Heather Hettrick, PT, PhD, CWS, CLT, CLWT brings to light the various pathophysiologic causes of lymphedema. She also provides an overview of the magnitude and prevalence of lymphedema on a global scale. Dr Hettrick concludes by defining the differentiating factors within each stage of lymphedema, while also providing clinically diagnostic criteria for associated disease processes.
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Heather Hettrick, PT, PhD, CWS, CLT-LANA, CLWT
Department of Physical Therapy
Nova Southeastern University
Ft Lauderdale, FL
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TAPE STARTS – [00:00]
Heather Hettrick: So now, we’re going to just go and talking a little bit about lymphedema, the etiology like what it is, some of the different stages because I think it’s just important to understand those concepts. But when you look at lymphatic pathophysiology, there are many conditions, diseases and trauma that can lead to pathology of the lymphatic system. There can be organic changes that affect the transport capacity, functional changes or even a combination. So, organic changes could be like atresias, hypoplasias, hyperplasias, lymphangitis, thrombosis, clots, trauma, injury, there’s a lot of different things that can cause organic changes to that system. And then there are functional changes as well. There can be valvular insufficiency so much like our veins if the valves aren’t working properly that can be problematic. Neural insufficiencies with the vessel wall at rhythmic pulsations of the collectors, which actually contract. Lymph vessel spasms, there’s a whole bunch of different things that can come on, but well under all of that what ends up happening, is you get a reduced transport capacity and that’s going to impact how much lymph is formed, meaning how much is picked up for those interstitial tissues, how much is then transported and ultimately brought back to the venous angles and also can be a combination.
So, again, I think it’s important not all swelling is equal. We know that, yes.
Participant: Is the Stemmer cells [indecipherable] [00:01:22]
Heather Hettrick: No, I’ll talk about Stemmer. I’ll talk about this Stemmer, that’s a clinically diagnostic test we can use to, when it’s present, is a positive sign for true lymphedema.
Participant: [Indecipherable] [00:01:34]
Heather Hettrick: Can I talk about that in a few minutes, because it’s a great question and it’s key to what we’re talking about here, absolutely. Oh, I just want to-- you’re jumping ahead, but you’re thinking great. Okay, thanks.
So, you can look at this list, this is not an all-inclusive list but what’s really important to remember is we need to be really good at our differential diagnoses because it’s going to guide our interventions. And so, as you were just saying, there are some tests we can do to know why we’re dealing with a lymphatic failure, are we dealing with CHF? Because those tissues respond differently to our assessments, right? So, CHF edema, for example, is very watery, very doughy, comes on very suddenly and it resolves pretty quickly. Where true lymphatic edema or lymphatic failure, it’s a progressive condition so initially, it might still pit because there’s not a lot of fibrotic changes initially.
But over time, if it’s not treated that fibrosis leads to hardening of that skin and over time, you’re not going to be able to pit that tissue, it’s not going to leave an indentation in their skin. This is when you can also, if it’s an upper extremity or a lower extremity, do that Stemmer sign where it’s literally, can you try to tent the skin on the back of a finger or the toe? If you cannot, you cannot tent that skin, you cannot touch down your fingers right now, you should be able to tent the skin. That means it’s a negative Stemmer sign so there’s no lymphedema. If you’re unable to tent the skin, we call unfortunately, I didn’t name it but we call it ‘sausage toes’. You cannot tent the skin because of those fibrotic changes, that’s a positive Stemmer sign and always, always indicative of lymphedema because of the fibrotic changes.
Now, somebody may have a negative Stemmer sign. It doesn’t mean they don’t have lymphedema. It just may mean that it hasn’t been around long enough to lead to fibrotic changes. And you’ll notice over time because if they don’t respond to conventional therapies, rest, ice, compression, elevation, it’s still persisting, start thinking this is likely a lymphedema. Okay, because most of these other conditions will respond to traditional therapies. Lymphedema needs to be treated a little bit differently.
Okay. So, not all swelling is equal. Lymphedema again is accumulation of fluid in the extremity or body part as a result of damage or loss of part of the lymphatic vessel system and again this is a mechanical insufficiency. There is a mechanical problem with how that system operates. Compared to just other edema, which is an abnormal excess accumulation of serous fluid not protein rich, but serous fluid in the tissues and the lymphatic system might be overwhelmed, but it’s not permanently damaged. So, this is just a dynamic insufficiency, the system is overwhelmed. You know, you’ve been on your feet all day, your shoes don’t fit as well, you’ve been traveling across country, your feet swell. At the third trimester of pregnancy, your legs get swollen. So, again, they’re different conditions, protein rich versus low protein.
