Has anyone been diagnosed with curtaneous vasculitis?

[Galaxy2]

I would love to hear from anyone with this diagnosis. Many Thanks

Jenny

[Ziggy]

Hi Jenny I have over many years now had biopsies taken and originally I had Leukocytoclastic Vasculitis ( Kingston Hospital Biopsy) and then at St.Georges they came up with P-anca vasculitis, which I understand is same/as Curtaneous Vasculitis that you are talking about. If you think we are talking on the same channel, I am willing to help or talk about the strange long termed illness, as it would be interesting to hear what you suffer from or what it causes?

[Winter64]

Hi Jenny, My biopsy showed Leukocyctoclastic vasculitis too (Hallamshire Hospital) I also have various types of urticaria. As I understand it Leukocyctoclastic vasculitis can also be known as cutaneous vasculitis, Hypersensitivity vasculitis and Urticarial vasculitis. Would love to share info with you and Ziggy about this as I havent met anyone in the UK with this yet,

Karen

[Elainekay42]

Hi Jenny,

my son has a diagnosis of uticarial vasculitis (Cutaneous vasculitis?) but has loads of other symptoms too. Chest pains, shooting pains, swollen hands and lumps under his feet to name but a few. He is newly diagnosed and is on seretide 50 for wheezing as a form of asthma developed, anti-histamine for the uticaria and now 400mg of Plaquenil every day. so,I am guessing the uticarial vasculitis was what they could determine from the skin biopsy. I dont know if Uticarial vasculitis would cause all these symptoms? he also has a constant pulse rate of 100 plus everyday.

Are you getting these symptoms too?

Elaine.

[John_Mills Volunteer in reply to Elainekay42]

It sounds almost certain that Urtcarial Vasculitis is just a symptom of a wider disease. It sounds as if your son may have a form of vasculitis called Churg Strauss Syndrome (EGPA) If this is the case he does need treatment asap by someone who knows about Vasculitis. If you private message us telling us where you live and where your son is receiving his treatment we maybe able to suggest someone.

[vickylou01 in reply to Elainekay42]

I am having a 24 hour ecg currently as my HR is running high. I have just had biopsy conformation of urticarial vasculitis.

[Galaxy2]

Ziggy, Karen and Elaine

Many thanks for your relpies.

To give you a history of my symptoms; my auto immune symptoms started 25 years ago when I was twelve but misdiagnosed with ME, I went from being a really sporty child to being totally exhausted and had 5 years out of education, back then other than my complete exhaustion I was covered in a rash and pretty much slept away most of those years. The years after that I developed odd symptoms here and there but always worked full time and felt ok ish. I was really prone to being aneamic and suffered hair loss as a result, one thing that has always been alarming to me is my heart rate (Elaine, that is the first time I have heard someone else suffering from similar symptoms) it reaches well over 200 on exercise, 226 being the highest recorded and during my pregnancy my resting heart rate was between 120 and 130, which it has been recently again. I have had a whole range of tests on it but nothing has been discovered.

Four years ago I started getting bruising all over my lower legs and feet and was told I probably had HSP but this diagnosis was changed because the bruising continued and I was told if it was HSP it would disappear within 6 months. After seeing different specialists I ended up seeing a rheumatologist who diagnosed vasculitis caused by Sjogrens syndrome, at times my legs would really swell up aswell as bruise and I would only have to knock my skin or scratch it and the blood vessels would break. My specialist told me I must be feeling terrible but had probably just got used to it and didn't realise how bad I was feeling. I started taking Hydroxichloroquine which did initailly help, but I didn't feel that the vasculitis was having much of an impact on my life but last Summer things really went downhill, I started feeling so tired all the time and developed a really high heart rate again, the bruising on my legs was worse and I was covered in a rash that itched so much I didn't know what to do with myself and was having to have sleeping pills just to get any sleep. Then I started getting numbness in my legs and then my arms, this would only last short amounts of time and then turn to aches, my joints ached too and I have all sorts of strange aches and pains that come and go. I have been on predisolone and mycophenolate since the summer and am slowly seeing some improvement. All this hasn't been helped by me rupturing my achilles tendon in September as a result of Sjogrens and taking predisolone, the weight my arms has had to take by being on crutches has made the numbness in my arms my main problem. I went to St Thomas's last month and was told my diagnosis was curtaneous vasculitis and the numbness is caused by the inflamed blood vessels passing through nerves beneath the skin. I have never had a biopsy but have been diagnosed by both my own specialist and at St Thomas's by them looking at my legs and taking blood.

