Unusual Presentation of Livedo? This ... - Hughes Syndrome A...

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Unusual Presentation of Livedo? This is also happening right now during my “ clotting rush” along with my classic livedo. ( INR is at 4.0)

KellyInTexas profile image
KellyInTexasAdministrator
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I can get this at any time. Often a red pin prick dot will be in center of white balanced ring. Never itches.

This was last night at our pharmacy. Walgreens ( a sister company to England’s Boots.)

I believe it’s a component of livedo.

I’m trying to understand what is going on with my vascular system/ clotting/ auto immune. I’ve been doing it for 16 years- started in my pregnancy with our 16 year old son.

Does anyone else present with this And does your doctor have an accurate understanding of what this is? Is it clearly connected to the APS livedo?

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KellyInTexas
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lupus-support1 profile image
lupus-support1Administrator

Symptoms

Livedo reticularis can be developed at any age. The usual affected sites are the arms, legs, trunk and rarely it also observable on the back. Following are the symptoms of livedo reticularis:

Purple or bluish red discoloration on the skin surface

Pale coloration at the central of the discoloured area.

The discolouration is not even; it is mottled

Pain is associated in some cases

Skin ulceration may develop which produce discomfort and pain; this is more prominent in lower limbs

Numbness

Increase of body temperature

Inning or burning sensation

‘Falling asleep’ condition may develop at the upper or lower limbs

The condition becomes worsen with chilled temperature

Causes of Livedo reticularis

The lacy appearance is plainly a result of an impaired blood circulation caused by thrombosis in capillaries and arterioles. The reddish blue discoloration is associated with several underlying pathological conditions that cause swelling of the venules. These include:

Sneddon syndrome

It is a non-inflammatory arteriopathy in which the mottled discoloration of the skin has a relation with cerebrovascular disease. In this condition, some hereditary factors can lead to systemic vascular diseases, including strokes.

Cutis marmorata telangiectatica congenital

This uncommon, sporadic congenital vascular anomaly often affects the blood vessels of the skin.

Idiopathic livedo reticularis

Unsteady flow of blood through the veins, particularly in young females, could occur for unknown reasons. However, the sudden network-patterned cutaneous discoloration is completely innocuous with mild episodes of ulceration on the breasts, abdomen, buttocks and thigh, caused by heat.

Polyarteritis nodosa

Self-destructive immune cells often attack the medium and small-sized arteries, resulting in a severe type of vasculitis.

Fibromyalgia

The lacy discoloration of the skin can also be attributed to fibromyalgia, a chronic disorder marked by musculoskeletal pain, fatigue and tenderness.

Livedoid vasculopathy

It is a chronic, recurrent, painful skin disorder that involves lower extremities and feet. The thrombotic condition is non-inflammatory in nature and oftentimes leads to ulceration in the legs.

Dermatomyositis

In adults and young children, this inflammatory disorder manifests into muscle weakness and a distinctive skin rash. Swelling of the blood vessels may give rise to a reticular cyanotic discoloration of the skin.

Antiphospholipid syndrome

It is a complicated condition in which the presence of antiphospholipid antibodies in the blood leads to excessive clotting and low platelet count. Formation of such clots may either cause brushing or a blotchy, purplish rash. The disorder has often been linked to morbidity in pregnancy.

Cryoglobulinaemia

The stagnation of blood in the arteries and venules is frequently caused by precipitation of cryoglobulins at low temperatures.

Homocystinuria

It is an impaired metabolism of the amino acid methionine, which results in deficiency of calcium in the body.

Arteriosclerosis/Cholesterol emboli

Dilation of capillaries and venules could also be associated with thickening and hardening of the arterial walls.

Ehlers-Danlos Syndrome

It encompasses a group of disorders in which mottled cutaneous discoloration, laxity and joint hypermobility are the common hallmarks of the syndrome.

Systemic lupus erythematosus

The mottled reticulated vascular pattern is common in lupus patients since it causes spasm of the dermal ascending arterioles.

Autonomic nerve damage

Physical trauma or injury may lead to autonomic neuropathy in some cases. One of the most common manifestations of this condition is the abnormal rate of blood flow through the small arteries and capillaries that results in a bluish, lace-like discoloration of the skin. Palmar erythema, a vascular disease of the palms, has a similar cause.

Livedo reticularis Treatment

Appropriate medical care can be initiated to the affected patients on the basis of the results acquired from the diagnostic tests and exams. The aetiology of the condition is instrumental for treatment. Idiopathic livedo reticularis is a self-limiting condition that could be controlled by keeping the legs warm with the aid of a heating pad. Asymptomatic form of the disorder may come and go without the knowledge of the patients. Drug-induced livedo reticularis requires immediate withdrawal in order to reduce the attacks. However, the net-like purplish discoloration of the skin could become a permanent problem if not treated in time. The dermatologic manifestation is itself not treatable unless doctors recognize the underlying disorders. Delayed treatment may cause complications like thrombocytosis and thrombohemorrhagic disorder. Patients with antiphopholipids can be administered low-dose aspirin and hydroxychloroquine. Plasmapheresis could be carried out for cryoglobulinaemia. Immunosuppressive drugs such as cyclophosphamide and azathiprine can be used to cure polyarteritis nodosa. Livedoid vasculopathy-affected individuals may receive anti-platelet agents, anticoagulants, pentoxifylline and phenformin. On the other hand, anti-inflammatory medications like corticosteroids are suitable for treating dermatomyositis. Healthcare professionals prescribe cholesterol drugs, beta-blockers, and ACE inhibitors for lowering blood pressure and controlling the heart rate. The frequent episodes of the cutaneous condition can be prevented by avoiding cold condition and strenuous exercises.