So, the etiology of lymphedema, it can be either primary, congenital so somebody is born with a congenital defect of their lymphatic system or it can be acquired, which is a secondary lymphedema. So, primary lymphedema, 83% of the cases manifest before the age of 35, that’s called a lymphedema praecox and a 17% manifest after the age of 35 and they’re called a lymphedema tarda.
I don’t know why 35 is the cutoff but that’s basically where we start to see some of the different changes in our patients. Eighty-seven percent of all cases of the primary form of lymphedema occur in the female population. Again, we’re not sure if it’s a genetic component, if it’s hormonally related.
Now, it can occur at birth so it’s present upon birth that’s called Milroy’s or the more common form is Meigs syndrome, which typically happens around puberty. So, all of a sudden, sometimes it’s precipitated by an ankle sprain from a sport injury and the swelling just doesn’t resolve. This is often where it’s first manifested. And typically in this condition, the lower extremity is more often involved than the upper.
So, there are a lot of different congenital conditions involving lymphatic pathophysiology, it can be related to hypoplasias, hyperplasias, aplasias or inguinal node fibrosis or PIN mass syndromes. So, these are all different reasons why there could be congenital malformations of the lymphatic system. To really understand where that level of disruption is coming from, they need to do some tests basically to the lymphatic system, but they can basically determine where that dysfunction is happening.
So, this is just an example of the Milroy’s disease. You can see the child is present at birth. You can see the large edematous limbs. Typically, it’s under development of lymphatic tissue. So, if it’s not present at birth it happens within that first year of life, it can start coming on. And they can also be associated with an intestinal dysplasia. So, they can have a lot of nutrient absorption problems. And for these patients, we often see also upper extremity involvement in Milroy’s syndrome.
Then, there’s Meigs disease, it’s the most common of all primary condition, non-congenital familial type, it’s inherited. Again, it’s also a problem of hypoplasia with the vessels for some reason, usually triggered by traumas, as I said usually comes on around puberty, that’s why we think that there’s a hormonal component. And there’s a genetic predisposition in 30% of the cases, but usually this will be presenting bilaterally in the lower extremities. It won’t be symmetrical, but it will be bilateral presentation.
And another one I just wanted to point out because this gets missed is the Distichiasis syndrome and this is a variant of Meigs disease, but what happens is patients report that their eyes are constantly tearing or it feels like something’s in it because they basically have eyelashes on these inner or these along the posterior border of their lid, which become an irritant, right? It’s rubbing the eye and it’s causing irritation. And basically what happens, eyelashes develop at the same time embryologically as a lymphatic system as it comes out of the venous system, this is also linked to the lymphatic system. So, your eyelashes develop at that same time. So, what happens, these patients often go to the doctors and they remove those aberrant eyelashes, but they missed the underlying condition that these patients actually have a form of lymphedema. And it may not be clinically evident at the time, but sometimes it can appear alone or other times with heart defects, cleft palate, cranial sacral or cranial facial problems as well, but it does get missed. So, if you see these row kind of eyelashes where they don’t belong, you want to think that this is often a component of a lymphatic failure, primary form.
This is just a slide for your information to kind of show you some of the genes that have been isolated leading to different syndromes associated with lymphedema and down at the bottom is kind of showing you some of the chromosomal anomalies. One of the more common ones that we see is Turner syndrome and in some of the trisomies, but you can see all of them end up with some component of lymphedema. But they have an underlying genetic component as well. We’re getting better and better at isolating some of the genes that are actually involved with lymphedema.
So, what happened? So, we talked about congenital and then we have secondary and this is acquired in our lifetime. Anybody, unfortunately, basically could get this. It could be a side effect of dissection or radiation of the lymph node so commonly associated with breast cancer. Trauma or surgery especially repeated surgical procedures in the same area with a lot of excess scar tissue can lead to disruption to those local lymphatics. Chronic inflammation due to bacterial, viral, fungal, silica, which is podoconiosis parasites like filariasis, which will briefly mesh, even rheumatoid arthritis can lead to a secondary form of lymphedema. Malignant tumors and different types of blockages of the lymphatic and venous returns. So, there’s a lot of different ways that a secondary lymphedema can develop, okay? But it’s something that normally we’re born with a healthy system and something happens to it to disrupt it.