I really hope that one day i will get back to normal, I haven't worked for 4 months and am sleeping so much at the moment, simple daily tasks are leaving me exhausted. I just don't know what my future outlook is and my specialist can't really tell me.

If any of this similar to your symptoms? Any advice would be greatly appreciated.

Many thanks

Jenny

[Winter64]

Hi Jenny,

Im so sorry that you have suffered for so long. Definitely some of the symptoms I have are the same as yours. With my vasculitis I also have Chronic Urticaria, Dermatographism, delayed pressure urticaria and angioedema. My main symptoms are chronic fatigue, giant wheals on most parts of my body (bizarrely except the bottom of my legs) hair loss, muscle pain mainly concentrated in my lower arms, chest pain, (just been checked for angina) night sweats which go in cycles, gut bleeding, short term memory problems, Raynauds, Cryofibrnogenaemia and sometimes vision blurring. Been checked by Immunology, rheumatology, gastroenterology and dermatology. My bloods show constant raised CRP levels, sometimes a low raised ANA and not a lot else. Rheumatology wanted to discharge me straight away because they couldnt find anything systemic, (even though urticarial vasculitis has a non systemic variant) so my main consultant is my immunologist.

I have tried Hydroxychloroquine but after 4 months made me feel suicidal so I had to come off it. I currently take Dapsone as my main med but its effectiveness is wearing off as I have had some wheals and angioedema in my throat this week and the gut bleeding is back. I take Fexofenadine and Hydroxyzine, both antihistamines aswell and tranexamic acid to help the swelling from my angioedema.

I have had this for 7 years this month and I am no nearer really finding out much about it and have had a lot of too-ing and fro-ing with diagnosis, despite positive biopsy result.

Immunology now want to try Cyclosporin to try and knock out my immune system to see if my CRP levels then stay down. My next appt is March so I will know more then, but must admit I am scared.

I cant get an answer from anyone as to what causes my muscle pain, I find it difficult to do things like peel potatoes because holding a knife or peeler hurts, or holding a pen at work and no-one seems to take it seriously or will treat it.

I have told my consultants that I can just about cope with 24 hour itching and burning and having a face swollen all over but its the constant exhaustion and muscle pain that gets me down the most.

I am hoping if the inflammation goes down the muscle pain will improve and the exhaustion, but im not holding my breath

I get numb hands but I have Vitamin B12 injections and this has helped with this, so it might be worth checkign your levels. I also take Vitamin D and calcium as both those levels were down too. Do you get twiching spasms in your arms, I have had this for the last couple of weeks and it feels really

weird. Thank you for putting your story on here, becasue I was begninnign to feel like everything was in my mind because its not one of the immediately main life threatening types of vasculitis, its hard to get anyone to take it

seriously and the effect all the various symptoms have on your life. Karen

[Elainekay42]

Hi

I will give you a quick run down on what has happened to my son. He is 20 years old and has Aspergers.

About 2 years ago, he started to develop sensitivity to things like bubble bath and sun cream but we did not think much about it. Then he started to develop severe reflux and terrible heartburn and chest pains. He has had chest pains that have been put down to stress for several years.

He had a gastroscopy done and although he was only 18 he was diagnosed with Barretts disease (pre-cancerous cell change to the lining of his easophogus). he was put on Omeprazole for the symptoms. It is very, very rare for somebody to have Barretts at 18.

Early last Summer his hayfever suddenly went out of control. he came home one evening and all the exposed parts of his body, especially his face went bright red. It looked like he had sunburn. That night, his eyes became so swollen he could not open them. the GP put him on a higher dose of antihistamine that helped a little.

He also started to complain that he felt like he had a constant chest infection. The GP could hear a rattle so gave him anti-biotics that only helped for a little while. He also complained he found it an effort to breathe sometimes. he had a constant cough.

Within a week or so he then kept complaining that his hands were hurting but we could not see anything. He then developed uticaria all over his body. Several G.P's saw him and even took pictures as it was so bad. They said it was just an allergy and gave him steroids and anti-histamines.

