Hypersensitivity/Leucocytoclastic/Urticarial Vasculitis

Heart health and blood vessels
1

Hi,

I have had Urticarial, hypersensitivity vasculitis for 4 years now in November, trying to get a diagnosis has been a nightmare. I am 45 years old. For the first 2 years I was diagnosed with idiopathic urticaria and tried on loads of medications. None worked so they sent me for a biopsy this confirmed Leucocytoclastic vasculitis.

I have pressure urticaria on my feet and if I wear anything with a waistband or shoes I swell up and itch- which means loose elasticated waists and trainers or flipflops - attractive!

I also have angioedema which makes my throat and face swell up - even more attractive!!!

Still trying to get medications right, so far I am on Fexofenadine, double dose 360mg, Tranexamic acid, hydroxyzine, various other antihistamines some prednisolone, back at hospital tomorrow and I think I am in for Dapsone to add to my collection.

I have never found anyone else who has what I have so Im soooo happy to find this today. I dont seem to have the red spots on the legs, I get giant urticaria all over my body and they itch and burn like mad, especially on the inside of the top of my arms and inside thighs. If I lean against anything I get weals when I am really bad. All the meds keep it sort of under control but I was poorly at Christmas with flu and it all came back with a vengence and it has taken it 6 months to start to calm down again, so the lady with the bladder infection, that could be why you feel more poorly at the moment.

I have read everything I can find on this condition as even the hospital dont seem to know quite where it is going to go. I have regular bloods but they cant get my inflammatory markers down even with all the meds yet.

They wanted me to take chemo type meds but I said no so I have started on all the ones with less side effects and now they are pulling out the big boys to try and get it all under control as none have worked.

I developed gastro problems this year and where I havent had a camera isnt worth mentioning, I am bleeding internally but no-one can find where and why? The doctor and I think my insides swell and bleed but everytime they try and catch it on camera it has stopped as it is intermitent. I get fatigue all the time which I know is part of the condition and at times complete exhaustion so I go to bed as soon as I get in from work. My hair falls out and I get night sweats at times and as I live in Yorkshire its not due to nice sunny weather!

You need to be strong with the hospital and your doctors and research everything they give you. I do loads of research and find this has helped me make decisions on my meds. I have a fantastic GP though and she really helps me manage my condition.

My best advice is to take it easy on yourself, dont do too much if you can help it, if you get stressed or work too many hours you will know about it.

If you get sick with something else you will feel generally crappy for ages after. Immune system knackered and I work in a large office so hand gel is a must in winter to try and keep the bugs at bay. Find a sympathetic doctor.

I have felt completely alone with this condition as I have never come across anyone else with it who understands quite how rough you feel each day. So thank you ladies for posting your comments, you have made my day!

2

Hello

I am 24 and have been diagnosed with urticarial vasculitus.

I feel awful! Whilst I was pregnant it went!

My symptoms include chronic urticaria. Abdomal pain. Joint pain. Chronic fatigue. Photsentsivity.

Hot sweats.

Currently I am taking prednisone. Dapsone. Fexodenadine. Ratinidine. And hydroxyzine hydrochloride

I feel so poorly! Any suggestions would be welcomed thank you

3

Hi,

I'm a 32yr old female and I've been suffering with the disease since I was 24 and apparently it is more

common in women than men.

I was only diagnosed with urticarial vasculitis in 2010 after 3 years of being told it was idiopathic angioedema with urticaria and there was nothing they could do.

I have also felt very alone with this diagnosis as so few people suffer with it - there is the vasculitis UK

website which is helpful and they do have regular regional support groups which can be helpful.

My symptoms include;

Dark red spots and blotches on my skin - usually on my face

Urticarial rash and photosensitivity

Angioedema - for which I carry an epipen

Abdominal and joint pain

Hot sweats - especially at night, which really disturbs my sleep

Periodically I have low C4 complement when I have a bad attack.

I currently take cetirizine 60mg daily (normal dose is 10mg daily) chloroquine 250mg twice daily and

prednisolone when I need it. I've also been on 2 month long pulse doses of oxytetracycline for the rashes I

get on my face and that seems to help.