And we see these patients, right? Upper extremity, lower extremity and I’ll talk briefly about the, sorry, clickers not working too well. Okay.
So, incidents and prevalence. Now again, these numbers are likely low, but primary lymphedema here in the United States roughly about 2 million. Secondary, the acquired form a little bit more about 3 million, but in the world, the most common form is filariasis, which is the parasitic form of lymphedema. And that’s kind of interesting in that up to 120 million people actually have an active infection of lymphatic filariasis. It’s the leading cause of lymphedema in the world. There’s over 1.2 billion people at risk for developing this form of lymphedema. So, it is a very large problem. And, you know, lymphedema is really probably the biggest problem, we never really appreciate it or knew about, right, especially it’s how we’re educated. But I want to share with you too a little bit about this. It’s a parasitic infection spread by mosquitoes. They’re the vector. It’s the leading cause of disability in the world, the leading cause. It is actually 1 of 13 neglected tropical diseases and 1 of 6 that’s actually considered illimitable. So, we’re on track for eradication in Haiti by 2020-2022, which is great, that means there’ll be no new transmission of lymphatic filariasis, but all these patients have significant lymphatic damage with very involved lower extremity involvement.
So, again, it affects 120 million people in 83 countries across the world and in the Western Hemisphere as I said, Haiti is one of the endemic hotspots but so is Recife, Brazil and some of the other Caribbean areas.
Now, these are some newer numbers that I put in because I think this is important to realize that, you know, we know chronic venous insufficiency edema, which is actually a phlebolymphedema, affects about 300 million people, but I think that’s really underreported number. We know that 2 to 5% of all Americans have some changes associated with CVI so that could be anywhere from 6 to 16 million people with a phlebolymphedema. And we know that patients that have a CVI that are on the CEAP classification, it’s about 5% of the population. So again, that’s about 16 million people. So, these numbers are a lot higher than we originally appreciated and that’s a lot of people. And you can see the other facts about post-mastectomy, primary lymphedema, but really about one in every 40 people worldwide has some form of lymphedema. It’s a pretty large number and yet we’re still behind as far as educating people on how to manage this and even recognize it.
So, this was just information for you about cancer and lymphedema. I think the only time it sometimes gets properly recognized is in the context of cancer, but it’s not just in cancer patients, right. So, we do know that it’s often reported in breast cancer patients who had all or part of their breasts removed and axillary lymph nodes removed. So, the numbers generally range about 30%, that’s on average number of the patients that will develop a post-secondary breast cancer related lymphedema. But lymphedema in the legs may occur after surgery, after gynecological cancers or prostate cancer, even lymphoma or melanoma and this can occur in almost 70% of the population. And in men, it could be one or both legs, but also genitalia. And I think if you’re seeing some of these gentlemen with these swollen limbs, it’s really important to ask that sensitive question, do you have swelling in your genitalia as well? They’re not going to voluntarily give you that information, okay. But it’s a very big problem that can affect them in a lot of different ways and we can manage it. It’s treatable.
So, again, when we look at lymphedema patients, the single largest group is comprised of cancer therapy recipients basically here in the Western Hemisphere, but I’d actually argue that and say that it’s actually probably phlebolymphedema that we see more of. We don’t diagnose it as readily. So, the statistics are here for you just to have that information to compare. I know most of us in here probably dealing more with lower extremity patients, but it’s really important to factor in, you know, was it a gynecological cancer? Was it a prostate cancer? Was there problems with the inguinal nodes? Is there other issues going on with the genitalia, things like that because that can also be contributory to the formation of lymphedema. Okay.
And, you know again, lymphedema does not discriminate. We often hear about in context, it’s a female related problem, no. Men get affected too; they’re both subject to primary and secondary lymphedema. So, you can see again the statistics here. About 20% of breast cancer patients, men or women, will develop a secondary lymphedema, 30% of cancer of the genitals will get lymphedema, 50% who treated cancer of the vulva get lymphedema. So, it’s a large number of patients, 20 to 50% of these patients for various forms of cancer develop lymphedema.
