Adiposis Dolorosa/Dercum's Disease Wikipedia info copied

Adiposis dolorosa, also known as Dercum's disease[1] is a rare condition characterized by multiple, painful lipomas. These lipomas mainly occur on the trunk, the upper arms and upper legs. The understanding of the cause and mechanism of Dercum disease remains unknown.[2] Possible etiologies include: nervous system dysfunction, mechanical pressure on nerves, adipose tissue dysfunction and trauma.[3] HistoryEdit Dercum's Disease was originally described in the medical literature in 1892 by the American doctor Francis Xavier Dercum.[4] Currently, the foremost authority on Dercum's Disease is Dr. Karen Herbst, M.D., Ph.D. EpidemiologyEdit Dercum’s disease most commonly appears between the ages of 35 and 50 years of age.[3] It is five to thirty times more common in women than in men.[3] Originally, Dercum proposed that the condition mainly affects postmenopausal women. However, a 2007 survey has revealed that 85.7 percent of the included patients developed Dercum’s disease before menopause.[3] The prevalence of Dercum’s disease has not yet been exactly established.[3] SymptomsEdit Multiple lipomas and neuropathic pain are the cardinal symptoms of this disease. The associated symptoms in Dercum’s disease include obesity, fatty deposits, easy bruisability, sleep disturbances, impaired memory, depression, difficulty concentrating, anxiety, rapid heartbeat, shortness of breath, diabetes, bloating, constipation, fatigue, weakness and joint and muscle aches. [5] Regarding the associated symptoms in Dercum’s disease, only case reports have been published. No study involving medical examinations has been performed in a large group of patients.[3] CausesEdit The aetiology of the disease is unknown. Proposed, but unconfirmed aetiologies include: nervous system dysfunction, mechanical pressure on nerves, adipose tissue dysfunction and trauma.[3] DiagnosisEdit A diagnosis of Dercum's disease is based on patient history and the physical findings. There are no specific laboratory test for this disease. Ultrasound and Magnetic Resonance Imaging can play a role in diagnosis.[6] TreatmentEdit Few convincing large studies on the treatment of Dercum’s disease have been conducted. Most of the different treatment strategies that exist are based on case reports.[3] Treatment of Dercum's disease is usually targeted towards pain relief rather than lipoma removal. Currently, there is a lack of scientific data on the use of integrative therapies for the treatment or prevention of Dercum's disease.[7] There are not enough studies done to prove that diet and supplements could help with the disease.[8] Treatment methods include: Surgery Surgical excision of fatty tissue deposits around joints (liposuction) has been used in some cases.[9] It may temporarily relieve symptoms although recurrences often develop. Medication[3] Traditional analgesics The pain in Dercum’s disease is often reported to be refractory to analgesics and to non-steroidal antiinflammatory drugs (NSAIDs}. However, this has been contradicted by the findings of Herbst et al. They reported that the pain diminished in 89% of patients (n=89) when treated with NSAIDs and in 97% of patients when treated with narcotic analgesics (n=37). The dosage required and the duration of the pain relief are not precisely stated in the article.[3] Lidocaine An early report from 1934 showed that intralesional injections of procaine (Novocain®) relieved pain in six cases. More recently, other types of local treatment of painful sites with lidocaine patches (5%) (Lidoderm®) or lidocaine/prilocaine (25 mg/25 mg) cream (EMLA®) have shown a reduction of pain in a few cases.[3] In the 1980s, treatment with intravenous infusions of lidocaine (Xylocaine®) in varying doses was reported in nine patients. The resulting pain relief lasted from 10 hours to 12 months. In five of the cases, the lidocaine treatment was combined with mexiletine (Mexitil®), which is a class 1B anti-arrhythmic with similar pharmacological properties as lidocaine. The mechanism by which lidocaine reduces pain in Dercum’s disease is unclear. It may block impulse conduction in peripheral nerves, and thereby disconnect abnormal nervous impulse circuits. Nonetheless, it might also depress cerebral activity that could lead to increased pain thresholds. Iwane et al. performed an EEG during the administration of intravenous lidocaine. The EEG showed slow waves appearing 7 minutes after the start of the infusion and disappearing within 20 minutes after the end of the infusion. On the other hand, the pain relief effect was the greatest at about 20 minutes after the end of the infusion.[3] Based on this, the authors concluded that the effect of lidocaine on peripheral nerves most likely explains why the drug has an effect on pain in Dercum’s disease. In contrast, Atkinson et al. have suggested that an effect on the central nervous system is more likely, as lidocaine can depress consciousness and decrease cerebral metabolism. In addition, Skagen et al. demonstrated that a patient with Dercum’s disease lacked the vasoconstrictor response to arm and leg lowering, which indicated that the sympathicusmediated local veno-arteriolar reflex was absent. This could suggest increased sympathetic activity. An infusion of lidocaine increased blood flow in subcutaneous tissue and normalised the vasoconstrictor response when the limbs were lowered. The authors suggested that the pain relief was caused by a normalisation of up-regulated sympathetic activity.[3] Methotrexate and infliximab One patient’s symptoms were improved with methotrexate and infliximab (Remicade). However, in another patient with Dercum’s disease, the effect of methotrexate was discreet. The mechanism of action is unclear. Previously, methotrexate has been shown to reduce neuropathic pain caused by peripheral nerve injury in a study on rats. The mechanism in the rat study case was thought to be a decrease in microglial activation subsequent to nerve injury. Furthermore, a study has shown that infliximab reduces neuropathic pain in patients with central nervous system sarcoidosis. The mechanism is thought to be mediated by tumour necrosis factor inhibition. [3] Interferon α-2b Two patients were successfully treated with interferon α-2b. The authors speculated on whether the mechanism could be the antiviral effect of the drug, the production of endogenous substances, such as endorphins, or interference with the production of interleukin-1 and tumour necrosis factor. Interleukin-1 and tumour necrosis factor are involved in cutaneous hyperalgesia.[3] Corticosteroids A few patients noted some improvement when treated with systemic corticosteroids (prednisolone), whereas others experienced worsening of the pain. Weinberg et al. treated two patients with juxta-articular Dercum’s disease with intralesional injections of methylprednisolone (Depo-Medrol). The patients experienced a dramatic improvement. The mechanism for the pain-reducing ability of corticosteroids in some conditions is unknown. One theory is that they inhibit the effects of substances, such as histamine, serotonin, bradykinin, and prostaglandins. As the aetiology of Dercum’s disease is probably not inflammatory, it is plausible that the improvement some of the patients experience when using corticosteroids is not caused by an anti-inflammatory effect.[3] Alternative Treatment CVAC sessions Dr. Karen Herbst conducted a pilot study utilizing CVAC™ sessions in 2010. A touch-free method was utilized, which is delivered via a high-performance altitude simulator, the Cyclic Variations in AltitudeConditioning™ (CVAC™) process. As a pilot study, 10 participants with AD completed pain and quality of life questionnaires before and after 20–40 minutes of CVAC process daily for 5 days. The Cyclic Variations in Altitude Conditioning™ (CVAC™)(CVAC™ Systems, Inc; Temecula, California, USA) process is an alternative and potentially revolutionary method of touch free cyclic hypobaric pneumatic compression for treatment of tissue edema and, therefore, edema-associated pain [4]. The CVAC service is available for improved fitness only. It is not available to diagnose or treat any disease or other medical condition [5]. References External linksEdit Dercums Disease Research Dercums Foundation NORD (National Organization of Rare Disorders) Dercum's Disease (www.dercum.org) Dercum's disease on e-medicine Dr. Karen Herbst, M.D., Ph.D. Online Support Groups

