Vascular EDS and Angina Bullosa Haemorrhagica

[Saassii]

I have recently read that there is a connection between Angina bullosa haemorrhagica and vascular EDS. All I know so far is that I've been getting loads of ABH and I'm hypermobile in many joints, along with other EDS symptoms present. Does anyone have any information on this at all please? Thank you.

May be relevant. I lived in a building with water damage and subsequent microbial contamination without knowing. I discovered this when a pipe burst in an adjacent area. I was told the area had been made safe. Yet within two weeks horrifying pain and symptoms made me realise it was not and Angina Bulla Haemorrhagica lesions in my oral cavity began then and have not stopped. Any help and thoughts are welcome. Thank you.

[Jay66]

I have not heard of this one. There are dental effects from EDS, to the extent that there is even a periodontal version (and was in the past - old type 8) and many of us, myself included, have endless and varied problems with teeth and gums. This study wjgnet.com/2218-6263/full/v...

suggests some cross over with diabetes type 2 and hypertension.

Interestingly, the periodontal EDS type most closely matches my very hypermobile mother's (all 4' 9" of her - lost all her teeth before age 26). I don't think we are pEDS though because I don't have the pretibial plaques (odd colouring on the front of your shins).

However, if you think you are vascular it is imperative you get a proper diagnosis done asap.

[Barnclown in reply to Jay66]

Thanks for that link: hadn’t found that doc before! It gives THE best & most thorough info i’ve read yet!

Here is a link to quite a good clear concise source of introductory up to date info on ABH. I’ve been researching ABH since the 1970s...i’d say medicine has not yet understood ABH as fully as it hopefully will some day

emedicine.medscape.com/arti...

[Jay66 in reply to Barnclown]

Oh Barnclown I am now sure I have read about this before when you have posted, but my memory is now so bad! I am sorry I didn't connect it in my head. I was supposed to be starting a new (very part time) job today (postponed) and have literally no functioning memory at the moment.

[Barnclown in reply to Jay66]

WOW: PLEASE: no apology needed...OMG you’ve helped SO MUCH by giving us this link to this REALLY GOOD doc on ABH. no way can anyone remember all the topics that come up....am greatly relating to your reply cause i’m extra wooly headed at the mo due to some particularly lengthy & troublesome health stuff under investigation. It’s GREAT you’re getting into a part time job: hope you’ll let us inow how you get on XOXO

[Barnclown]

Yes, medics think my early onset primary immune dysfunction & connective tissue disorders are causes underlying my angina bullosa haemorrhagica. I’ve been on HealthUnlocked a lot for almost 10 years and there have been others posting pics like yours & asking if anyone else gets these...my memory is that these posts are mainly here on the EDS UK forum and on the Lupus UK forum. I usually reply giving some info about ABH.

For what it’s worth, my ABH began in my early 20s, was diagnosed by a dermatologist in my 40s, and am now 64. I am not aware of any of my family members having ABH

My primaries are:

Infant onset systemic lupus

hEDS

The Primary Immunodeficiency bone marrow illness Hypogammaglobulinaemia (below range immunoglobulins G, A & M...which predisposes me to infections of all sorts by preventing me being able to make antibodies)

And i gather my early onset secondaries are fairly typical of hEDS/lupus/PIDpatients:

Sjogrens (severely affecting me multisystem, and causing lots of hassle in all my “holes”, especially my mouth)

Small vessel vasculitis

Simultaneous raynauds & erythromelalgia

Peripheral neuropathy

Infectious urinary tract disease

Crohns-like chronic intestinal psuedo obstruction

Lichen sclerosus

keratitis

ABH

Etc etc

LUCKILY, i’m in tertiary care at an international NHS university hospital where clinicians have spent years working hard to investigate & diagnose my multisystem illnesses, and all their resulting combined therapy treatment plans are helping a lot. But i have had to consistently fight long & hard to achieve this relative success...and, after a lifetime of this, inevitably my multisystem debilitation is relatively great. I continue to work hard as my own carer + health care advocate. There are lots of others here doing the same. I am hugely grateful for the solidarity here

I am replying at length because my ABH was one of the first manifestations that made me realise sonething was wrong with my connective tissues. Alongside the ABH i get other spontaneous tissue bleeds in my hands, feet & lower legs ...in my case all these “paroxysmal haematomas” occur without any sort of local trauma &/or direct contact pressure...and, of course, i bruise relatively easily all over my bod anyway

I’ve said above that I think my daily combined therapy lupus meds are actually damping down my ABH, because the lesions seem to occur less when my SLE etc are damped down & cooperative (same goes for the spontaneous tissue bleeds all over my bod)

I hope something in all that is useful to you

🍀🍀🍀🍀 Coco

[Saassii in reply to Barnclown]

Thank you all so much for the help and links, greatly appreciated. Thanks so much Barnclown for taking the extended time with your help. May God bless and strengthen each of you during these hard times called life.

Can I ask, have you/have any of you been exposed to microbial contamination from some type of water ingress at home or work? I never had an ABH until I was exposed suddenly. I'd been having issues from an unknown mold source, but they weren't as savage and I'd never seen an ABH until the second exposure. I had sudden onset of a bunch of symptoms y'all seem to be having. I've read EDS predisposes people to greater susceptibility with molds and bacteria. Now I'm literally wondering how many people this was the triggering event for potentially? Has anyone lived or worked in a water damaged building and been around mold/bacteria?

[Barnclown in reply to Saassii]

Interesting questions! Because i’m at high risk of infection due to all my primary illnesses, especially my PID bone marrow disease, and, despite conscientious lifestyle management, i’ve lived most of my life constantly infected with whatever could infect me (bacterial, fungal, virus pathogens) there is a chance the sort of infection your wondering about could be one of the underlying favtors causing my version of ABH. But so far my immunology clinic’s monitoring hasn’t spotted a specific fungal/mold infection in my case. In the other hand, immunology has diagnosed early onset pockets of persistent multisystem bacteria-driven sepsis in my sinuses, upper jawbone, stomach & urinary tract.

[Saassii in reply to Barnclown]

Dang. I know those microbes inhabit the nasal passages. I know I had a tonne of bladder problems relentless for years until I changed my diet to exclude all foods susceptible to molds and all high histamine foods and I moved out of the water damaged building.

I've now become so hypersensitive to mold/bacteria that I can usually know it's somewhere within seconds or longest within minutes. The burning pain that starts is debilitating. But if this reaction were not triggered alerting me, then I'd be getting damaged unwittingly just like before.

Places I can't manage around because the microbes are generally too high are all drains, dry/wet, in showers, window tracks, air conditioners, carpets, curtains hung near windows not clean. I'm so terribly sensitive now. I wonder if there's any source in your home increasing your discomfort? May be safe for others, but maybe not for you?

Gentle hugs x

[Barnclown in reply to Saassii]

Again: interesting. But, no, other than 5 months’ daily exposure inutero to the notorious artificial osetrogen DES (diethylstilboesterol), we know of no relevant historic of current reactions to environmental exposures in my case...lucky for me, my condition is well understood & managed. I am satisfied with that, and, of course am v cautious about avoiding all pathogens.

Am sorry you’re having a really tough time & i wish you every best wish

[Saassii in reply to Barnclown]

Thanks so much, I'm sorry I'm having a rough time too lol. It's really nice to to have highly intelligent people who understand and can offer support, advice and helps.

It's comforting to know you've hEDS with ABH and that it isn't vEDS. I got super concerned it was more associated with vEDS. I've had no genetic testing at this point, kinda don't want to.