Now, this is going back to your question about the Stemmer sign. So, you can see a nice picture of what this looks like. Well here, she cannot tent the skin. You see how hard and bound down those toes look? That’s kind of why they called sausage toes versus here, where you can tent the skin, okay. So, this would be negative, this would be positive.
Now, it’s due to that protein content, so that high protein content leads to fibrotic changes over time. So, this is a definitive diagnosis for lymphedema, okay? The positive Stemmer sign. But just because you can’t tent it doesn’t mean they don’t have it. They just may not have those fibrotic changes yet, very easy test to do, very easy test to do. Okay?
As stages, as a general rule, lymphedema is very slow but it’s progressive. We do not have a cure, but it’s readily manageable and I can tell you these become some of the most compliant patients when you tell them finally that what they had has a name, it is a disease, but it’s manageable because for a long time, they’ve just been told, don’t worry about it, there’s nothing that can be done. Sometimes they’re told they’re just fat. I mean there’s a lot of problems on how we approach our patients with these conditions. So, typically, it’s a condition that does move through stages and here the stage zero basically is subclinical. They really don’t have signs and symptoms at this point. The transport capacity of the lymphatic systems reduced, okay. So, it’s not working as well, but it’s able to cope with the normal amount of lymphatic load. So, these patients, it’s not often clinically detectable, but anybody that’s gone through a significant trauma or a surgery or cancer or CVI, they’re at this phase. They’re at a subclinical phase.
Sometimes they might have subjective symptoms saying, you know, my arm or my leg feels a little heavier or I know it a little more swelling in one leg than the other, but oftentimes this goes relatively undetected. But these patients are at very high risk for the ongoing development of moving into stage one. Okay. So, stage one, this is known as the reversible stage. So, here, you start to see an accumulation of protein-rich edema. So, these patients when you do your pitting assessment, their tissue will still pit. It’ll leave an indentation because it hasn’t been around long enough to really lead to a lot of fibrotic changes. It’s starting but it’s still able to pit. Sometimes they do reduce with elevation; this is why it’s called reversible. Sometimes they wake up in the next morning and they see that it has gone down. It tends to somewhat be transient, you’re not going to see a lot of skin changes, but this can progress to the next stage if we’re not addressing it properly. So, it’s really important to pick this up early and recognize it for what it is.
So, stage two, now it’s called a spontaneously irreversible. And the difference here is now you start to see the skin changes. So, again, you might see a clinically persistent swelling. It’s not going to get better by just elevation. It’s not going to get better in the morning. You’ll start to see that pitting becomes much more difficult to induce. It might still be able to give a little bit of an indentation but it’s not soft like it was before. The connective tissue proliferation becomes much more pronounced so you start to see this fibrosis happening and this condition can really be lifelong, but these are the patients we really want to treat, right? The stage ones and the stage two’s, because we can get them back to a subclinical stage. We can get them back to a near normal size.
The challenge comes with stage three. And this is unfortunately called lymphostatic elephantiasis basically just because the skin changes are very similar to elephant skin changes, unfortunately. No pitting is present. There’re significant fibrosis and sclerosis. You can see there’s a wide variation in how patients present in this stage, you can have pretty significant edema but a lot of heavy skin changes. Or you might see maybe not so much, maybe not a lot of edema, but a lot of associated skin changes. So, these are they’re really more complex patients and we see these clinically. These are the real challenges in our clinical settings.
So, again, this is just a summary slide for you. It’s important to appreciate whether it’s malignant or benign. The main difference is that if it’s truly a malignant lymphedema, the onset is very sudden. And it typically happens proximal to distal because the malignancy is usually involving a blockage of the lymphatics. Okay, it could be a tumor; it could be a spread of cancer. It could be a whole bunch of things going on. So, typically benign is very slow progression. It’s usually unilateral, normal skin color. They might have a positive Stemmer and there are really no complaints of pain with these. They have heaviness or achiness, but it’s not really painful. But when you look at somebody with malignant lymphedema what happens is it’s very sudden, it’s very rapid. It has that proximal to distal progression as you can see kind of here. They have report a lot of pain which is a telltale sign and you can see a lot of associated skin changes. So, we will sometimes see actually the metastases come through on these fungating wounds. So, that the metastases leaching into the actual skin dermaline and beyond. Okay, we’re good. That’s a good way to set up for the next session.
