical Manifestations of Pemphigus</center></h3>
<p>While pemphigus is broadly described as being either pemphigus 
foliaceus or pemphigus vulgaris, based on histology, there are 
subdivisions of these two types. It's hard to get different sources to 
agree on what these subdivisions are, so I settled for the grouping 
offered by <a href="https://rarediseases.org/rare-diseases/pemphigus/" rel="noopener" title="This link will take you away from steemit.com">NORD, National Organization for Rare Diseases.</a> Please note, these subdivisions may overlap, or appear sequentially.</p>
<hr>
<p>NORD lists six subdivisions, two of these have already been discussed in some detail:</p>
<h6>Pemphigus Foliaceus</h6>
<h6>Pemphigus Vulgaris</h6>
<hr>
<p>I'll say a few words about the other four subcategories:</p>
<hr>
<hr>
<h5><center><strong>Endemic Pemphigus</strong></center></h5>
<p>I referred to this earlier in the blog as occurring in the 
rainforests of Brazil and Colombia. I have also read an article which 
relates <a href="http://www.scielo.br/scielo.php?script=sci_arttext&amp;pid=S0365-05962012000600003" rel="noopener" title="This link will take you away from steemit.com">similar cases in Peru</a>
  to the same type of endemic disease.  The suspicion has always existed
 that an environmental factor triggers the disease in genetically 
susceptible individuals.  A fascinating  2016 article in the <a href="http://www.jimmunol.org/content/196/5/2041" rel="noopener" title="This link will take you away from steemit.com"><em>Journal of Immunology</em></a> suggests that the environmental exposure might be the bite of a sandfly.  In this case, pathogenic IgG4, instead of IgG1 <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5816565/" rel="noopener" title="This link will take you away from steemit.com">(which is often the culprit)</a> is implicated.</p>
<hr>
<hr>
<center><a href="https://commons.wikimedia.org/wiki/File:Lutzomyia_longipalpis-sandfly.jpg" rel="noopener" title="This link will take you away from steemit.com"><img src="https://steemitimages.com/640x0/https://cdn.steemitimages.com/DQmek1ZdDxGMd113k7GkHf16e7uyJQQNjrFWbZPSeDihDAN/Lutzomyia%20longipalpis-sandfly2%20Ray%20Wilson,%20Liverpool%20School%20of%20Tropical%20Medicine%202.5%20and%20PLOS%20One%204.0.jpg" alt="Lutzomyia longipalpis-sandfly2 Ray Wilson, Liverpool School of Tropical Medicine 2.5 and PLOS One 4.0.jpg"></a></center>
<p><em>Sandflies are found all over the world, but this variety is only 
found in the so-called 'New World'--which would encompass Brazil, Peru 
and Colombia, the countries where endemic pemphigus has been identified</em>.</p>
<hr>
<hr>
<h5><center><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3539423/" rel="noopener" title="This link will take you away from steemit.com"><strong>Pemphigus Erythematosus (Senear-Usher syndrome)</strong></a></center></h5>
<p>This may be an overlap disease with lupus erythematosus.  Patients 
have serology that is positive for lupus (ex: anti-SM and 
double-stranded DNA antigens, hallmarks of  lupus) and also have 
histology and clinical presentation compatible with pemphigus.</p>
<hr>
<hr>
<h5><center><strong>Pemphigus Vegetans</strong></center></h5>
<p>This is described by most sources as a rare, localized, benign form of PV.  Partly because of its rarity, it can be <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3505428/" rel="noopener" title="This link will take you away from steemit.com">difficult to diagnose.</a></p>
<hr>
<hr>
<h5><center><strong>Drug-Induced Pemphigus</strong></center></h5>
<hr>
<center><a href="https://commons.wikimedia.org/wiki/File:Mercapto_Group_General_Formulae.png" rel="noopener" title="This link will take you away from steemit.com"><img src="https://steemitimages.com/640x0/https://cdn.steemitimages.com/DQmac6SwXctrmGLoxyPa6QNuUoLTFxUPj84rpYzkMYnucTP/Thiol%20Group%20J%C3%BC%20public2.jpg" alt="Thiol Group Jü public2.jpg"></a></center>
<p><em>The  -SH in the formula above is a sulfhydryl, and it is this 
that characterizes the thiol group of drugs. This group of drugs is 
implicated in the <a href="https://www.ncbi.nlm.nih.gov/books/NBK499864/" rel="noopener" title="This link will take you away from steemit.com">development of pemphigus.</a></em></p>
<hr>
<p>This is probably one of the most important parts of my blog today:  
pemphigus may be caused by drugs that are commonly prescribed.  In many 
cases, the disease will clear up when the drug is withdrawn, but this is
 not always the case.  Also, pemphigus may not develop until months 
after the <a href="https://www.dermnetnz.org/topics/drug-induced-pemphigus/" rel="noopener" title="This link will take you away from steemit.com">drug has been commenced,</a> so it's hard to draw a relationship between the drug and the disease.    According to <a href="https://www.ncbi.nlm.nih.gov/books/NBK499864/" rel="noopener" title="This link will take you away from steemit.com">StatPearls,</a> drugs are actually the most likely cause of pemphigus, especially pemphigus foliaceus.</p>
<p>Certain classes of drugs are more closely associated with the disease
 than others.  Thiols (especially d-penicillamine) have been implicated.