I do find that I tire easily and i'm more susceptible to infection.

My consultant is Dr Hillary Longhurst at the Royal London Hospital and she has been fantastic since she took over my care in 2010.

I find that if I push myself too hard then I suffer the consequences so I try to listen to my body when it's telling me to relax and take it easy. Anything that puts added pressure on your immune system can affect the

disease. Also things like the type of contraception that you are on (for women!) can affect it also as hormones do have an effect, hence the improvement when pregnant.

It would be great to hear from other people and what they find helpful.

Thanks

N

4

I too have the same as you Nic, I carry a epi pen. Get chronic urticaria. Angiodema. Joint pain. Photosensitivity. Abdominal pains and chronic fatigue. I have now been on steroids for nine months. I am gradually reducing now I have started azathiaprine. I take loads of anti histamine too along with dapsone. It's just awful I literally feel I have been run over by a train. I just wish my body could think it was pregnant and then it would go away. Have u found anything helps you!

5

I am a 53 year old female and was diagnosed with urticarial vasculitis 20 years ago. I suffer with most of the symptoms that others have posted. I was originally diagnosed by Prof. Greaves at St Johns Hospital in London by blood tests, biopsies etc. The only treatment I have ever been given is antihistamines. Previous comments of those that have been diagnosed much more recently have been a real eye opener for me - who haven't I been offered these other treatments when today I sit here with hot rashes all over my legs, feet, arms, hands, head, waist and backside! I am currently experiencing what I call a flare up, which leaves me swollen, sore, in pain and feeling very tired and low. What causes it - it was put to me by Prof. Greaves that it was my body's way of saying I can't cope with life and if I didn't have the rashes I probably would be heading for a breakdown. One thing I do know is that if I drink red wine it doesn't help or is it that I have a drink (very occasionally) to cheer me up but thanks to all above I am now going back to my doctor and demanding the latest treatment. Thanks smile

6

Hi Alice and Paula,

It's really good to hear from other sufferers and to find out that you guys suffer from the same symptoms as well.

Paula - I changed to a progesterone only pill on the advice of my consultant as this is better for vasculitis suffers and it has helped. My consultant also thinks that steroids don't really work all that well for the vasculitis and they only really help in short courses to reduce the length of a flare up. I also try to wear a sensitive skin sun block when I'm out in direct sunlight in the summer - not great for the tanning but it helps!

Alice - I have been through quite a few treatment regimens and my current one is the best yet. I used to have 4/5 attacks a month, sometimes ending up in hospital, but since going on my new meds a couple of years ago I get less attacks with reduced severity.

The only things I find that help are rest (sleep) and taking care of myself. I do think exercise helps, even though

a lot of the time you feel too unwell to do anything!

I'm going to go to one of the vasculitis UK regional meetings just to try and get as much info as I can.

Best wishes

Nic

7

I am also 53 and have just been diagnosed today with Urticarial vasculitis. Am a bit shell shocked as I expected my rash to be allergy related. I am taking prednisolone and Fexofenadine to keep me comfortable. Today my Derm consultant mentioned Dapsone. Any advice from anyone gratefully received.

Will this go away? Googling vasculitis has just scared the wits out of me, is this different?

8

I am to scared to go on the pill incase the hormone changes set me off worse. I am taking dapsone.

Prednisone - which I am trying to reduce to come off them have been on big doses for the last 91/2

Months. Fexafenodine. Ratinadine. Levoceterize. Ad cal and azathiaprine

I have found improvements since being on the azathiaprine. One of the hardest things is the fatigue!

Do any of you think u know what triggered it?

I am on fb and they have a vasculitus page. The Stuart strange page it is vasculitus uk.

There's a website too with lots of info.

9

its strange that so many us start to experience symptoms related to vascultis at age 24. Its frustrating to know that they can't find out whats causing and it really takes a toll on your life emotionally, phsyically and mentally. I hope and pray for everyone to be healthy as long as they are alive. 