Elevated levels of blood calcium can reduce the flow of blood through the narrow capillaries and veins.

KellyInTexas profile image
KellyInTexasAdministrator in reply to lupus-support1

I do have the classic livedo on arms and legs. this is just not classic. I’m wondering if it’s also livedo, because it does not look like my “ classic livedo” which is picture perfect text book.

lupus-support1 profile image
lupus-support1Administrator in reply to KellyInTexas

I am sorry but I don't understand what you mean. The medical name is livedo reticularis.

If you are concerned, go and see your doctor. You can take photos as well.

KellyInTexas profile image
KellyInTexasAdministrator in reply to lupus-support1

Yes, I do know the full name. I have been properly diagnosed with the proper full diagnosis of livedo reticularis.

Professor Hughes simply called it “livedo” with me so I assumed it was common ...I figured you would know what I meant.

I’m trying to ascertain the extent of the auto immune component versus the anti coagulant component because right now while I’m clotting to try to make the best decision I can about very aggressive auto immune suppression. I’m reclotting quite badly so im seeing if there is a tie here. My INR staying close to 4.0 by vein and I’m continuing to clot.

lupus-support1 profile image
lupus-support1Administrator in reply to KellyInTexas

It's the clotting that is important and I hope you get the help you need.

KellyInTexas profile image
KellyInTexasAdministrator in reply to lupus-support1

Thank you, Ros. I hope so, too! This is the 6th DVT in 12 months.

lupus-support1 profile image
lupus-support1Administrator in reply to KellyInTexas

I am sitting in hospital with my husband who has been diagnosed with a DVT! He doesn't have APS or SLE, but I do!

You really need a specialist to take proper care of you because 6 DVTs in 12 months suggests otherwise.

Wishing you a speedy recovery and good wishes,

Ros

KellyInTexas profile image
KellyInTexasAdministrator in reply to lupus-support1

All the best to your husband! He couldn’t have a more qualified wife ... so sorry.

My doctors are following dr Hughes guidelines and clinic letter for me. They are doing RAPS trial protocol.

It seems a sub set of us fail traditional methods and need immuno suppression. ( we clot despite proper anti coagulation. Not a blood flow issue.) This is why I’m asking about more auto immune clues.

lupus-support1 profile image
lupus-support1Administrator in reply to KellyInTexas

Thank you for your good wishes.

Unfortunately, autoimmunity is not well understood. I follow dedicated researchers at University College Hospital, London, into B cells etc. One researcher's son has joined his father in this research.

In Israel, a certain type of worm, seems to have a positive impact for certain patients with SLE. Prof. Yehuda Schoenfeld at Tel Hashomer has patients willing to digest it! I would wait until the secretions are turned into a drug. He explains this on YouTube!

There are no 2 patients exactly the same, even though we share similarities!

It can also make us feel many things: frustration, anxiety and depression are common and "normal" responses!

If you want a chat, PM me anytime!

Ros

KellyInTexas profile image
KellyInTexasAdministrator in reply to lupus-support1

Thank you! I’m sure I’m feeling all these things... even enough to try worms! I do know of this professors work. He is interested in auto immune over reactions to vassinatiins . ( he is a proponent of vassinatiins- not to be misleading.)

Our son is on the autism spectrum ( unless they changed that again this week! Ha!) - with a very mild case of Asperger’s syndrome. And.. APS now. He’s 16. Very highly gifted- IQ.

So I find dr. Y Schofield ‘s interest on that very poignant.

MaryF profile image
MaryFAdministrator in reply to KellyInTexas

I think a lot of people have it, in less of a text book appearance, we are all different, hope you feel better soon. MaryF

KellyInTexas profile image
KellyInTexasAdministrator in reply to MaryF

Well, I have the “ text book “ appearance, but then I get this strangeness also.

I was just wondering if others experience it. Or, if it is something else entirely.

Ray46 profile image
Ray46

Livedo reticularis is a livedo that looks a certain way, and not like your picture, so your picture isn't it, _but_ it is probably still _a_ livedo and probably the same underlying cause - at a guess, since you've been clotting venous, insufficient venous drainage from the extremity.

This is very likely (just one more not-a-doctor opinion) another symptom showing that your APS is not under control, and whether it looks "reticularis" doesn't matter, particularly as you say you have characteristic reticularis elsewhere anyway.

More info: The reason livedo reticularis looks the way it does, and the characteristic of net/lace appearance, is explained by the first diagram on this page: clinicalgate.com/other-vasc... That diagram is used elsewhere as well, and I've found it the most helpful in understanding what is (thought to be) behind livedo. I don't have it, but after so many vague questions from doctors about "do you get a rash (no not like that)" it took some research and pictures to convince myself.

KellyInTexas profile image
KellyInTexasAdministrator in reply to Ray46

Thanks Ray. Yes, I posted a photo of my legs in classic livedo R. A few months ago- I was 3.5 INR.

Thanks again for info!

KellyInTexas profile image
KellyInTexasAdministrator in reply to KellyInTexas

By the way Ray, while I have a moment and it’s on my mind, because I’ve thought this often about you at random yet inconvenient times ( like while doing the shopping - it’s when I seem to have a quiet moment to think and process!) ...

I wanted to take a “ real” moment to thank you. I’ve noticed time and again on this forum with so many people you really do take the time to give well thought through answers, which means you take the time to genuinely listen.

Your acumen for procuring relevant papers are incredibly helpful- especially to me . My eye sight would never allow me to do this.

A proper thank you is in order.

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