    Phenols have also been implicated.  Interestingly,  diet can also be
 a source of phenols and thiols, so foods that contain these...chives, 
garlic, onion, black pepper, cashew and mangoes...should be avoided by 
anyone who has been diagnosed with pemphigus.</p>
<p>According to <a href="https://www.dermnetnz.org/topics/drug-induced-pemphigus/" rel="noopener" title="This link will take you away from steemit.com">DermNet NZ</a>,
 about 50% of the cases caused by thiols will clear up when the drug is 
withdrawn.  Disease caused by non-thiol drugs, "have a better 
prognosis".<sup><a href="https://steemit.com/submit.html#footnote3" rel="noopener" title="This link will take you away from steemit.com">3</a></sup></p>
<p><a href="http://www.ijdvl.com/article.asp?issn=0378-6323;year=2018;volume=84;issue=2;spage=137;epage=147;aulast=Ranugha" rel="noopener" title="This link will take you away from steemit.com">Anti-hypertensives</a> and <a href="https://rarediseases.info.nih.gov/diseases/7355/pemphigus-vulgaris" rel="noopener" title="This link will take you away from steemit.com">NSAIDs have also been implicated.</a></p>
<hr>
<hr>
<h2><center>Treatments</center></h2>
<p>The cornerstone of treatment remains corticosteroids, administered 
systemically, topically or a combination of the two.  These drugs can 
have serious side effects, including:  cataracts, diabetes, 
osteoporosis, psychosis, chronic migraine and increased susceptibility 
to infection.  The effects have been found to be <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1746020/" rel="noopener" title="This link will take you away from steemit.com">dose dependent.</a>  The goal, for physician and patient, is to take the lowest effective dose. This treatment strategy is referred to as <a href="https://www.valueinhealthjournal.com/article/S1098-3015(14)00294-0/fulltext" rel="noopener" title="This link will take you away from steemit.com">'steroid sparing',</a> and it often involves a balancing act between drug efficacy and drug effects.</p>
<hr>
<p>Some other treatments for pemphigus are:<br>
<a href="http://www.pemphigus.org/azathioprine-in-the-treatment-of-autoimmune-blistering-diseases/" rel="noopener" title="This link will take you away from steemit.com">Azathioprine</a><br>
<a href="http://www.e-ijd.org/article.asp?issn=0019-5154;year=2016;volume=61;issue=4;spage=418;epage=426;aulast=Sardana" rel="noopener" title="This link will take you away from steemit.com">Cyclophosphamide</a><br>
<a href="https://www.uptodate.com/contents/initial-management-of-pemphigus-vulgaris-and-pemphigus-foliaceus/abstract/5" rel="noopener" title="This link will take you away from steemit.com">Mycophenalate</a><br>
<a href="https://www.ncbi.nlm.nih.gov/pubmed/23772610" rel="noopener" title="This link will take you away from steemit.com">Methotrexate</a><br>
<a href="https://www.gene.com/media/press-releases/14727/2018-06-07/fda-approves-genentechs-rituxan-rituxima" rel="noopener" title="This link will take you away from steemit.com">Rituximab</a><br>
<a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4540507/" rel="noopener" title="This link will take you away from steemit.com">Dapsone</a><br>
<a href="https://jamanetwork.com/journals/jamadermatology/article-abstract/420177" rel="noopener" title="This link will take you away from steemit.com">IV infusion of immunoglobulin</a><br>
<a href="https://www.mdedge.com/dermatology/article/97747/wounds/plasmapheresis-refractory-pemphigus-vulgaris-revisiting-old" rel="noopener" title="This link will take you away from steemit.com">Plasmapheresis</a></p>
<hr>
<h4><center>Causes/Antagonists</center></h4>
<p>We've already covered drugs and diet.  Genetics seems to play a weak 
role, particularly in some families. According to GARD, Genetic and Rare
 Diseases Information Center, <a href="https://rarediseases.info.nih.gov/diseases/7355/pemphigus-vulgaris" rel="noopener" title="This link will take you away from steemit.com">"