10

Hi.  I'm 39 year old female, live in Chicago.  I was diagnosed with Urticarial Vasculitis about 2 years ago thru biopsy.  The 6 months prior to that were the worst being that i had no idea what was wrong with me!  It caused trips to the ER, inability to work, depression, and I looked like a hot mess!  

I'm actually sitting on my couch with ice pks on my legs at this moment because I have been dealing with a pretty aggressive flare-up these days.  

I had many rounds of prednisone, which I try to avoid because of all the side effects that long term use can do.  I did a good 18 months of a daily cocktail of:  40 mg ceterizine, 100 mgs dapsone, colchicine (I can't recall how much but it was minimal).  I ran out of dapsone and colchicine in August 2014 and decided to see what happens.  At this point, all of the meds were causing a whole new world of problems that included severe pain in my feet and loss of my menstrual cycle.  Honestly, I'm just sick of dealing with it.  Fortunately, I have found that my condition is not as severe as it used to be.  

6 months later, I have a regular period again, I can work normally, and I am somewhat control my symptoms with benadryl and 40 mg of loratadine (instead of ceterizine).  Ibuprofen is often times my best friend because I do get alot of body soreness whenever I have the hives.  The worse the flare-up, the worse the body soreness.  Sometimes I feel so sore you would think a personal trainer beat me up at the local gym!  

I have found that a nice cocktail of vitamins has helped.  I take a daily multivitamin, vitamin C, biotin, and turmeric supplement.  The turmeric is great for it's anti-inflammatory properties.  It doesn't help the hives per se, but it has done wonders for the swelling i often experience in my hands and feet.  

I am going to see a new primary care physician on the 8th so I'm hoping that a new doctor with fresh perspective might give me other options.  This is so rare and it truly is life altering.  I will let you know what i find.  

Best of luck to all of you!  at least we know we are not alone smile

11

I hav e an additional "Differential" Diagnosis of Lichen Planus  and Nodular Prurigo/Prurigo Nodularis. The itching and pain is excruciating!!!!

12

Hi

I was diagnosed with hypersensitivity vasculities/leukocytoclastic vasculitis in 2009. I was 24 years old then and was about to get married.

I am an Asian, from Philippines. I couldn't understand at that time how I got it. No one in my family has it. Only to find out that the cause was unknown.

Mine started to appear as a rash. Then turned to some sort of a black scab on my right foot. I thought I hit somewhere my foot and so I got a wound. It became itchy after the scab was removed. Then a lot more started to appear. I almost postponed my wedding because of it. Initially, I consulted a derma and gave antihistamine for it coz he thought it was just an allergy. Then I stopped my antihistamine and more rashes started to appear until my right foot got swollen. I went to see an internist and did a lot of blood labs, all were okay then.

I was diagnosed by a dermatologist thru a biopsy. I was given immediately prednisone (tapered) that run for about 2-3 months. Rashes stopped appearing months before I got married in May. However, I already have brown marks of my previously then rashes on my right foot and even the left foot.

After that I was only given diprogenta for my rash but stopped it also after some time. I was okay then when I had a flare up in 2012. It was my worst attack as I was already having difficulty while walking. I decided to see an Immunologist/Allergologist that time. I was given prednisone for a months but since no improvements and my wbc were high as well as my neutrophils, I was given Colchicine, Aspirin and Iterax and Solcoseryl Gel for my rash. My wbc, esr and neutrophils went down and my fresh/open rash started to dry up, then got better. My maintenance meds since then was Aspirin and switch to levocetirizine. It was also that time that we found out that my trigger were food allergies and allergic rhinitis.

With my avoidance to my allergens, I got better. I would normally have rash just few days before I get my period, but it disappears immediately.

Now I'm still in aspirin and cetirizine to control my allergic rhinitis. No flare ups so far. Also I'm currently 10 weeks pregnant. Hopefully I'll be fine the whole duration of my pregnancy. So far I would get a rash just on my right leg and dries up faster. Next month I will undergo series of lab test to check everything since my immunologist informed us that pregnancy may trigger my vasculitis and my condition may be considered as high risk. Hopefully me and my baby will be fine.