The next week, we went on holiday for 3 weeks leaving my Mother-in-Law to look after my son. During this period things got a lot worse. The rash became uncontrollable even on steroids. He had painful lumps on his feet that hurt him so bad he could not walk .He actually had to crawl across the floor to call his Nan. His hands became so swollen he could not keep his rings on his fingers. He had terrible night sweats and vomitting. My son was taken to A&E but was sent away with more steroids. He was still covered in the rash and complained of tummy pains, aching joints, muscle weakness in his arms that was sometimes so bad he said he could not even lift the duvet on his bed.

I could not believe this was just an allergy. Even the nurse in A&E said she thought it was something to do with his immune system. He had now been reffered to a Dermatologist but I researched an immunologist in our area and our wonderful GP managed to get my son an emergency referral.

The Dermatologist finally took a skin biopsy in September but we were not given an appointment to get the results until December!

The immunologist took blood and chased to get the biopsy results. Her initial thought was that he has Mast Cell problems until the skin biopsy came back confirming vasculitis. He was also diagnosed with Astmah and had a steroid inhaler (Seratide). He had a constant cough but a chest x-ray was negative.

In November he still felt really ill and his friends started to comment that his hands kept shaking for no reason and he had a patch of hair missing. He ached all over. I contacted the Immunologist who had him admitted to hospital where he was given a massive dose of antihistamine and was taken off the steroids. We were told his trypaste levels were raised and something about too many white blood cells. His blood results showed inflamation.

Due to his Aspergers he refused to stay in hospital so I have monitored him at home.

The immunologist says he has Uticarial Vasculitis with mast cell involvement. She did the scratch test to confirm his body was making too many histamines (you could write his name on his body with a slight scratch!). Throughout this time he has had a fast pulse (100 plus at rest) which was due to his body fighting inflamation.

He is now having monthly blood tests. he had a urine test last visit which has detected protein so we are now getting another one done.

Today he is better as he has no uticarial rash but has terrible aches and pains, He gets shooting pains in his arms, legs and head and randomly develops large solid lumps (usually above a vein) that come and go. He has pain in his head that has been diagnosed as sinus pain and for a while started to go deaf in his left ear. The chronic coughing has returned and odd bumpy rashes keep coming and going. He had a finger swell last night that was really painful. His eyes also had slight swelling and He complained of terrible chest pains that have subsided today.

I am worried about the cough as he also said he is coughing up dark stained mucus regularly. He smokes (as all his mates do!) so it has been put down to that.

We have an appointment to see the Stomach consultant at the end of Feb to get the results of a Barium swallow he had in November and to see what can be done about his reflux as it is really bad at the moment.

He is always unwell but I am hoping the Plaquenil will help soon.

Any advice about his situation would be appeciated.

Thanks

Elaine.

[John_Mills Volunteer]

Elaine, I really think you need to get a referral to someone who knows a lot about vasculitis. And preferably very soon. (When you say that he has an appt in Feb to get the results of a barium meal examination done in November. it doesn't exactly sound like first class treatment! Nor does waiting 3 months for the results of a skin biopsy). You mentioned that he now has an asthma type cough. Hypersensitivity vasculitis usually goes away when the cause is removed. It would be a great help If you can find out from your GP about this "having too many white cells". If they were "Eosinophils" it is almost certainly Churg Strauss Syndrome, which is what I suggested earlier. The dark stained sputum is worrying, as is the proteinurea and the ear being affected. The rash that you describe and most of his other symptoms do sound like vasculitis. A cutaneous rash is a symptom of several types of vasculitis.

Dr David Jayne at Addenbrookes Hospital, Cambridge is a world expert on vasculitis and lupus. I think it is not too far from where you are to Cambridge.

If it turns out it is not a type of systemic vasculitis, then at least it would narrow field in the search for the cause. Your GP can refer you to Cambridge. It could be a very worthwhile trip!

John

[Elainekay42]

Hi,

I will look into this as soon as I can. The blood test they are doing every month is for, liver, bone, electrolyte and a request for CRP on one form. The second form we have has full blood count and ESR ticked. The last 2 tests he has had were for Trypaste as well but I do not know if that is included this time.

I am also worried about the dark sputum. I do agree he should not smoke but I do not know anybody else of his age having this problem.

