Mondors disease and Axillary Web: likely overlap

Options
kira66715
kira66715 Member Posts: 4,681
edited June 2014 in Lymphedema

With the recent posts about Mondor's and the scientific literature describing it as venous and rare, I did a pubmed search, and found it's also commonly found on the penis (go figure) and I reviewed the lecture notes I had from Jodi Winicour, and they have started to do biopsies of it using special staining to differentiate veins and lymphatics and the biopsies showed some sclerosed lymphatics, which are the same pathology as axillary web, and some cases of phlebitic veins:

http://www.ncbi.nlm.nih.gov/pubmed?term=Mera%20mondor%27s%20disease

Dear Editor,

Mondor's disease, first described by Mondor in 1939,1 is an inflammatory vascular disorder characterized clinically by a linear subcutaneous nodule most commonly affecting the thoracoepigastric, lateral thoracic or superior epigastric region. There is currently no consensus whether this disorder is of venous or lymphatic vessel origin.2 We recently encountered a rare case of Mondor's disease developed in a cervical lymphatic vessel.

This 20-year-old man was referred to our department for the evaluation of a subcutaneous induration on his left neck that was resistant to antibiotics prescribed by his internal medicine practitioner. On physical examination, a 5-cm long, Y-shaped, cord-like, moderately tender subcutaneous induration was detected (Fig. 1). Laboratory examinations of his blood including coagulation tests returned normal results. A biopsy specimen revealed that subcutaneous vessels were obstructed by organized thrombi and that the vessel walls were replaced by fibroblasts. Narrow recanalized lumens were seen in the area of organized thrombi (Fig. 2a). Weigert staining detected few elastic fibers in the affected vessels (Fig. 2b). Cells lining recanalized lumens and lymphatic vessels in the stroma were positive for D2-40, a specific marker for lymphatic endothelial cells (Fig. 3).3 Based on our clinical and histopathological findings, we made a diagnosis of Mondor's disease arising from lymphatic vessels.

Figure 1. A rope of Y character type of approximately 5 cm in length subcutaneous tumor with an oppressive pain was admitted of his left neck.
image

Figure 2. (a) There were some middle-sized veins or lymph ducts which were blockaded and the walls were replaced by fibroblasts and many re-communicated veins or lymph ducts were observed by the blockaded part. (b) Elastica van Gieson stain, in comparison with the nearby vein, showed a few elastic fibers in the sick part.
image

Figure 3. The stain showed that the positive parts of D2-40 were on the endothelial cells of re-communicated veins or lymph ducts.
image

Most patients with Mondor's disease present with lesions on the chest wall; for example, the thoracoepigastric, lateral thoracic or superior epigastric region. Our review of the published work detected only two cases of Mondor's disease of the neck.3 Although the etiology of this disorder is unclear, it may reflect the response of the local vasculature to trauma, muscular strain, febrile illness or contact dermatitis around the affected vessel.

As our patient is a carpenter who regularly carries heavy loads on his left shoulder that put strong pressure on the affected site on his neck, we posit that this occupational factor may underlie his development of Mondor's disease at this unusual site.

Whether Mondor's disease arises from veins or lymphatic vessels remains unclear. In our case, immunohistochemical study showed positive staining for D2-40, a specific lymphatic marker, and the absence of elastic fibers in the vessel wall. These findings clearly demonstrate that the lesion arose from lymphatic vessels. To the best of our knowledge, this is the first report of Mondor's disease in which the lesion stained positive for D2-40.

We presented a case of Mondor's disease with several peculiar features. First, the lesion was on the neck, a very rare site in patients with Mondor's disease. Second, occupational factors were involved in disease development and, third, there was immunohistochemical evidence that the disease was of lymphatic origin. To clarify the histogenesis of this disorder, other cases should be subjected to immunohistochemical study using D2-40 staining.

Another study of the penis, stated it was lymphatic and yet another study of the breast, showed that in 14 patients, the cord was a vein or artery:

http://www.ncbi.nlm.nih.gov/pubmed/19548938

J Eur Acad Dermatol Venereol. 2008 Feb;22(2):168-73.

Objective recognition of vascular lesions in Mondor's disease by immunohistochemistry.

Ichinose A, Fukunaga A, Terashi H, Nishigori C, Tanemura A, Nakajima T, Akishima-Fukasawa Y, Ishikawa Y, Ishii T.

Department of Plastic Surgery, Graduate School of Medicine, Kobe University, Kobe, Hyogo, Japan. ichise@med.kobe-u.ac.jp

Abstract

BACKGROUND: Mondor's disease (MD) is considered an inflammatory condition of superficial vasculitis that develops mainly in the anterolateral thoracoabdominal wall. The pathogenesis of the disease has been controversial, however, because of the lack of histopathologic methods for differentiating between the small vein and the lymphatic vessel.

AIM: To objectively examine the origin of vascular lesions in MD, we investigated the endothelial cells of their blood and lymphatic vessels.

METHODS: Immunohistochemical examinations were carried out on specimens involving vascular lesions from 16 patients with MD, using antibodies against von Willebrand factor and human lymphatic vessel endothelial hyaluronan receptor-1, which specifically discriminate between lymphatic and blood vessels.