13

I know it's an old thread, but I couldn't find another...thought I'd share...

Here’s my story, and it seems like I’ve got away easy compared to you lot

I’m a 50 y/o male in the UK with no health issues.  The first week of August 2016, I had bizarrely itchy outer ears.  The following week, on getting off a plane my feet were swollen, knee joints painful and Achilles tendons very sore.  I had to be helped out of my seat

On arrival home, my hands were phenomenally itchy and I had spots appearing on the outside edge of my hands.  Also, every time I blew my nose there was blood in it.  All these symptoms worsened over the next 4 days, along with spots appearing symmetrically on the inside of my knees/buttocks/lower trunk and my hands starting to swell.  The spots were the least of my concerns given they just looked bad but weren’t at all itchy.  GP did bloods and urine – no inflamatory markers and my kidney and liver function were fine.  I self-medicate with antihistamine and ibuprofen.  I saw a dermatologist towards the end of August with a general diagnosis of Leucocytoclastic Vasculitis (LCV), possibly a more specific one of HSP (and perhaps Wegener’s Granulamatosis given the nose issues).  Best bit of treatment advice was to wear flight socks, which helped with the foot swelling, but made them smell horrifically.  Generally, symptoms waxed and waned on a 24 hour cycle

After seeing my GP again he said there my blood test showed evidence of a recent strep infection which can sometimes be a precursor of HSP, so in his mind, the HSP diagnosis is confirmed.  I start on Naproxen (and Omeprazole) which provides substantial joint relief.  Just before that I suffered a bout of angioedema on my eyelids, face, lips, tongue, gums and throat – not nice.  GP was worried this was an allergic reaction to the Naproxen, but the swelling predated this

Roll forward to December and all the symptoms still wax and wane – ie. it hasn’t gone away.  GP declines to put me on steroids as his (single previous HSP) experience is when you come off them, the symptoms return.  I’ve been having bloods and urine analysis on a 4/6 week basis.  The worst part for me was the facial angioedema – it does sometimes seem to be triggered by temperature changes.  I’ve been wearing compression gloves for the hand swelling.  GP refers me to a more local dermatologist.  After contacting the original one he runs a battery of recommended blood test so I can take the results to the new one

The new dermatologist does not believe I have HSP for 3 reasons: a) she’s not persuaded by the prior strep infection evidence, b) it hasn’t cleared up, and c) I have low blood complement levels.  New diagnosis of Hypocomplementemic Urticarial Vasculitis – looks like a more chronic condition than HSP.  I have biopsies.  I get referred to a rheumatologist – she’s the auto-immune expert.  She agrees with the HUV diagnosis.  I start on prednisolone 20mg and over the next few weeks the symptoms decline substantially (apart from the face swelling after the first dose – yikes!), but not completely.  A bout without Naproxen proved a foolish adventure

Biopsy results come back confirming vasculitis (seemed a bit pointless really). I am started on Microphenalate Mofetil, (500mg a day rising to 1g a day) an immuno-suppressant, with a view to reducing the steroids and seeing whether this resets my immune system.  7 days in, I feel fine, even started to exercise again, no compression socks or gloves

Saw an ENT Consultant yesterday, nasal douching and steroids drops twice a day.  Some very unsettling words were said about the long term effects of vasculitis in the nose

Worth noting that over the 5 months, I’ve piled on the weight largely from my lower activity levels.  I’m sure the steroids haven’t helped either