Any questions on, okay, I am going to start here.
Participant: [Indecipherable] [00:20:01] parasitic. There is a good chance it is not always parasitic.
Heather Hettrick: Yes. No, it’s not always parasitic. The leading cause of lymphedema worldwide is filariasis, which is a parasitic form of lymphedema. Luckily here in the States, we don’t see it unless somebody came to the States and actually already had it. The lymphatic elephantiasis or lymphostatic elephantiasis is just how we classify the stage three. So, you could still, you know, somebody who has lymphatic filariasis might be a stage three. We staged that lymphatic filariasis a little bit differently. They actually have seven stages just because of the way that the skin manifests. It’s a little bit differently due to the parasites but for here, us here in the States we usually will see stage one, stage two, stage three and stage three being the worst.
Participant: [Indecipherable] [00:20:51] something parasitic.
Heather Hettrick: Yeah. Maybe, or maybe they were worried about, you know, bioburden didn’t know what to do but, yeah, your history should help pick that up if they’ve emigrated from an at-risk country or something. It’s not something we get here.
Participant: [Indecipherable] [00:21:16]
Heather Hettrick: Okay. Sure. Well, and there’s also a form of tourniquet-induced lymphedema but that’s pretty evident because you can see where the tourniquet banding is. So, that’s another form too, but yeah, lymphatic filariasis is a little bit different. So, yeah.
Participant: Dermal phlebosclerosis [Indecipherable] [00:21:37]
Heather Hettrick: Yes. Dermal phlebosclerosis, we start to see those papillomatosis that really hard skin. You see a lot of associated skin changes just because it’s very progressive. Yes.
Participant: [Indecipherable] [00:21:48]
Heather Hettrick: So, a patient without CVI or diagnosed CVI that higher risk for DVT, yeah, yeah, they are actually, but I would also come back and say that if they have a lymphedema they likely have CVI. It’s just sometimes that piece is missed. Just again because of that interconnectedness. But what you’ll see with these patients sometimes is they become, it impairs their activities of daily living. They may not be ambulating or walking as well. They may not be wearing compression. They may not be getting care so they do become more at risk for things like a DVT or other complications. Actually, infection is a huge problem with these patients because of that fluid burden. Yes.
Participant: Filariasis that you are talking about could be elephantiasis [Indecipherable] [00:22:41]
Heather Hettrick: Yeah. Eradication actually involves an annual administration of albendazole and ivermectin. I think I said that right, Dr. Treadwell, I know you’re in here? He just walked out. Okay. It’s an annual cycle of two medications. Ivermectin is actually what we use for heartworm and albendazole basically and also use, we put this deck in fortified salts that people eat then just with regular cooking. So, usually after about five years of getting this annual dose it eradicate, it kills the parasites. It makes the patients really sick initially so they have a lot of flu like symptoms as the parasites die. But then they’re not continually to cause a problem and if they get bit by another mosquito it’s not going to transmit the disease to somebody else. Most of these patients are bit in childhood. It takes thousands of bites actually to actually get lymphatic filariasis, but clinically the signs don’t manifest until their early 20s. And by then the damage is quite extensive.
So, we talk about elephantiasis. So, it’s the way we describe the skin changes whether it’s due to filariasis, podoconiosis is a silica based form. We see this a lot in Ethiopia. So, the farmers are barefoot and they walk around and the volcanic soil is so sharp that actually gets in through the bottom of their feet and lacerates their lymphatic systems. So, it’s significant involvement of the feet and lower extremities. So, that’s a silica based form. And even patients here whether it’s post mastectomy or it’s trauma, or it’s radiation or it’s just whatever it is, you know, the severe stage of stage three we call lymphatic or lymphostatic elephantiasis because the skin changes are so pronounced. And that’s what’s unique about lymphedema is the protein that stays behind in those tissues that just leads to these massive fibrosclerotic changes. And we’ll talk about those skin changes coming up.
I thought there was another question, yeah.
Participant: [Indecipherable] [00:24:43]
Heather Hettrick: Lipodermatosclerosis?
Participant: [Indecipherable] [00:24:48] orange.
Heather Hettrick: Oh, Peau d’orange, yeah.
Participant: [Indecipherable] [00:24:52]
Heather Hettrick: Yes.