RESULTS: The histopathologic findings clearly showed thrombophlebitis in 14 patients, a lesion originating in the lymphatic vessel in one patient, and sclerosis that consisted of the artery together with veins in another.

CONCLUSION: This study suggests that almost all cases of MD are due to thrombophlebitis, with a small minority due to lymphangitis or other conditions. We believe this study will contribute to the better recognition of the factual changes in the condition designated MD.

And a review of Mondor's:

http://www.ncbi.nlm.nih.gov/pubmed/19575733

Clin Exp Dermatol. 2009 Oct;34(7):753-6. Epub 2009 Jul 2.

Mondor's disease.

Alvarez-Garrido H, Garrido-Ríos AA, Sanz-Muñoz C, Miranda-Romero A.

Department of Dermatology, Hospital Clínico Universitario de Valladolid, Valladolid, Spain. helenaalvarezgarrido@hotmail.com

Abstract

Mondor's disease (MD) is a rare condition, which is considered a thrombophlebitis of the subcutaneous veins. It commonly occurs on the anterolateral thoracoabdominal wall, but it can also occur on the penis, groin, antecubital fossa and posterior cervical region. The clinical features are a sudden and typically asymptomatic onset of a cord-like induration, although some patients report a feeling of 'strain'. It is a self-limiting process that lasts a short period of time, which may be the reason why there are few reports about its diagnosis and treatment. Its pathogenesis has remained unclear, because of the lack of methods to reliably differentiate between veins and lymphatic vessels. Immunohistochemical staining for CD31 and D240 has been identified recently as the best method to distinguish small veins from lymphatic vessels, making it a valuable technique in diagnosing not only MD, but also many other diseases in which veins or lymphatic vessels are affected. MD has been associated with several systemic diseases such as breast cancer and hypercoagulability states, thus laboratory studies are recommended to exclude any possible systemic disorders. As this condition is usually a benign and self-limiting process, vigorous treatment is only recommended when the process is symptomatic or recurrent.

So, it appears that Mondor's can involve lymphatics as well as veins and the use of the new staining methods makes it clearer that lymphatics are involved.

So, Mondor's and Axillary Web likely have a clinical overlap. 

Kira

Comments

  • christine47
    christine47 Member Posts: 1,454
    edited March 2011

    thanks kira,

    some of the same info I read, looks like the etiology is unclear, mine is improving and I do not appear to have any TE, although I think this puts me at risk.  I will continue to stretch and observe.  I have symptoms, but this seems more related to my TEs not from the cords.

  • toomuch
    toomuch Member Posts: 901
    edited March 2011

    Kira,

    As always, thanks for your thorough research.

     "Narrow recanalized lumens were seen in the area of organized thrombi (Fig. 2a)."

     This must be the reason that gentle stretching, rather then popping, is now recomended for treatment. My first LE therapist popped my cords. I am guessing this further increases the risk of LE. Bummer.

  • Erica3681
    Erica3681 Member Posts: 1,916
    edited March 2011

    Makes sense to me. I've had both cording and Mondor's disease. The pain (sharp, stinging) was the same. So far, no lymphedema, but I know I'm at risk and am careful. 

  • kira66715
    kira66715 Member Posts: 4,681
    edited March 2011

    bump for eveberry

  • Hindsfeet
    Hindsfeet Member Posts: 2,456
    edited March 2011

    Thanks Kira...I scanned it...will read more carefully tonight. The first lumpectomy sentinal removal, I believe what I had was Monitors. It was in the chest wall, and looked like a vein. What I have now is cording. It looks like the pics in the information you posted. The nurse looked at it and said, oh, yes...that's cording. I have one large cord from the armpit to the elbow and another one that is smaller. Thanks again for the information. Power is knowledge. I don't want to do anything that would put me at risk for LE.

    How much exercise can you do with cording? What about a vibrator? Does it go away on its own? Can it recur? I've read so much lately that my mind is on overload...so If you mentioned this earlier, I might have either forgotten or missed it :) 

  • kira66715
    kira66715 Member Posts: 4,681
    edited March 2011

    Everberry, the ususal treatment for cording is gentle stretches and treatment by a PT. The stretches are like the yoga child's pose with arms overhead, or arms over your head with elbows straight, or clasp hands behind your neck--elbows out to the side--and bend to one side and the other.

    Likely best to avoid intense exercise while in the acute state of cording, as cords are lymphatics, so they're not doing their job.

    Usually cords go away, but can linger.

    They can recur.

    Never heard of using a vibrator, but it may be too rough on the tissues.

    Kira

  • christine47
    christine47 Member Posts: 1,454
    edited March 2011

    I have trucal cords which are resolving (no edema, that I can tell), I have pain but feel like its more at TEs and worse right after chemo.  I am thinking about going out this weekend and buying a light compression camisole, like a Spanx.  I can not stand any type of bra, the band just bothers me too much.  I am going to ask about seeing a LE therapist, but it may be a few weeks.  Any thoughts on compression for trucal cords? 