Thank goodness for company sponsored private healthcare…

I'll update when I have something else to add

14

Hi, I read your post and wanted to ask how things were going. I'm also 39 and live in Chicago. I moved here two years ago and last year, I broke out all over in crazy itchy (what I thought were hive, bites, I don't know what...). I went to my GP and they had no idea, so they just gave me an insane amount of steriods (like 15 pills a day, not sure what the mg were). It didn't help and also made me want to crawl out of my skin - couldn't sleep with heart racing and gained like 8 pounds in two weeks... So, after three to four weeks, the 300 spots all over my body with the exception of my face (thankfully) finally went away so I stopped thinking about it. Fast foward to April, same thing happened again. I went to a derm and they did a biopsy, said it was Urticarial Vasculitis and sent me to Rhuematoligist. They did a ton of blood work and said one of my C complements was low and something else was really high, but still couldn't diagnose. Lasted for a month and then went away. Fast forward again to now, all happening again - miserable. I went back to the same derm who did the first biopsy, she did two more. She also asked me a bunch of other stuff and has now linked things together... I have Raynauld's which is pretty common in Chicago except for when it's in conjunction with vasculitis, canker sores and an occassional red nose (which I again contributed to the cold weather). She believes it's Lupus even though my ANA was negative. So... I'm wondering if this is something you've explored too? I guess there are 11 symptoms of Lupus, and generally people have at least four. I'm in research mode like everyone else on this site, and feeling alone... 

15

Your story is almost exactly like mine. Please contact me asap. 

16

Just thought I'd update...in case anyone was interested...

I'm now on 3 x 500mg of Microphenalate and 1 x 5mg of Prednisolone

I've been juggling the steriods and 5mg seems a little low as I get some very minor soreness in my feet and achilles tendons.  However it's so minor I'm happy for it to be the price of a lower steriod dose

My nose finally cleared up about a month ago.  Nasal douching and steriod drops seemed to have fixed it, but I do believe that the drops became part of the problem.  I had 3 days or so when my nose/sinuses/mid-face really were quite painful so I stopped them...and the small amount of bleeding stopped.  It's not 100% sorted as it's still a bit crusty

That, along with my thin and discoloured skin on my elbows and heels seems to be the only symptoms

My complement levels have returned to the normal range

However, the word "chronic" keeps getting mentioned when I see the dermatologist who's in charge of my care.  The plan is to reduce the immunosuppresants over time...how long that timeframe may be is anyone's guess

17

Hello, I must tell you how relieved I am to have found this discussion. The diagnosis process has been anything but easy. I was officially diagnosed with Urticarial Hypersensitive Vasculitis in January of this year. I am 27 years old and unfortunately dealt with the unfortunate misdiagnosis over and over again one year prior. This of course lead to ER visits and being on high doses of prednisone for just about a year. Currently I am being treated with Dapsone, I was using 4x's the amount of antihistamines but was able to have the doctors help ween me off. Unfortunately it's always a guessing game as to what is causing the flare ups, alcohol, stress, hot and cold weather, sunlight, having your period; but I have to be honest I'm terrified of having another flare up. The burning and stinging all over my feet, legs, back, arms and chest, the swelling, pain, and it somehow managed to effect my nerves?!...it felt like random lightning bolts all over my body. Clearly the physical aspects of vasculitis are awful but how do you manage the emotional? I can't get the fear of more flare ups to go away. Hope everyone here is doing well with their vasculitis.

18

Hi,

I have chronic continuous urticarial Vasculitis.

After years of visiting dermatologists I discovered this is an immunology problem & now I visit the immunology dept at the hospital.

Recent studies prove antihistamines are in effect against ccuv. I was on very high dosages of prednisolne ...80 mg per day & then taper. Rituximab is currently the most effective drug but it's very expensive. Biosimilar Rituximab has been approved in the UK.

Good Luck. Paul

19

Hi Winter,

How are you now ?

I ask this cos it 7 years since your post.

Cheers,

Paul

20

I am diagnosed with chronic continuous urticarial Vasculitis as well, it has been 2.5 years and never stopped for 1 day and the condition is getting worse day by day and I rely on steroid. managed to stopped steroid for 1 month and break out again recently, now on Prednisolone 5mg x 2/day but not improved much yet. Dr asked me to take 16mg dose but I just wanna try myself.

after reading this forum, I feel that im not alone but i feel stucked at well, seem no solution to cure our problem, I feel so bad. Im planning to be on vegetarian and hope its better, bcoz i dunno what to do now.