[mkerpics]

Hello Everyone
I just joined a few weeks ago and have already gotten a lot of great advice. THANK YOU! I've been looking everywhere to see any mention of a disease called Urticarial Vasculitis. This is what I suffer from in addition to Lupus. It is mainly seen in patients with Lupus and is VERY rare. There are two types of this disease based on whether or not you have complement deficiencies (C1q,c3, and c4). Anyways...I have never seen anyone else mention it and I thought I would ask if anyone had it or had even ever heard of it. I have the systemic form of the disease as well but some of its worse symptoms are hives that can cover your whole body and intermittent episodes of angioedema. My lips swell up out of no where about twice a week. I used to have wheezing episodes as well but I have been free from that for a couple months. I've had this for about 21 months although I had many months of a spontaneous remission. I have been told by my rheum that there is little information available online b/c the disease is so rare. I have only been able to find case studies but no real people. I would love to find someone else that has this or has even heard of it.
Thanks
Mary

 

[Clare.T]

Hello Mary

I have heard of it yes and I think there have been a couple of people on the forum with it but I can't remember who and don't know if they are still active members.

I first heard about C1Q when my daughter had an episode of angioedema which I mentioned chattily to the rheumatlogist I was seeing at the time.
He suggested she get tested for complement deficiencies and told me about their association with " lupus like illness". The first testing got loused up because the samples were incorrectly stored and as she had no further episodes we didn't pursue it.
However she has recently started having very bad gastric upsets and asked her GP about the possibility.

I have read that lupus due to chronic complement deficiencies is very rare.

There is an article about hypocomplementemic urticarial vasculitis on emedicine and also one on DermnetNZ, if you google the term. There are several pictures on Dermisdoia. General links to those sites on Symptoms forum, pinned notice about Skin and Lupus

How is it responding to treatment ?
It would be very nice for you to find somebody with the same, to compare notes with.

Bye for now

Clare

 

[cath]

Hi Mary,

Nice to meet you.

I have urticarial vasculitis as part of my SLE too. Actually it was that that sent me to the doctor in the first place. I was going mad with the itching.

It took several wrong diagnosis before my rheumy eventually renamed it. We went through primary billiary cirhosis, allergies, idiopathic utrictia, mastocytosis...

These days I mostly forget I have it, appart from being dermographic. It is well controlled for me with a combination of pred, plaquenil and an antihistamine. I also get wheezy without the meds. You are the first one to mention this as a symptom:wink2: . i've previously had anaphylaxis and have asthma, so a epipen and inhalor are part of the pharmacy in my house, but fortunately are very rarely needed:blush:

How are you coping, and how is your lupus in general?

Looking forward to getting to know you better,

X C X

 

[mkerpics]

Wow!
Thanks Clare
I can't tell you how excited I am to meet you! I'm sorry that you also have this ailment but grateful to have someone that can relate. I too have had anaphylactic reactions out of now where and have had to use both epipens on the way to the hospital (the first one quit working after about 20 mins). I also have the dermatographism. I have hypocomplementic urticarial vasculitis (c1q, c3, and c4 deficiencies) with systemic symptoms. I have joint pain/swelling, fevers, fatigue, and significant angioedema. I'm on Pred, plaquenil, Imuran, as well as a TON of antihistamines. I just started the plaquenil and Imuran in October. So far I have not had much of a response from the medications but I've had to stop the Imuran a few times b/c of other illnesses. To be honest....I'm not quite sure if my joint pain, fevers, and severe fatigue is from the UV or Lupus. My rheum insists that it's from the UV so I have to take her word. So....were your complement levels also deficient at the time of your diagnosis???I've been told that this version is more difficult to treat.
Thanks so much for your response
Mary

 

[cath]

Hi Mary,

Nice to met you too:blush: .

It sounds like you have had a harder time setting symptom control than me .

I've had a low C4, an boarderline C3, but probably due to consumption (flaring) rather than a congential deficiency. My levels are nice and normal at the moment:blush: .