    I also have some back and muscle pain, had this off and on prior to BC, feel like it is related to poor posture and some scoliosis.  I have always had tremendous relief with warm baths, I admit I am still getting in tub some nights.  For me this is better than pain meds and now I can not take ibuprofen because of chemo GI issues this is my only solutions, do you all think this is harmful?

    Any input is helpful, Thanks.

  • Binney4
    Binney4 Member Posts: 8,609
    edited March 2011

    Christine, a warm bath is a fine thing (for all kinds of reasons!Smile). It's the long, hot, soaky baths where you raise your core body temperature that can cause lymphedema problems. The National Lymphedema Network says to keep the water temp around 102°, as a guideline.

    You can sure try a cami, and if it doesn't cause you more pain with the cords it should be fine. Might even feel better. Give it a try, and do let us know what you discover.

    By all means get a referral to a well-trained LE therapist -- an evaluation, baseline measurements, and risk reduction tips are invaluable.

    Be well!
    Binney

  • christine47
    christine47 Member Posts: 1,454
    edited March 2011

    Binney,

    Thanks for your response, I just got back from the mall and bought a spanx brand camisole, it is very smooth and comes up nice and high under the arms where I have such a full feeling. So far it feels good (been wearing it about 1 hr).  I think this may just be the ticket.  Should I sleep with it on?? 

  • Binney4
    Binney4 Member Posts: 8,609
    edited March 2011

    Christine, I find camis hard to sleep in -- they twist or roll up. Yuck! You might want to order a breast/chest binder like the Expand-a-band. You'll find a link here:

    http://www.stepup-speakout.org/breast_binders_lymphedema.htm

    I've found the Expand-a-band folks to be quick and efficient. Be sure to order the lined one -- much more comfortable.

    Really glad the Spanx is working well so far -- whew!Smile

    Gentle hugs,
    Binney

  • christine47
    christine47 Member Posts: 1,454
    edited March 2011

    Binney,

    I really think some of you should be getting paid for your knowledge on these sites!  I wore the Spanx for several hours this afternoon, for the most part I think it felt better, maybe just wishful thinking.  I did take a short nap, and it rode up some, so your are right, probably should not sleep in it.  I have an elastic binder from my initial surgery (BMX with TEs)  and just put that on as I lounge in bed to watch TV, not going to push my luck sleeping in it yet.

     I believe it is the same as on the site.  It is not lined, so I may try and order a lined one tommorrow.  The pressure feels good right under the breasts. 

    My copay for a specialist is $20, do you take visa? 

    thanks again.

  • steelrose
    steelrose Member Posts: 3,798
    edited March 2011

    Just wanted to chime in and let everyone know that after two months of dealing with cording, my arm is back to normal! I've been religious about stretching, and it seems to have worked. No guarantee that it won't return, but I'm 100% better right now. There's hope, ladies... hang in there!

    Also, am a fan of the Spanx camis. Very comfortable.

    Best wishes to all...

    Rose.

  • moogie
    moogie Member Posts: 499
    edited March 2011

    Can anybody describe the difference between MONDOR'S and WEB SYNDROME pain? Mine is burning and sharp down both sides, particularly tender in the middle of the ribcage on the sides. The pain down my arms is like nerve pain with a tightness in my armpit and radiating to my neck.

     Finally go for a therapy visit this week after almost 6 months waiting for insurance situation to be ironed out. Pain is bad, and even a hug can set it off at my sides.

  • Hindsfeet
    Hindsfeet Member Posts: 2,456
    edited March 2011

    moogie...I understand. I have the burning pain down my left side. I don't have pain that goes to the neck. My underarm is actually feeling better today. I do have web cording of three long strands at the surgical node site to the elbow. At first it was one, then two, and this morning I notice another one beginning. Kira has excellent website explaining cording...web cording. I am hoping with early intervention that it will resolve before becoming something like lymphdema. I've learned a lot here since my surgery a few weeks ago that enlightens me to know what to do right now...prevention is key.

    Monitors...I had that four years ago when I had the right nodes removed. It seemed like a large vein that ran through the breast to the top part of my stomach...it was weird. After one pt appointment and  exercises in a matter of a few weeks or so it disappeared. The cording seems to be worse in that it is hard to lift my arm.

    I hope your insurance works out. If you go to the hospital, usually they will work with you. It is important to take care of this before it becomes lymphdema. One thing I learned here is ... not to be tough and over use your affected arm. Not worth it.

  • steelrose
    steelrose Member Posts: 3,798
    edited March 2011

    I had rib pain as well, same side as affected arm. It was very hard to stretch through this as pain was intense. The rib pain was the first to subside, followed by my arm. So weird. I would encourage all to get PT if possible!

    Rose.

  • kira66715
    kira66715 Member Posts: 4,681
    edited September 2011

    bump for Tina--is this the thread?

    Kira

  • Anonymous
    Anonymous Member Posts: 1,376
    edited September 2011
    Kira, I think it's this one. On the fourth page under the section, "Cause, symptoms and types of cord," it mentions a woman who had surgery to diagnose a lump that was felt while she was being treated for cording. Figure 1a in the appendix is a photo of the cord with palpable lump.




    http://www.stepup-speakout.org/elisabethaws.pdf

Categories