 Predisposition...is linked to genetic factors...histocompatibility 
complex (MHC) class II molecules, in particular alleles of human 
leukocyte antigen (HLA) DR4, appear to increase susceptibility to 
pemphigus vulgaris."</a></p>
<p>There are known environmental antagonists, besides drugs, diet and, possibly, sandflies.  One of these, very avoidable, <a href="http://www.pemphigus.org/tag/sun-exposure/" rel="noopener" title="This link will take you away from steemit.com">is the sun..</a>or any form of <a href="https://www.ncbi.nlm.nih.gov/books/NBK431072/" rel="noopener" title="This link will take you away from steemit.com">UV radiation.</a></p>
<hr>
<h5><center>Ending on a Personal Note</center></h5>
<p>I have a serious UV allergy, which encompasses not only sun but 
indoor sources of UV.  This may be one of the factors that has kept 
pemphigus at bay all these years.  I avoid the sun.  My wardrobe offers 
evidence of that.  I have a closet full of hats.  So I decided to end 
the blog on a rather humorous note:  a GIF that shows just <em>some</em> of my hats :)</p>
<center>A Few of My Hats</center><br>
<center><img src="https://steemitimages.com/0x0/https://cdn.steemitimages.com/DQmdmiagTW48VKUEBvzYhoSzcB18zKzWXYQfbN2ym6wWopw/hat%20blend2.gif" alt="hat blend2.gif"></center>
<hr>
<p>Anyone who wants to use any of my GIFs, for any reason, may do so without restriction.</p>
<center><img src="https://steemitimages.com/640x0/https://cdn.steemitimages.com/DQmTDqHVniPuLrhgLZxBCzjKQYcdiCXVL6Vp6qnRSHYWKe2/accent%20accent.jpg" alt="accent accent.jpg"></center>
<h4><center>Footnotes</center></h4>
<p><sup><a href="https://steemit.com/submit.html#footnote1" rel="noopener" title="This link will take you away from steemit.com">1</a></sup>Merck Manual Professional Version: <a href="https://www.merckmanuals.com/professional/dermatologic-disorders/bullous-diseases/pemphigus-vulgaris" rel="noopener" title="This link will take you away from steemit.com"><em>Pemphigus Vulgaris</em></a></p>
<p><sup><a href="https://steemit.com/submit.html#footnote2" rel="noopener" title="This link will take you away from steemit.com">2</a></sup><a href="https://commons.wikimedia.org/wiki/File:Pemphigus_immunofluorescence.jpg" rel="noopener" title="This link will take you away from steemit.com">Pemphigus immunofluorescence</a></p>
<p><sup><a href="https://steemit.com/submit.html#footnote3" rel="noopener" title="This link will take you away from steemit.com">3</a></sup>DermNet <a href="https://www.dermnetnz.org/topics/drug-induced-pemphigus/" rel="noopener" title="This link will take you away from steemit.com"><em>Drug-induced pemphigus</em></a></p>
<hr>
<hr>
<h4><center>Some Sources Used in Writing This Blog</center></h4>
<ol><li><p>Sequence Characterization of DSG3 Gene to Know Its Role in High-Altitude Hypoxia Adaptation in the Chinese Cashmere Goat  <a href="https://www.frontiersin.org/articles/10.3389/fgene.2018.00553/full" rel="noopener" title="This link will take you away from steemit.com">https://www.frontiersin.org/articles/10.3389/fgene.2018.00553/full</a></p></li><li><p>Companion Animals, Too, Can Get Pemphigus  <a href="http://www.pemphigus.org/companion-animals-too-can-get-pemphigus-2/" rel="noopener" title="This link will take you away from steemit.com">http://www.pemphigus.org/companion-animals-too-can-get-pemphigus-2/</a></p></li><li><p>Pemphigus in Dogs  <a href="https://vcahospitals.com/know-your-pet/pemphigus-in-dogs" rel="noopener" title="This link will take you away from steemit.com">https://vcahospitals.com/know-your-pet/pemphigus-in-dogs</a></p></li><li><p>Pemphigus Vulgaris  <a href="https://www.merckmanuals.com/professional/dermatologic-disorders/bullous-diseases/pemphigus-vulgaris" rel="noopener" title="This link will take you away from steemit.com">https://www.merckmanuals.com/professional/dermatologic-disorders/bullous-diseases/pemphigus-vulgaris</a></p></li><li><p>Orphan