I'm never sure exactly what symptom is caused by what, and I supect the docs often aren't either... for example I have petechia, purpura and thrombocytopaenia etc from time to time, but whether it is secondary to the lupus, APS or vasculitis, I haven't a clue. It responds nicely to pred for me, so I am happy to be grateful for a solution:blush: .

How was your lupus diagnosed?

Bye for now,

X C X

 

[mkerpics]

Hi again Cath

I was just recently diagnosed with Lupus in October after being referred by by allergist for 18 mos of severe hives and angioedema (with one period of remission). I tried every medication imaginable (up to 23/day) including high doses of prednsione but my symptoms still persisted. Also, I developed Hashimoto's about the same time and had intermittently low WBC counts. I also had ITP about two years ago with a platelet count of 7,000. My first symptom however was a low platelet count during my pregnancy 7 years ago and chronically low blood counts off and on since then. My hematologist took very good care of me though. He followed my ANA's but they were always neg so he did not think I had Lupus. I've had muscle aches, fevers, and fatigue in spurts that lasted a month or so each time for the last 4 years. I stopped going to the doctors for them about 3years ago b/c nothing ever turned up. I really wasn't surprised about my diagnosis b/c deep down I figured that I was probably developing Lupus. I am a Registered nurse and already knew a little about autoimmune diseases. I started seeing a familiar pattern when each of my doctors periodically checked my ANA (always negative until recently). I assumed they were checking for Lupus but no one really said anything. I started doing a lot of research on Lupus and realized that Lupus usually evolved so I figured it would happen one day. I must admit.....I was angry but I got over it in a few weeks. So here I am...living and learning about Lupus. So....the rheum did a screening for everything and my ANA was positive with speckled pattern. My C1q, c3, and c4 were very low. My white count was a little low and my urticaria turned out to be UV which is usually only seen in people with Lupus. I also often have the malar rash which I always though was just a flushed face. My rheum said that it was a malar rash so I didn't argue. That about wraps up the last 7 years of my life...except that I had two more kids with two more high risk pregnancies....now I know why!
Sorry so long...maybe it's the prednisone. lol
Mary

 

[ROLA]

Hi
How are you mkerpics ??
Hope u feel great & healthy
I have had vasculities scince 3 years ,,,its not urticarial ..
I mean it's not itchy ...but it's ugly ...very ugly ..
actually it is the reason i went to the doc for ....!
it started on my right leg ..then the other leg ..
i dont feel confident ! i cant wear short skirt any more....!

what do you do to cover it up?
do you think it can start on my hands ??(hope not)
Does this ever disapear ??


sorry for my questions ...&sorry if thier is any thing wrong with my english


Regards
Rola


Rola

 

[mkerpics]

Dear Rola
Urricarial vasculitis is very different than most types of vasculitis. I get hives (inflamed, itchy, red, welps) all over my body that last for approx 24-48 hrs. Everyday they are in different spots and sometimes they cover most of my body including my hands, face, and lips. There is nothing that you can do to cover these up because they can become large and inflamed. I mostly just avoid public when they are bad. My family and neighbors are used to seeing me like this so I don't try to cover them. I just dress accordingly to avoid the inevitable stares (we all look twice at something that looks unusual). I wish that I could be of help to you but my rash seems quite different. I have noticed many other posts from people with different types of vasculitis however. I would post a new thread under related conditions called Vasculitis....I'm sure that you will get many responses. There a a lot of caring, helpful people on this site that would love to share their experiences and ideas.
Good luck to you.