I also forgot to mention that when he was having hearing problems in his left ear he had pain in his head that came in bouts that was so severe he could not even speak while it was happening. He also had a lump the size of an egg come up on the top of his head. He described it as shooting pains in his head followed by a headache that felt like "pressure". After these headaches he would bleed a little out of both his nostrils when he blew his nose. The Immunologist checked there was no swelling on his brain by looking behind his eyes. Although they said it was not "normal" they never told us what the cause was apart that it was likely to be a sinus problem.

Tonight he is coughing and complaining of pain when he inhales to the point he struggled tonight to use his inhaler. His chest hurts a lot and he said it feels like it is his lung.

I have told him we will see the GP and see if we can get another opinion.

I will keep you posted as to our progress.

Many thanks

Elaine.

[Galaxy2 in reply to Elainekay42]

Hi Elaine

It sounds like your son's health problems are very complex and whilst I can relate to many of them certainly not all of them. John is an excellent person to talk to and a world of information. I wish you luck trying to get another opinion and agree with John that he needs to see a vasculitis expert. I have found it infuriating with all the waiting and delays for appointments and think that you need to be really pro active in pushing for appointment dates and making it clear how serious this is. When I finally got to a specialist I felt so much better having things explained to me and having my questions answered.

Good Luck

Jenny

[Elainekay42]

Thanks Jenny.

I will update here as soon as we get any further.

My son has already had to deal with Aspergers, reflux and Barretts Disease as well as some other genetic problems that made his jaw overgrow and poor dental formation that meant he had no "bite". An orthordontist has helped with that but here we go again with another medical problem. he also has to take Zolpidem to get any sleep.

He is so young to have all these problems but he is very brave and tries to just get on with life.

I really hope you feel a little better soon.

Elaine

[Belg]

There is a facebookgroup for people with urticarial vasculitis. If you want to join us, be free!

facebook.com/groups/6303851...

[Belg]

Hypocomplementemic urticarial vasculitis (HUV) is a rare form of cutaneous small-vessel vasculitis

Characterized by recurrent episodes of urticaria and painful, tender, burning or itchy skin lesions, often associated with extracutaneous involvement but usually with no significant peripheral nerve damage. Patients with this condition are likely to have systemic involvement, including angioedema, arthralgias, pulmonary disease, abdominal or chest pain, fever, renal disease, and episcleritis. Hypocomplementemic urticarial vasculitis is thought be an autoimmune response involving a specific region of complement 1 (C1). It can present as or precede a syndrome that includes obstructive pulmonary disease , uveitis, systemic lupus erythematous (SLE), Sjögren's syndrome, or cryoglobulinemia (which is closely linked with hepatitis B or hepatitis C virus infection). Some cases of hypocomplementemic urticarial vasculitis respond to therapies commonly used for the treatment of SLE, including low-dose prednisone, hydroxychloroquine, dapsone, or other immunomodulatory agents.

Hypocomplementemic urticarial vasculitis (HUV) Synonym(s)

Mac Duffie hypocomplementemic urticarial vasculitis

Mac Duffie's syndrome

McDuffie hypocomplementemic urticarial vasculitis

McDuffie syndrome

[Elainekay42 in reply to Belg]

That is really interesting. The Immunologist has referred my son to an Opthalmist as she now suspects uveitis. His breathing is bad tonight and he is coughing up black mucus. We have also just noticed 2 bald patches where he has lost hair (he is only 20). I dont know if this is relevant to anything?

His pulse was 105 again and he has had the first rash appear in weeks. It just goes on! Shooting pains again and he says it feels like something is squeezing inside his chest. Would this all be UV?

Thanks Belg.

[Lupylass]

Hi there Elaine and sorry to hear about your son's problems.

I thought I'd share my story too, which gives a slightly different slant. However you've already been given good advice and your son would benefit from vasculitis specialist follow-up.

My story

In 2008 I started to feel extremely tired. This went on for a few months, and just as I got round to making a GP appointment I developed a rash. I was eventually referred to a dermatologist who diagnosed urticarial vasculitis. They tried numerous anti-histamines and steroid creams, but they would only work for a few days and then the rash would come back with a vengeance. In the meantime, I was still extremely tired (I ended up having to take time off work), developed joint and muscle pains with swellin, hair loss, swollen glands intermittently, eye problems ranging from bloodshot eyes, swollen eyelids to having gritty eyes with discharge at times. I was referred to an ophthalmologist in case it was uveitis or vasculitis but my eyes were fine in both respects.