Products: Hope for People With Rare Diseases  <a href="https://www.fda.gov/drugs/drug-information-consumers/orphan-products-hope-people-rare-diseases" rel="noopener" title="This link will take you away from steemit.com">https://www.fda.gov/drugs/drug-information-consumers/orphan-products-hope-people-rare-diseases</a></p></li><li><p>Pemphigus   <a href="https://rarediseases.org/rare-diseases/pemphigus/" rel="noopener" title="This link will take you away from steemit.com">https://rarediseases.org/rare-diseases/pemphigus/</a></p></li><li><p>Pemphigus  <a href="http://www.pemphigus.org/research/clinically-speaking/pemphigus/" rel="noopener" title="This link will take you away from steemit.com">http://www.pemphigus.org/research/clinically-speaking/pemphigus/</a></p></li><li><p>Mechanisms of Disease: Pemphigus and Bullous Pemphigoid <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5560122/" rel="noopener" title="This link will take you away from steemit.com">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5560122/</a></p></li><li><p>Nature and functions of autoantibodies <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2703183/" rel="noopener" title="This link will take you away from steemit.com">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2703183/</a></p></li><li><p>Layers of the Skin <a href="http://library.open.oregonstate.edu/aandp/chapter/5-1-layers-of-the-skin/" rel="noopener" title="This link will take you away from steemit.com">http://library.open.oregonstate.edu/aandp/chapter/5-1-layers-of-the-skin/</a></p></li><li><p>Usefulness of desmoglein 1 and 3 in serodiagnosis of pemphigus 
vulgaris and its correlation with disease activity - ELISA study  <a href="http://www.jofs.in/article.asp?issn=0975-8844;year=2014;volume=6;issue=2;spage=104;epage=107;aulast=Gandhi" rel="noopener" title="This link will take you away from steemit.com">http://www.jofs.in/article.asp?issn=0975-8844;year=2014;volume=6;issue=2;spage=104;epage=107;aulast=Gandhi</a></p></li><li><p>Definition of human autoimmunity--autoantibodies versus autoimmune disease     <a href="https://www.ncbi.nlm.nih.gov/pubmed/19963079" rel="noopener" title="This link will take you away from steemit.com">https://www.ncbi.nlm.nih.gov/pubmed/19963079</a></p></li><li><p>Immunoglobulin G <a href="https://www.sciencedirect.com/topics/neuroscience/immunoglobulin-g" rel="noopener" title="This link will take you away from steemit.com">https://www.sciencedirect.com/topics/neuroscience/immunoglobulin-g</a></p></li><li><p>Natural Antibodies Bridge Innate and Adaptive Immunity <a href="http://www.jimmunol.org/content/jimmunol/194/1/13.full.pdf" rel="noopener" title="This link will take you away from steemit.com">http://www.jimmunol.org/content/jimmunol/194/1/13.full.pdf</a></p></li><li><p>Serum IgG Subclasses in Autoimmune Diseases <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4602543/" rel="noopener" title="This link will take you away from steemit.com">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4602543/</a></p></li><li><p>Kinetin Improves Barrier Function of the Skin by Modulating Keratinocyte Differentiation Markers<br>
<a href="https://www.ncbi.nlm.nih.gov/pubmed/28223740" rel="noopener" title="This link will take you away from steemit.com">https://www.ncbi.nlm.nih.gov/pubmed/28223740</a></p></li><li><p>Desmocollin and desmoglein – different roles in desmosomes <a href="http://jcs.biologists.org/content/127/10/e1001" rel="noopener" title="This link will take you away from steemit.com">http://jcs.biologists.org/content/127/10/e1001</a></p></li><li><p>Learn more about Acantholysis  <a href="https://www.sciencedirect.com/topics/medicine-and-dentistry/acantholysis" rel="noopener" title="This link will take you away from steemit.com">https://www.sciencedirect.com/topics/medicine-and-dentistry/acantholysis</a></p></li><li><p>Potential Risk Factors for Cutaneous Squamous Cell Carcinoma 