Mary

 

[londongirl]

me too

hi there, just to say quickly, yes i have this, and like the other member this was initially the start of the lupus diagnosis trial, of which i am still going through,,,, i use the blow up like a alien, my forehead, lips nose mouth eyes etc, at one stage i looked like the lion king,,,,, it is really awful, and uncomfortable and painful aswell, i also have had this spread to other parts of my body aswell such as my hands and fingers, arms,,, i firstly had to go through all the skin patches etc allergies test e tc,,,, but i knew this was not an allergy,,,, since i have been taking the plaquenil, and anti histamens, things have improved greatly, and touh wood have not flared for a while know, even though i have felt tingling in lips and face etc,,,, i also have had anaphaleptc shock, which is use to carry and still do an epi pen with me, but gad to say have not used it for a long time, so at least you know you are not alone in this, and hopefully things will improve for you too,,,, so take care, love londongirlxxxx

 

[mkerpics]

Dear Cath and London Girl
I'm just curious....could you live a normal life during the time that you had angioedema? I have had this condition for 21 mos (with several months in remission) and find it very difficult to make commitments. I have had constant angioedema flares about 2/3 times a week despite meds since April. My doctors have tried lots of medications. They're still hopeful that they can find the right combination of meds.
I can't work b/c the angioedema has happened at work and the swelling can take up to 12 hours to go away after taking all the meds. Stress seems to make the swelling even worse. Everyone I really care about knows/sees my condition most of the time so I'm mostly OK with is. I have always been a very capable and reliable person and now I'm not so that is frustrating. How long did your angioedema last?
Thanks
Mary

 

[Mootie]

Hi Mary

I am so excited to see your post!!!! I have urticarial vasculitis and I have never met anyone else with it. I have never been able to share with anyone this unusual affliction. It is a very isolating condition.

I have had delayed pressure urticaria for 12 miserable years. Along with it I have angioedema, joint pains, stomach problems, asthma, rosacacea (malar rash maybe) food intolerances, polyps, fungal sinusitis, osteomalacia, osteoporosis and other things I have forgotten. I have had cranial polyneuritis caused by the vasculitis as it damaging my nerves and organs.

I have been on plaquenil for two years now and it has changed the severity of the symptoms. Currently I have active vasulitis and I saw my Immunoligist and Rhuemetologist at the same appt last Monday and they have started me on Inocid in addition to my other medications.

I have been seeing a Rhuemy now for 2 years since my immunolgist of 12 years referred me. So far I don't have a definite for lupus but I am on all the drugs.

Nice to meet you and hopefully we can learn from each other. There is not a lot of info about UV and there is no other support site available either. I am not sure if I am in the right place but a lot of the lupies have similar problems and can relate and this has been a great help to me.

Sincerely,

Mootie

 

[Mootie]

Hi Again,

I have just been reading some more of the other posts to get some more info on what others have been going through. I too have been tested for mastocytosis, hep c, and even though I have not had an anaphylactoid episode I have had anaphylaxis.

The biggest problem with the angioedema is with my feet. For a few years it was difficult to find a pair of shoes that I didn't react to. If I brought new shoes I would have a week of **** while I broke them in. Since I have been on plaquenil I can even wear shoes that cover my whole foot. Still can't wear runners tho. Consequently this condition changed my life and after 8 years of working full time I went part time and then progressed to full disability retirement.

Anyway, I don't want to exhaust you but I want to share with you the medications I am on as I am the best I have been, perhaps we can compare our treatment.

Daily

400 Plaquenil
180 mg Telfast (fexofenadrine) Allegra in the US
2 x Ranitidine
75 mg Endep at bedtime with 50mg Phenergan for bad nights
Calcitriol x 2 - Vit D Deficiant
Seretide daily
Ventolin as reqd
Inocid x 2
Celebrex as reqd

It has taken many years of trial and error to get the correct combination of drugs, it has been a real challenge.


Mootie

 

[cath]

Hi Mary,

I'm sorry to hear that you are still suffering so much despite medication.

I guess I am very fortunate, in that I'm relatively asymptomatic so long as I take my meds.

On the occasions where I had to stop antihistamines for skin patch testing/dermatology appointments/allergist etc, it was absolute ****. I couldn't work, put clothes on, sleep, do anything except go crazy. Without meds, I needed pretty regular salbutamol to keep my airways open. Fortunately I've never had to do it for more than 2-3 days at a time, and now I'm diagnosed find that health care profesionals believe me, and don't want to see it for themselves.