Current 11/18

Since taking the anti inflammatory and muscle relaxers regularly I have had relief from the dead arm syndrome I was experiencing. I have discontinued the blood pressure med entirely (advised doc at last appt. & blood pressure check was ok , my normal)

However, attempting to stop or skip doses of other meds has not shown good results. Immediately I begin to feel the symptoms in my right arm/ shoulder/neck again. I try sleeping on my stomach but ultimately wake on my back or side with pain and numbness with continued weakness for a period of time after (seems longer each time).

The doc & I also discussed prior diagnosis of Costochrondritos vs.Tietze syndrome which is associated with dercums & the only differentiating factor of actual/palpable swelling, which is what was felt at the lower right side of my ribcage and at the sternum.

Over the last few weeks I have begun to have issue with right knee, stability & annoying noises & catching sometimes. Then one night, With deep knee bend (over side of tub with pressure on/below knee cap) I experienced an excruciating sensation of burning and tearing along the outside (laterally?). It almost felt like pulp beneath my skin was ripping or pressing through.  Since that occurrence two weeks ago I have realized I pivot/twist quickly at work with added weight of trays, a lot! And now even just sitting with it bent aggravates. It's not visibly swollen or apparently different from the left knee (possibly because I'm already on the prescribed meds that would be indicated) but with just the slightest touch below the front of the kneecap I feel pain laterally.

I have begun to wonder if the (AD adiposis dolorosa/aka dercums) that affects my adipose tissue is having the same progressive destruction now on my cartilage?

The arm issue began with nerve pain and progressed with popping and clicking and cracking in my shoulder and neck. Now, my lower back, hips and knees seem to be following suit.

I already have known back issues from levo-scoliosis to synovial filled bone cyst, and cervical stenosis to degenerative arthritis it seems the immuno- component of this wants to help my problems but over compensates somehow making everything much worse!

Mid-back / Thoracic nerves to organs article

http://www.ucmg.net/mid-back-3-5-06.htm

Connective Tissues

<a href='http://biology.about.com/od/anatomy/a/aa122807a.htm'> biology.about.com/of/anatomy/a/aa122807a.htm</a>

Complication ~ A story of Dercums

My almost 24yr old daughter (and mother of Priscilla the most amazing granddaughter EVER!) was recently involved in a car accident and upon her sBarubsequent medical care Scheuerrmans spine was inadvertently discovered.

