Frustration over HSP Diagnosis and Treatment

Well, the know disease is HSP, a rare form of leuckocytoclastic vasculitis. 

In fact, I told the doctor, in consultation with an infctious diseases specialist that this is vasculitis, instead they kept on harping that it is neuropathic disorder and pain. 

So, I changed the doctor and he diagnosed it correctly. But not before there was substantial progression of the disease without correct diagnosis and treatment. All the symptoms where there for HSP, but diagnosis in HSP is done clinically and biopsy, not by a lab test. 

 

This is especially true when the doctor has no idea about HSP and is not listening to patient or going in the wrong direction for treatment because he cannot diagnose what is the problem with the patient. 

 

A neuropathic disorder for a vaculitis condition?  Well as I mentioned I went to skin doctor and of course they were just clueless.  It's still bizarre to me that a walk in pharmacy clinic got lucky on the second visit to just happen to prescribe me prednisone on my earlier cases because it did work for me.  No doctor but the correct immunology specialist had any idea what HSP even was or bothered to research my condition to try and categorize it or suggest the correct specialist.   Sorry to hear you had such a bad progression although I'm not sure without knowing a cause from specific allergy which drugs work still.   

It my case, the cause was some bunch of infections 

sore throats, infections, colds, etc that cause the immune system response are really more like triggers aren't they?  Nobody knows what actually causes the HSP disorder.  For example, maybe a gene gets mutated or the body under some condition either due to a drug (cold medication or whatever) changes the way it behaves, but nobody knows what causes this change, or what the change even is.  If they did, they could make some progress on how to identify and treat it.

HSP has very specific symptoms ... namely, 

Skin rash or burning sensation which will cause darker/violet skin (latin of purpura is violet). 

HSP is caused by either infection, chemicals, or drugs/medications. 

There is not such things is "nobody knows". Only the inexperienced doctors have no idea about HSP. 

This is not a mutation disease. Those are genetic / hereditary disorders, but HSP is not one of them. 

HSP has very specific symptoms ... namely, 

Skin rash or burning sensation which will cause darker/violet skin (latin of purpura is violet) - 100% , joint pain (arthralgia) - 90%. 

Abdominal pain - 70-80%

Kidney involvement in 25-30% cases. 

I have to respectfully state some alternate opinion and hope you can point to your causes being in fact causes.  From everything I have read they are simply triggers to starting the immune system reacting and it is the improper immune system reaction that is HSP.    Also the symptoms are a grouping and as many cases on this forum show, it can be many varied combinations of these to varying degrees.   I believe the confusion again is that HSP is not really a particular disease.  It is more like a classification of these types of symptoms that results from the immune system response disorder.  

What they refer to as causes are what bring about or triggers it.  It isn't explaining the root of the cause like why the body has changed to now respond this way incorrectly.   I say this because it isn't something from birth, it is something that develops and can possibly go away.  You can't say definitely what it is or what it isn't, only describe what wrong is happening.

"Causes

In Henoch-Schonlein purpura, some of the body's small blood vessels become inflamed, which can cause bleeding in the skin, abdomen and kidneys. Why this initial inflammation develops isn't clear. It may be the result of the immune system responding inappropriately to certain triggers.

Nearly half the people who have Henoch-Schonlein purpura developed the disease after an upper respiratory infection, such as a cold. Infectious triggers may include chickenpox, strep throat, measles and hepatitis. Other triggers may include certain medications, food, insect bites or exposure to cold weather."

1. HSP is indeed Trigger from the immune complex deposition on small blood vesseals - is either by one of the three causes 

      A) INFECTIONS

     B) CHEMICALS

     C) DRUGS

There is proof that this is indeed the cause. I have many medical professional publications that prove this fact. 

2. The sysmptoms are not too much varied, although there are variations; but generally classified in the  those i described above. 

 There is also one that does not cause abdomical pain, but only causes arthralgia (joint pain). 

Thenr there is one that causes abdominal pain with mild diarrhea, but does not cause joint pain. 