All the best,

Take care

X C X

 

[mkerpics]

Dear Cath

Thanks for all of your help. Please let me know if anything changes in your condition. It's always nice to know that there is someone else that can share their experiences. This is a very isolating disease. This website provides so many opportunities. I know that our doctors don't have the time to discuss the details of how lupus and other autoimmune can affect your life. I guess that's why we are all here.

Dear Mootie
I'm so excited that your condition sounds so similar. I can't decide if I have dermatographism or delayed pressure urticaria. I think I've been diagnosed with dermato.....but it seems like delayed pressure. Even wearing undergarments can create a problem. I can't even sit/sleep in the same position too long. I have hives in the shape of my bikini line every morning. Maybe that's TMI....Anyways...my situation sounds very similar to yours. How was your UV diagnosed? Have you met many of the diagnostic criteria for Lupus? I've just heard that UV is normally only seen in those with Lupus.
Here's my meds

Nortriptyline 75mg
Allegra 180bid
Prednisone 20mg
Synthroid 100mcg
Imuran 125mg
Plaquenil 300mg
acyclovir 400mg bid(recent shingles outbreak)
albuterol prn wheezing
fosamax
atarax 25-100mg four times daily (dose changes based on severity)
zyrtec elixir 30mg prn angioedema

Our meds are very similar. My doctor wanted to switch me to cellcept recently but she's feels that it's too risky since my recent shingles outbreak. I guess the drug can cause severe shingles. I am increasing the Imuran to 150 to see if any improvement. If not...we will begin the process of getting authorization of rituxan.

It's good to chat w/you. Where do you live?
Mary

 

[Mootie]

Mary,

Thanks for the reply, good to bump into you on here!!!! Yes we are on the same meds but I try not to take too much predisnone as I always have a reaction when I taper.

It does sound like you have delayed pressure urticaria. It is the most difficult to treat and does not respond well to medications. Incidentally, my daughters have urticaria, one cannot break into a sweat (cholinergic) and the other one cannot tolerate wearing any pantyhose or anything that clings to her skin. I think we must be from another planet.

Yes, I can get hives from sitting, underneath my bra straps, using scissors or cutting sandwiches but there has been a big improvement since I have been on plaquenil.

My diagnosis came 2 years ago when I lost the ability to swallow and my ENT discovered that I had a paralysed vocal cord. I had a few days of hives around my neck and the angioedema reduced the blood flow to the cranial nerves, the 6th, 8th and 10th (vegus nerve) I think. I lost the use of my shoulder as well. It took about a year to recover fully from this and my voice was reduced to a whisper. I could not eat normal food for months. This was a very rare and unusual event and because of a high sed rate, the urticaria, UV was the dx I have been given. I had CT Scans, MRI and gave buckets of blood.

Since then the urticaria has been very agressive, like someone is pouring boiling water on my skin. I have lumpy bruises wherever I get hives. I get a lot of infections and headaches and I worry about having a stroke although my blood pressure is always low.

Luckily, I have an excellent team of Drs. In April 2006 I had a Nissan Fundoplication for severe gastric reflux. The operation was a complete success and I feel a lot better. The reflux seemed to get very severe when the urticaria was at its worst. It is such a weird thing to have and because most of my reactions happen at night a lot of people don't believe that there is anything wrong with me. I don't worry about any of that now as my husband is my witness and my Doctors have observed my condition when I have been in hospital.

There are some great articles on DPU and a Dr Malcolm Grieve from England is a respected authority on all the urticarias. There is not much around about UV though.

I have regular blood tests and although my ANA is spiked 1:1380?, (not sure if this is correct, but something like that) I don't have a dx of lupus. My CRP is usually elevated as is the eosinophils, which has been a concern. The other tests I have are for ANCA, C3 and C4, not sure what ANCA is tho. Mostly these are normal. It is interesting to note that I have had a good response to the plaquenil.

