Anyone: have you been diagnosed with Achenbach syndrome (paroxysmal haematoma of fingers (& toes)) or Ehlers Danlos?

Anyone: have you been diagnosed with Achenbach syndrome (paroxysmal haematoma of fingers (& toes)) or Ehlers Danlos?

In September I posted a ? asking if anyone has what I called "tissue bleeds" in their fingers & toes, and I described my version of these. Basically, they don't start due to injury or friction necessarily. At first there is redness, swelling and burning pain, then the deep bleed is red & blue, leaving a deep area of purple, which fades over 3-4 days. The symptoms can cover a small area of tissue, say 1cm square, or a larger area. These bleeds happen several times a month, and seem more frequent when I start dropping my pred dose during a taper (I have SLE, simultaneous RP & EM, global hypermobility etc etc).

At my regular rheumatology clinic in late oct the registrar said she thinks these bleeds are either Achenbach or Ehlers Danlos (an aspect hypermobility syndrome). She says the SLE treatment plan I'm on (plaquenil, Amitriptyline, pred tapers & about to start mycophenolate) is appropriate, and no extra treatment for my bleeds is necessary because there is nothing serious underlying these particular bleeds: there is a concern that I could be developing vasculitis.

So, aside from the very few people who replied to my ? In sept, i'm left wondering: does anyone else here have these sort of tissue bleeds? Perhaps the terms my rheumy uses to cover these bleeds will ring a bell: has anyone here been diagnosed with Achenbach Syndrome ((paroxysmal haematomas of fingers (& toes)) or Ehlers Danlos?

If the reply is yes to these ?s, how frequent are your bleeds & how do you manage them? And are you on similar treatment plans to me?

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  • Hi yes I to have bleeds in my fingers, not nice and exactly as you describe. I also have dvt in both calfs so have to be careful. When this happened the first time ended up in a & e and saw a vascular surgeon who said it was a spontanious bleed and not to worry. She saw me twice 3 days appart and was convinced that was what it was. I have Behcets and at the mo am treated with aza and infliximab and pred. How do i cope well basically ignore them unless the swelling is so painful then see gp. Who also does nothing. Hope this helps if anything else comes to light will post.

  • Yes you are helping! Many thanks. I think ignoring them is the way to go: not easy because they do not feel or look innocent. But apparently they sort of are..because the interesting thing is that in all my forums only 2 or 3 have replied to my 2 questions saying they have something like this sort of bleed...which makes it tempting to believe this is sort of unusual and possibly less well understood than more recognizable (aka serious or sinister) types of bleed....but I'm no expert (except I do know these bleeds, having had to cope with them for 40 years, and seen myversion of them worsen)

    (My HU forums I posted these ?s on are: Lupus uk, sjogrens, raynauds & scleroderma, Ehlers Danlos & vasculitis...)

    V grateful

  • I have not officially been diagnosed but am having the same symptoms. They come when I am terribly stressed. It is very painful. I have a history of a brain aneurysm and varicose veins and wonder if I should get checked out... it seems I may have an integrity issues with my vascular system.

  • Hello nhorelka

    We now know more about the mostly v early onset combined immune dysfunction & connective tissue disorders that have been affecting me multisystem for decades. But most days i thank the rheumy i mention in my post, because she was the first to recognise that my hypermobility is the Ehlers Danlos type....and is affecting the integrity of my vascular system (eg i have always bruised easily, and this has gotten as i've aged worse - as have these paroxysmal marmatomas. Also my v early onset prolspsing piles are another sign...these became strangulated in my early 30s so were given an emergency full-on haemorhhodectomy). Alongside these vascular signs & symptoms, i exhibit others indicating hEDS, including dyautonomia which could be POTs, but because my all medics are diagnosing & caring for my case apptopriately we see no cause to consult hEDS experts a great distance from my rural hime

    Since this post 4 years ago, i have also been diagnosed with small vessel vasculitis & hypogammaglobulinaemia Primary Immunodeficiency. So my collection of primary & secondary multisystem conditions is typically large, as often is true of early onset immune dysfunction & connective tissue disorder patients. And, of course i'm on lots of combined therapy meds for all the treatment plans. I am responding positively to all this care...feeling very glad the health system takes my case v seriously.

    The point is: we now understand the combined causes underlying my version of these paroxysmal haematomas. I cannot say i get more of these when i'm stressed...but i do suspect i get more when one or the other of my illnesses is flaring. I agree, these are v painful.

    My feeling is that you'd do best to report your haematomas right away to your medics...yoir medical history alone makes me hope you report vvvvv soon

    Am wishing you every best wish

    🍀🍀🍀🍀 coco

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