3. HSP is a disease and classified as D69.0 one in ICD10-CM. 

4. The cause is also called autoimmune disorder. It is the way that immune system responds incorrectly.  Yes, it is not by birth or genetic disorder.

5. Why this inflammation develops is absolutely clear from 1,2,3 and 4 points above.

6. It is not "maybe". It is the result of immune system responding inappropriately to triggers mentioned in point 1 above. 

7. Everything you mentioned... URI/colds, chicken pox, strp, measles and hepatitis are correct and fall in INFECTIONS category as triggers for HSP. 

  

In fact, a dermatologist should know more about vasculitis than a GP because HSP is mostly a skin rash/burning sensation with dark/violet skin. 

But the problem is HSP is very rare, only 1-3 out of a million get it as Adults.

In Children, it is like 5-10 out of 100,000. 

You are one of the few who got lucky to get prednisone from someone other condition. Not everyone have been lucky or could consult a doctor who know this problem. Thats why it is frustrating and causes progressive disorder (of abdominal ischemia and kidneys), if not treated timely. 

 

So I'll give you another example as to my point.  Again, my point is that an infection is not a cause, it is a trigger.  Yes it causes the immune system to act, but having an infection was not what caused the body to activate the immune system such that it over reacts incorrectly.  Can you tell what I'm getting at?  Everyone gets infections but everyone does not get HSP and that is my point.  Infections don't cause HSP.  Infections are a trigger to bring on HSP if at some point the body from whatever the unknown cause is, responds incorrectly to the infection.  I apologize if I can't explain it any better, but what I'm trying to say is a cause of why you develop HSP is something more at the core then what triggers the response.

Also, the other reason I say this is because in my case it does not fall under any of your causes.  Even an allergy is what you refer to as a cause and triggers my HSP, however, I ate lots of chocolate when I was younger and throughout my life and didn't get it then.   Again, my point is that something either damaged or mutated something within my body so that I became allergic to chocolate and that triggers my immune system and leads to HSP symptoms.  Also, now because I completely avoid chocolate, when I get a cold, I don't get HSP.   I'm belaboring the point because it is significant.  You need to be able as a scientist to understand the very root of the cause, just like how they are genetically modifying  blood cells (bone marrow) now and curing sickle cell anemia.  You can't just say the cause of sickle cell anemia is blocking of sickle shaped cells in the blood vessels.  You have to go back to the root and determine why through the genetics they become shaped like a sickle instead of a smoother round shape.   With all the different kinds of drugs given for different things I'm not sure if those can cause the body changes or if the genetic mutations can also play into this but when this is understood, the cause will be described not as infection, but in terms of the genetics of the immune function.

Chocolate is a food chemical. 

You can see that chemical /food is a trigger that can cause HSP, an autoimmune disorder. 

Your other example about sickle cell anemia is comparing apples and oranges here.

This is getting a bit silly. LOL.

Yes, chocolate is indeed a chemical and

": A chemical is any substance consisting of matter. This includes any liquid, solid, or gas."

It isn't helpful being that broad defining things and if we can't get to the understanding of the actual biology it is not useful to continue my particular thread of discussion. I do appreciate all the support you are giving people so I'll do my points.

It is broad, but it is true ... Food chemicals cause allergy to some people and does sometimes trigger HSP. 

And that is the reason, this is so rare disease, and that many doctors are unaware of its pathological implications in medicine that they are not sure how to diagnose let alone treat them. 

So most of them do is treat it symptomatically, like give ibuprofn, etc. without understanding the cause, which is really an autoimmune dysfunction. 

 

My friend is a Russian doctor and when my son got HSP I asked her opinions ( she lives in Russia and I am in Norway). She advised me to keep him away from the chocolate and the red berries while he has HSP. She said in Russia they give the patients the special diet as well as a part of treatment.For me it was so strange to hear that I just ignored the diet part advise . But now when you mentioned it , I think maybe Russian doctors are right.