I do have a fair amount of pain in my body and there is no way that I could stay in bed all day as I will get bruises and pain so there is no rest to be had. Even taking off my clothes used to induce a flare, bumping my skin or stroking would bring on the hives. I always have a flushed face, cannot drink any alcohol and certain foods make me worse as well. I think I must produce too much histamine. I cannot take estrogen either as it makes things worse.

Mary I don't want to bore you with all of this but perhaps you can PM me anytime, I might even be able to hunt down a few articles to send to you. BTW I am from Australia.

I used to think I was the only person in the world who had such a strange affliction, well it seemed like I was til now. Looks like there are a few others in this group with the same problem. Hopefully we can support each other and maybe our Drs can learn something too.

Regards,


Mootie

 

[ROLA]

Dear mary
thank you for the inf. you gave me about urticarial vasculities
it's really nice to share & talk here in this site !
hope you have a healthy life ...



Regards
Rola

 

[Jynnieneal]

hello

Hi Mary,
It is nice to meet you:hehe: I too have Hypocomplimentic Uticarial Vasculitis. I was diagnosed with it this past September, but suffered with hives for the last four years. I know exactly what you mean by there being lack of information about HUV. I have encountered the same problem. I also know what you mean about your lips. I always say Hollywood actresses would die to have the lips I wake up with some mornings, and I get mine for free. Humor is what gets me through my days. I also was recently diagnosed with SLE. Nice meeting you.

Jyneal

 

[mkerpics]

Dear Jyneal

I am so excited to meet another person with this condition. It will be a real asset to all of us to keep in touch with one another. It seems that a lot of the new treatments for this condition are experimental so we can share our experiences with one another. I think that my rheum has treated only one other person for this condition and she was very sick and hospitalized so her treatment was much more aggressive.
I'm curious about your meds. Are they similar to mine? Also....Are you working? I'm just curious if anyone can hold down a job with this condition and if I will get well enough that I can return to work. It is very frustrating being only 33yo with this condition. I've been at home with my children for the last 7 years and worked only part time. Before that I worked only a few years full time as a Registered Nurse after college. I've been excited about begining a career after my kids are all in school and I'm wondering if this is a reasonable expectation.
I tried and tried to work this past year but my swelling just occurs too often. I would try to take larger amts of prednisone on the day before I worked just to make it through oneshift a week. I was on prednisone a lot more than I should have been just to work part-time. I finally gave it up but I'm hopeful that I can return again one day.
Is your c1q also deficient?


Mary

 

[Jynnieneal]

Hi Mary,

I agree we should stay in contact. My Rheumotologist or Nephrologist, have never treated anyone with this. They tell me not to worry they treat the lupus and the HUV the same way. Currently they have me on Prednizone, Cellcept, Lisinopril, AcipHex, Iron, Fosamax, and Calcium. I think I got them all. They reduced my prednizone to 40 mg per day, I was taking 60mg. They are weaning me off and having the Cellcept replace the prednizone.

I have had these hives and swelling for 4 years now. I don't know if other people continue to work with this condition, but as of now I am still working. However to be honset with you every day it gets a little harder and harder to get to work. There are some days my face is so swollen I can't even open my eyes. Others the hives and swelling are hidden by my clothing. I have found the hives lessen during the winter months, and flare up terribly during the warmer months. All I can suggest is you do what your body tells you to. If I could find a job that I could work from my home I would do it in a heartbeat.

I am not much older than you. I am 36 and I have 2 children also, but both of my boys are in school.

You asked about my Cq1 being deficent. I am not sure, I am going to my rheumotologist Feb and I will ask.

Take Care
Jyneal

 

[mkerpics]

Hi again
I'm just wondering if you're improving on the Cellcept? I am presently on Imuran but my physician was considering switching me to Cellcept. I've been on Imuran for about 12 weeks now with little improvement. I get shingles occasionally so she's worried b/c shingles is often associated with cellcept. I'm so sorry you're on so much prednisone. How do you stay sane?
I'm down to 20mg but I take more on the days that my lips swell.
I hope we keep in touch. I'm new at this too mut I think we can just PM one another.
Take care and stay healthy
Mary