include Oral Contraceptives: Results of a Nested Case-Control Study  <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2872290/" rel="noopener" title="This link will take you away from steemit.com">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2872290/</a></p></li><li><p>Pemphigus vulgaris presenting as gingival involvement  <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3505431/" rel="noopener" title="This link will take you away from steemit.com">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3505431/</a></p></li><li><p>When the Eyes Have It  <a href="http://www.pemphigus.org/when-the-eyes-have-it-2/" rel="noopener" title="This link will take you away from steemit.com">http://www.pemphigus.org/when-the-eyes-have-it-2/</a></p></li><li><p>Endemic pemphigus in the peruvian Amazon: epidemiology and risk factors for the development of complications during treatment  <a href="http://www.scielo.br/scielo.php?script=sci_arttext&amp;pid=S0365-05962012000600003" rel="noopener" title="This link will take you away from steemit.com">http://www.scielo.br/scielo.php?script=sci_arttext&amp;pid=S0365-05962012000600003</a></p></li><li><p>Overlapping IgG4 Responses to Self- and Environmental Antigens in Endemic Pemphigus Foliaceus <a href="http://www.jimmunol.org/content/196/5/2041" rel="noopener" title="This link will take you away from steemit.com">http://www.jimmunol.org/content/196/5/2041</a></p></li><li><p>A New Classification System for IgG4 Autoantibodies  <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5816565/" rel="noopener" title="This link will take you away from steemit.com">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5816565/</a></p></li><li><p>Autoantibodies in Senear-Usher Syndrome: Cross-Reactivity or Multiple Autoimmunity?<br>
<a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3539423/" rel="noopener" title="This link will take you away from steemit.com">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3539423/</a></p></li><li><p>Pemphigus vegetans: An unusual presentation   <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3505428/" rel="noopener" title="This link will take you away from steemit.com">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3505428/</a></p></li><li><p>Drug Induced Pemphigus  <a href="https://www.ncbi.nlm.nih.gov/books/NBK499864/" rel="noopener" title="This link will take you away from steemit.com">https://www.ncbi.nlm.nih.gov/books/NBK499864/</a></p></li><li><p>Drug-induced pemphigus <a href="https://www.dermnetnz.org/topics/drug-induced-pemphigus/" rel="noopener" title="This link will take you away from steemit.com">https://www.dermnetnz.org/topics/drug-induced-pemphigus/</a></p></li><li><p>Antihypertensives in dermatology Part II - Cutaneous adverse reactions to antihypertensives      <a href="http://www.ijdvl.com/article.asp?issn=0378-6323;year=2018;volume=84;issue=2;spage=137;epage=147;aulast=Ranugha" rel="noopener" title="This link will take you away from steemit.com">http://www.ijdvl.com/article.asp?issn=0378-6323;year=2018;volume=84;issue=2;spage=137;epage=147;aulast=Ranugha</a></p></li><li><p>Pemphigus vulgaris   <a href="https://rarediseases.info.nih.gov/diseases/7355/pemphigus-vulgaris" rel="noopener" title="This link will take you away from steemit.com">https://rarediseases.info.nih.gov/diseases/7355/pemphigus-vulgaris</a></p></li><li><p>Adverse effects of oral corticosteroids in relation to dose in patients with lung disease       <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1746020/" rel="noopener" title="This link will take you away from steemit.com">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1746020/</a></p></li><li><p>Patterns of steroid and steroid sparing regimens among older 
inflammatory bowel disease (IBD) patients with contraindications to 