Participant: [Indecipherable] [00:24:53]
Heather Hettrick: Peau d’orange is something we actually see more in patients with lipedema. So, lipedema is an abnormal fat metabolism where there’s a propensity for fat to be located from the ankle bones up to the hips. It’s almost exclusively in women. It tends to be diet and exercise resistant, so it’s very problematic. But we have a lot of great research coming out and actually for here in Tucson at the U of A, Dr. Karen Herbst is the leading research in lipedema in the country probably the world and she’s right here at the U of A. There’s great resources on lipedema because what happens with lipedema since there’s an abnormal accumulation of adipose tissue, it fatigues the lymphatic system and it fatigues the venous system. So, a lot of these patients with lipedema end up with a, follow me here, phlebo-lipo-lymphedema. Okay, so they end up with a CVI, the phlebo, the lipo from the lipedema and the lymphedema. Very challenging to treat, but you do a modified approach with complete decongestive therapy. But this is why that differential is so important. So, these patients with lipedema, it’s painful. Sometimes it’s called a painful fat syndrome. They get a lot of easy bruising and it’s symmetrical, okay. So, sometimes they say the upper body doesn’t match the lower body. It’s often misdiagnosed as obesity. So, a lot of these patients do have an obesity component, but it’s not the same thing. Okay.
Great resources for you is if you Google the disease they call fat, it’s an excellent, excellent video and also if you go to fatdisorders.com. So, the disease they called fat and fatdisorders.com will go into a lot of great information for you because about 11 to 15% of the population of the female population has lipedema. And as few people that get diagnosed with lymphedema, there’s even fewer that could get properly diagnosed with lipedema. Okay. Great questions. Yes.
Participant: [Indecipherable] [00:26:54] patient with stage three where we have papillomatosis and …
Heather Hettrick: Yes.
Participant: With that, how [Indecipherable] [00:27:03]
Heather Hettrick: Okay, what was the what?
Participant: [Indecipherable] [00:27:09]
Heather Hettrick: So, what happens is, that’s a great question. So, we see that fibrotic tissue changes the papillomatosis. The proteins are really the underlying problem because instead of being picked up by the lymphatic system and brought back to the venous system, they’re staying out in those interstitial tissues and they break down and they lyse. And what happens that causes a local inflammatory reaction and then you end up getting collagen deposition. You get those macrophages coming in. You get the adipocytes coming in and it wreaks havoc in there. And it just ends up manifesting as this chronic inflammatory state, which manifests in thickening of the dermis and you end up seeing papillomatosis, which is telltale for lymphedema. You’ll see hyperkeratosis, you’ll see potentially even lichenification. We’re going to talk about those skin changes, but that’s really, the underlying problem is the protein. That’s why with these patients it’s really important that unless they’re on a diuretic for something that they need to be on the diuretic for, you don’t necessarily just give patients with lymphedema diuretic. It works great at mobilizing fluid, doesn’t do anything about mobilizing protein. Protein is hydrophilic, right, it wants more water so it brings more water to the area, proteins proliferate and you end up actually making the condition worse.
Same thing with the pumps. So, pumps are great as an adjunctive therapy after complete decongestive therapy. But if you use them initially, they’re great at mobilizing the fluid. They don’t do anything about mobilizing the proteins. So, you end up making the condition worse and sometimes they can get a fibrosclerotic ring at the top of where the cuff is. So, pumps are great and they’re very useful, but after we’ve shown that system how to reroute itself and I’ll talk about that shortly. Okay. Yes.
Participant: [Indecipherable] [00:28:59]
Heather Hettrick: Right. I think it goes back to that awareness into that education component because I think people see edema and they naturally, oh, it’s a fluid overload. We need to mobilize the fluid and let’s do that through diuretics, which makes sense clinically. But to really appreciate the differential diagnosis of why that edema is present and if it’s really truly a lymphedema or a phlebolymphedema unless they need it for heart or liver or renal, it’s not indicated. And there’s some good research to support that. I know sometimes that’s hard, me as a PT sometimes calling the physician saying, you know, is there a reason why they’re on the diuretic? And I try to explain to the physician that this actually is making the condition worse and I try to give the reasons why? And usually they’re fine, you know, once they understand that process, but it’s a normal thing for people to associate swelling diuretic.