<a href= "http://www.spine-health.com/conditions/spinal-deformities/scheuermanns-disease-thoracic-and-lumbar-spine"> Scheuerrmans Spine </a>

Not surprising since MVAs are the most prevalent way inwhich spinal deformities come to be diagnosed.  And interestingly enough, as history repeats itself, similar to my own experience.

Back in 1985, when I was graduating from high school on the way to the ceremonies I was involved in an incident where i was ejeted from the car. 

Family Ties ~ A story of Dercums

So now that you know a little about how I came about this affliction its appropriate for me to mention the part where I am 46yrs. (inside 76) mother & grandmother, myself.

And while Dercums seems to be more prevalent among females vs. males,  of my 3 children (a daughter & 2 sons) my middle child Joshua was affected prior to his birth.  Born with a Mesenchymal Hamartoma... he spent the first few months of his life at Shands (renowned teaching hospital in Gainesville FL).  Where the majority of his Liver was removed.  Long story short, I am happy to report, he is soon to be 23yrs. of age without any current medical difficulties.


The surgeons and specialists all said not to blame myself.. while they tried to save his life indicating that it was an unexplained phenomenon.  That although its not understood why ... occasionally during development cells become confused and grow in a disorganized manner creating a tumor.

In this case I think I now can accept full blame as there is a close relationship between the lumps in my body and and the type of lump that developed in my son's liver.  Even though I didn't have the first clue that I carried this affliction (much less pass it on) until many many years later... in fact, not until a few years after my youngest was born ('97),  post-hysterectomy ('00),  and lipoma removal ('01) did my GP and I discover Adiposis Dolorosa and how all the pieces of my puzzle fit.


Now it seems that I can explain some of the previously unexplainable... its a relief to me even if no one else gets it.
This zebra isn't crazy!

 (*see prior blog entry for zebra reference.

Important note here: the following is where I believe the identifiers of Dercum's Disease describe emotional disturbance.)

I can't stop thinking about my poor grandmother's suffering and what may be in store for the lower branches of my family-tree.  It particularly haunts me what my daughter might endure especially since she resembles me so very much... not necessarily beginning or ending with our schuerrman's spine(s).



Stay Tuned for next instalment..

going the distance ~ A story of Dercums

I suppose I thought my grandmothers ailments were all simply part of the process of aging and various unrelated conditions .  However, now, because of my own diagnosis (approx 13 yrs ago with Dercum's) combined with the knowledge I've gathered since...  & in light of the familial link... I am convinced by the similarities of our health, when I talk with Meemaw about her medical history and symptoms ... that she must have it too!

That's part of strangeness of  Dercum's; it encompasses a vast array of conditions.  Nasty things, besides the painful lipomas (the ones that aren't supposed to hurt), displaying symptoms like IBS, Fibromyalgia, deep arthritic & nerve pain.  Inflammation discomforts with flare ups that make your bones ache and your fat hurts you all over.  Weakness and sleep disturbances are common so it seems. But, wait there's more.... the hits just keep coming ...I won't bore you with the rest of the details right now except to say that no matter what the complication might be ~ it causes pain disproportionate  (in comparison to others with similar difficulties in absence of this rare condition).

So with all that being said imagine you're a doctor whose patient comes to you on a regular basis with some new mysterious complaint seemingly unrelated to other symptoms or condition.   You may think this person is a hypochondriac , or a drug seeker because it couldn't possibly be as bad as they are describing nor as diverse as they are trying to explain.

Armed with all that medical knowledge when you hear those hooves approaching do you look for a horse or a zebra?  More than likely you address the issue conservatively and make a referral.

Where does that leave .. the zebra?
Feeling like maybe they're a little crazy ..

a walk in my Grandmother's makeup ~ A story of Dercums

I say "A" Dercum's Story because this one is simply mine.  The stories are many and most will probably go untold or possibly even discovered. They are vast & varied yet, like deja-vu, have a sense of familiarity.. hard to describe and even more difficult to understand unless you can walk in the suffering shoes.

Dercum's

My story begins with Meemaw, my maternal grandmother,  the most gracious, kind hearted, God loving, southern belle that has ever blessed this earth, undisputed!

She would be proud to tell you herself that today she is 87 + years of age.  She has done and seen more in the last 15 years of her life than most will ever get to experience, ever.  (Can you tell she makes me proud!?)

I could mearly hope to dream of becoming even half the woman of strength, character, determination and compassion.... I just wish that the part of her genetic make-up, where I most closely resemble her, Didn't Exist!!

I watched through my lifetime her subtle aches and pains that grew sometimes into bed rest others to full-on medical crisis, all the while she would try to hide her discomfort and just kept going til she simply couldn't anymore.  Always plagued by arthritis, joint problems, swelling & inflammation, a lifetime of thyroid pills, angina / heart / bp issues...I could go on (and I will) but, now I walk that same mile so I have a different appreciation for how much she really has given and continues to give of herself.

An interesting fact to note at this point is that she has never received a diagnosis (other than from me, and I'm certainly no doctor) of Dercum's Disease (aka: Adiposis Dolorosa, adipose tissue disorder, Fatty Tissue Rheumatism, et al) yet has received diagnosis of nearly every other complication or symptom,  associated with this rare condition!!

To be ccontinued...