tumor necrosis factor antagonists (ANTI-TNFS)       <a href="https://www.valueinhealthjournal.com/article/S1098-3015" rel="noopener" title="This link will take you away from steemit.com">https://www.valueinhealthjournal.com/article/S1098-3015</a>(14)00294-0/fulltext</p></li><li><p>Azathioprine in the treatment of Autoimmune Blistering diseases     <a href="http://www.pemphigus.org/azathioprine-in-the-treatment-of-autoimmune-blistering-diseases/" rel="noopener" title="This link will take you away from steemit.com">http://www.pemphigus.org/azathioprine-in-the-treatment-of-autoimmune-blistering-diseases/</a></p></li><li><p>A comparative effectiveness research of azathioprine and 
cyclophosphamide on the clinical and serological response in pemphigus 
vulgaris  <a href="http://www.e-ijd.org/article.asp?issn=0019-5154;year=2016;volume=61;issue=4;spage=418;epage=426;aulast=Sardana" rel="noopener" title="This link will take you away from steemit.com">http://www.e-ijd.org/article.asp?issn=0019-5154;year=2016;volume=61;issue=4;spage=418;epage=426;aulast=Sardana</a></p></li><li><p>Medline ® Abstract for Reference 5 of 'Initial management of pemphigus vulgaris and pemphigus foliaceus'   <a href="https://www.uptodate.com/contents/initial-management-of-pemphigus-vulgaris-and-pemphigus-foliaceus/abstract/5" rel="noopener" title="This link will take you away from steemit.com">https://www.uptodate.com/contents/initial-management-of-pemphigus-vulgaris-and-pemphigus-foliaceus/abstract/5</a></p></li><li><p>Methotrexate in the treatment of pemphigus vulgaris: experience in 23 patients      <a href="https://www.ncbi.nlm.nih.gov/pubmed/23772610" rel="noopener" title="This link will take you away from steemit.com">https://www.ncbi.nlm.nih.gov/pubmed/23772610</a></p></li><li><p>FDA Approves Genentech’s Rituxan (Rituximab) for Pemphigus Vulgaris<br>
<a href="https://www.gene.com/media/press-releases/14727/2018-06-07/fda-approves-genentechs-rituxan-rituxima" rel="noopener" title="This link will take you away from steemit.com">https://www.gene.com/media/press-releases/14727/2018-06-07/fda-approves-genentechs-rituxan-rituxima</a></p></li><li><p>Dapsone in the treatment of pemphigus vulgaris: adverse effects and its importance as a corticosteroid sparing agent  <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4540507/" rel="noopener" title="This link will take you away from steemit.com">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4540507/</a></p></li><li><p>Effect of Intravenous Immunoglobulin Therapy on Serum Levels of 
IgG1 and IgG4 Antidesmoglein 1 and Antidesmoglein 3 Antibodies in 
Pemphigus Vulgaris    <a href="https://jamanetwork.com/journals/jamadermatology/article-abstract/420177" rel="noopener" title="This link will take you away from steemit.com">https://jamanetwork.com/journals/jamadermatology/article-abstract/420177</a></p></li><li><p>Plasmapheresis in Refractory Pemphigus Vulgaris: Revisiting an 
Old Treatment Modality Used in Synchrony With Pulse Cyclophosphamide    
  <a href="https://www.mdedge.com/dermatology/article/97747/wounds/plasmapheresis-refractory-pemphigus-vulgaris-revisiting-old" rel="noopener" title="This link will take you away from steemit.com">https://www.mdedge.com/dermatology/article/97747/wounds/plasmapheresis-refractory-pemphigus-vulgaris-revisiting-old</a></p></li><li><p>Pemphigus vulgaris  <a href="https://rarediseases.info.nih.gov/diseases/7355/pemphigus-vulgaris" rel="noopener" title="This link will take you away from steemit.com">https://rarediseases.info.nih.gov/diseases/7355/pemphigus-vulgaris</a></p></li><li><p>Sun Exposure <a href="http://www.pemphigus.org/tag/sun-exposure/" rel="noopener" title="This link will take you away from steemit.com">http://www.pemphigus.org/tag/sun-exposure/</a></p></li><li><p>Photosensitivity <a href="https://www.ncbi.nlm.nih.gov/books/NBK431072/" rel="noopener" title="This link will take you away from steemit.com">https://www.ncbi.nlm.nih.gov/books/NBK431072/</a></p></li></ol>