And I think we just have to provide that awareness and that education will be really good with our differential diagnoses because there are times they need to be on a diuretic and that’s okay. But it shouldn’t be the only thing we use to manage the swelling with these patients. Good question.
This is a great segue moving into some of the skin changes that we see with phlebolymphedema and Dr. McGuire is going talk a little bit about this too. Okay. Because these are really classic telltale signs with these patients and it helps you with your differential. So, we know a lot about the CVI pathophysiology, right? They get the hypertension. It could be reflux, it could be insufficiency. It could be due to valvular failure of the deep, the perforating or superficial veins. It results in regurgitation, so we get that venous congestion, which leads to that venous hypertension again which leads to lymphatic hypertension.
Okay. So, that dermal backflow of the lymphatic fluid is critical. That’s what really causes fatigue. So, these impair the capillary function due to increased pressure. But remember too when you get dermal backflow, a venous congestion combine that with lymphatic backflow of lymphatic congestion, it will impair the arterial system too because now that arterial system cannot deliver blood and bring the nutrients and oxygen as well. So, it’s again that AVL combination.
So, this is just to review that, you know, we can have CVI or venous disease due to superficial vein failure or deep vein failure. Superficial is definitely more commons bilateral slow. It’s the valvular weakness. We end up with perfusion problems and sometimes they delay edema formation because we have that lymphatic safety factor. It can manage for a while, but then when it becomes impaired or dysfunctional then we start to see these more severe cases of phleboemphedema.
Whereas with deep vein failure, it’s usually post-thrombotic syndrome. So a clot basically causes destruction of the valve and it can happen a little bit more fast or more quickly. They get immediate edema, but that can too then lead to superficial venous hypertension. So, it’s a kind of a backwards approach. But both of these lead to venous disease and potentially ultimately ulceration.
Okay. So, with CVI we see that protein content initially tends to be low. So, these are kind of watery forms of edema initially. Typically the Stemmer sign will be negative in the beginning because it hasn’t been around long enough to cause a lot of the fibrotic changes. We know how these legs appear, it’s typical in the ankle gaiter area, the textures are brawny as you had talked about are hard. You can start to see a progression typically can be distal from the knee down, but we can see it from the thigh down as well. And they do respond to elevation initially the edema reduces with elevation or overnight when sleeping horizontally, but this is in the early stages, okay. So, our patients were talking about here with the phleobolymphedema had a little bit more of a longstanding progression.
So, CVI onset can be slow when their superficial involvement or rapid with deep system involvement. These patients complain of like achy feet, achy legs, distension type pain. Pain gets worse as the day progresses. They don’t like being in a dependent position. It’s uncomfortable. Their wounds if they have integumentary functions these are large weeping kind of superficial-type ulcerations. They’re not really painful per se, but they’re uncomfortable. Skin changes, we see that traditional atrophie blanche, which is the white scar tissue as you see on the bottom. There’s hemosiderin staining, which is really the body’s biological tattoo. So, the blood, the venous congestion sits in that interstitial tissues. The hemoglobin breaks down, releases the iron and the iron stains the skin from the inside out. It’s irreversible. Telltale sign for CVI. So, when you’re seeing your edema patients and you’re trying to say well, what’s going on here? If you see the hemosiderin you can automatically know there’s a CVI component.
And then you will see lipodermatosclerosis or, you know, that inverted champagne bottle presentation or that bowling pin presentation. The skin is tight, shiny. They have sometimes varicose veins, medial ankle flare and cellulitis is very, very common in these patients. And in fact, cellulitis is the number one reason why patients with lymphedema end up in the hospital. Okay. It’s very problematic because the fluid burden is so tremendous that it doesn’t really have anywhere to go and bacteria and microorganisms love stagnant fluid like that picture I showed you at the beginning, right, it’s just like a yucky pool. So, spider veins, medial ankle flare, varicose veins, telltale signs for venous changes as I said hemosiderin, lipodermatosclerosis, atrophie blanche. And then we see brawny skin that leathery hard kind of skin. So, what does this sound like? Lymphedema, right? The weeping blistering of the skin, sometimes the fluid, there’s so much fluid it has nowhere to go but out.
So, you might not even see a wound, but it’s leaking. It’s draining that’s oftentimes lymphorrhea. Yeah, you see that? Yeah, it has nowhere to go so it’s going to just pour out of that system, out of the legs. And sometimes that leads to that yellow crusting. You start to see that’s the lymphorrhea, which can be very caustic to the skin so then you end up with denudement.