Eventually some missing blood test results showed up which indicated that I had some sort of connective tissue disease. It was at that point that I was referred to a rheumatologist specialising in lupus, but who also ran a vasculitis clinic - I attend the vasculitis clinic to this day. I have hypocomplementic urticarial vasculiitis, and have also now been diagnosed with lupus (SLE), Sjogren's Syndrome and coeliac disease.

I was put on Plaquenil, which for me took about 6 months to work, but as a short term measure I also needed prednisilone to get my disease activity under control. I'm off the steroids now and am extremely well, I have no skin problems, and in fact all of my symptoms are all under control. I have to wear factor 50 sun protection at all times, regardless of the weather, and I do have to pace myself, but that doesn't stop me from doing regular exercise and working. I've been very fortunate in getting an early diagnosis and early medical intervention.

For me it has helped that I've understood what's been going on with my diagnosis and treatment, and for lupus sufferers in particular the medical team encourage patients to have a good understanding and to self manage. I get the doctors to send me copies of the letters that they send my GP, to help me understand.

I hope that your son can get the right medical team looking after him, the right diagnosis (whatever that turns out to be) and the right treatment as soon as possible.

I'm not sure if my story helps but I do see some similarities in some of the symptoms.

Take care

[Galaxy2 in reply to Lupylass]

Lupylass

Thanks for posting your story on here. I too have sjogrens, lupus and curtaneous/utricarial vasculitis and its really good to hear it can be kept under control. I have had a really bad six months and to hear that you have returned to work and are exercising is great as somedays I have wondered if my life will ever get back to normal, so thank you.

[Lupylass]

Jenny I read your story and you are having an awful time. St Thomas's is the place for lupus suffers so I truly hope that they will find the right combination of treatment for you.

I know I've been very lucky to feel as well as I do - I have my ups and downs but mainly I feel pretty much at 90% of the old me, which isn't bad at all.

So sorry to hear about your achilles. I had a partial rupture before I knew anything about having any of these horrid illnesses, and have achilles tendonitis just now, so am being careful so as to avoid a rupture.

I notice from your previous post that you got really bad in the summer - do you use sun protection? I am very photosensitive, but it isn't immediately obvious - sometimes it can be hours or days before the reaction is obvious, and sometimes I think the sun can have a cumulative effect. I've been advised to wear factor 50 all the time, and I take cod liver oil in order to help get my vitamin D intake. If you haven't explored this already it might be worth discussing with your medical team.

I hope you get on the road to recovery soon.

[Galaxy2 in reply to Lupylass]

Lupylass

Thanks for your message. I did know about the sun issue and think I had such a bad time in the Summer because of that. I went on holiday and because of my rash my skin was red raw and I couldn't put on my sun cream and made the mistake of thinking I was being protected under a parasol but it was canvas and I caught the sun through it and well thats when I started to feel really ill. Anyway, I will learn from that and plan to keep well out of it from now on.

Thanks for the encouragement.

Jenny

[RichardE Volunteer]

Hi Jenny,

Like Lupylass I too was diagnosed with Hypocomplementemic Urticaria Vaculitis a couple of years ago.

My symptoms were very similar : Started with inflammed eyes (diagnosed as Episcleritis) then vasculitic and urticarial rashes combined with occasional joint pain, swollen lips and breathlessness. Unfortunately, whilst waiting for a skin biopsy I had a heart attack (caused by the HUV - no other risk factors). And only at this point did I see a rheumatologist who eventually diagnosed the disease.

Further tests showed lung and kidney damage and I became haemolytically anaemic (accounting for the new symptom of extreme tiredness and breathlessness and requiring blood and iron transfusions until the HUV was brought under control). I was on massive ammounts of steroids to control the inflammation and Mycophenelate to suppress my immune system. Two years on, I'm almost off the steroids and feel back to about 90% of health.

But my point is that I believe half the problem with Vasculitis diseases in general is the variety of different symptoms that confuses the medical professions and delays diagnosis until something major happens to draw their attention to these rare diseases. In my case I feel I aggrieved to have had the heart attack but so lucky to have survived it ! My advice to anyone worried about their symptoms is to push your immunologist / rheumatologist to consider all the variants so that you can start treatment as soon as possible.

All the best,

Richard.