Participant: [Indecipherable] [00:35:21]
Heather Hettrick: Oh, wow! Well, that’s a challenge, yeah. But it just shows you hemodynamics, yeah, you know, yeah, I am trying to balance that literally. That’s interesting. So, again, stasis dermatitis, it’s a chronic inflammation. It’s that inflammatory response. Again, what’s responsible for inflammation? Lymphatic system. So, they’re also very sensitive to products with perfumes, dyes, preservatives. So, we really want to use more natural type ingredients with these patients. My personal recommendation, again this is me speaking, is I love olive oil-based products and I like coconut oil-based products. The reason being is it doesn’t shut off the skins respiration system, that moisture vapor transmission rate. Mineral oils and petrolatum-based products tend to occlude that system. So, I think using products that allow that skin to still breathe while it still provides some moisturization and emollient component is very, very effective and most patients tolerate those products very well because they’re natural.
And then lymphatic skin changes. So, these are telltale for lymphedema that hyperkeratosis. Okay, it’s classic with these patients. Scaly brown, gray patches of over proliferated keratin and again, because of the protein burden. Papillomatosis that lumpy bumpy skin or fibrotic wart like projections of the skin. These are benign, but they’re subject to torsion and they can be torn off with, you know, depending on how you are using compression. The nice thing is what you can tell patients is you can reduce these limbs pretty significantly, but in time you can resolve a lot of the fibrotic changes, but that takes a long time. There’s a lot of techniques we can do through manual edema mobilization techniques, fibrotic techniques, using different types of foams that we can use under compression that can really help provide almost like a microstimulation to these fibrotic tissues, to break them down and help soften those tissues. And you can actually feel this happening under your skin.
What I like to do with my students so they appreciate what these tissues feel like is imagine a very unripe avocado. You know, how hard that feels, it’s kind of lumpy, it’s bumpy. That’s this. That’s that lymphedema, that’s not pitting. It’s that lumpy bumpy skin, right. And then as the avocado starts to ripen, it gets softer. You can push on it. It gives a little bit so that’s actually what starts to happen as we start treating these patients. You can feel that fibrosis softening and that’s a really remarkable feeling when you’re working with these patients. So, another way to kind of appreciate pitting is feel like a marshmallow, you know, a large marshmallow how that feels, that’s that pitting edema feeling. So, I do a texture lab with my students and they hate me because I analogize everything to food.
So, but again, they can get very thick and fibrotic skin resembling alligator skin or a pine tree bark as you can see here and it can be really problematic. But there are some great products that we can use to remove a lot of those crusty things that, you won’t remove the papillomatosis that comes with treatment, but a lot of that crusty formation, hyperkeratosis, there’s things that we can do to manage that. And again, remember these patients don’t typically have pain. If they do, you need to do more a thorough investigation. It could be an orthopedic impairment because of this heavy fluid, you know, burden in their leg or their arm. It’s altering the mechanics or it could be something else so you want to work them up for that.
Skin ulcers are very, sometimes uncommon, but actually what we do see is that lymphorrhea that leaking they end up leading to denudement in skin because it just it’s very caustic. Think about what’s in that lymphatic load, right, it’s all the waste products endotoxins, MMPs, things we don’t want really on the surface of our skin. It breaks down the skin barrier function. So, these patients end up with progressive fibrosis, even lichenification, which you can see here in this picture. It’s very hard tree-bark like skin and it’s very difficult to manage, but it too can change over time. So, remember, it’s usually a distal to proximal progression when it’s benign. And these patients, if it’s bilateral, it’s going to be asymmetrical. Sometimes it’s hard to tell by looking at the patient, but that helps you with your differential because lymphedema will always be asymmetrical. You might have to do your circumferential measurements to know which limb is more involved but it’s always asymmetrical.
And onset again with benign, it’s slow and it can be progressive. This can develop over many, many years. Okay. And this is just kind of a nice little summary for you talking about some of the different skin changes you may see, but remember these patients because of the fluid burden are really at risk for a lot of infections. Cellulitis being one of the more common, but they can get a lot of different problems with their skin and this is why skin care is a big component of what we do to help manage these patients which we’ll talk about a little while. Okay.
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