Lori Kelly is a 38 year old F who presents with a history of lumps in fat tissue. Over last year has gotten worse with increase in size of lower pannus; pain and not sleeping. Has hundreds of nodules on her abdomen.
Diet: Only eats once a day and weight has increased.
One time her whole side of the body was numb including ½ tongue. Never found out what it was. With increase in HR gets nauseous, dizzy. Passed out once and almost passed out again. Does not drive. Has not had a tilt table test. ENT does not think it is vertigo or inner ear.
Since August 18th when HR and BP increased and could not feel her bladder, everything progressed – got more tired, head hurts all the time, not driving, has hallucinations (thinks she talked to someone and has not); sleeps real hard and has very vivid dreams. Head and arms will twitch right as she goes to sleep. Head twitches just around sleep.
Works very hard to not let people know that she is ill. Also puts energy into her job as a photographer that she may not have but must do in order to continue the job.
Prior to the birth of her second child she was healthy. In 2002 with the birth of her child had hemorrhage and blood clot. Her daughter was hospitalized at that time as well and her father died so lots of stress. Grandmother and uncle died within days of each other. Very stressful time.
Review of Symptoms:
General: Dry eyes. Feels that when she lies down, that fluid pools in her head. Pain is in head, legs, arms and stomach. Fatigue almost constant.
HEENT: Blurred vision. Eye pain.
Cardiovascular: Heart rate: When lies down BP normal. When stands HR is 140 and BP elevates. Had testing for adrenals and kidneys; all testing normal. Flushes all the time. When takes a deep breath, her hands and feet get hot.
Pulmonary: Sighs frequently (almost forced).
Musculoskeletal: Elbows hurt. Knee on left feels tight. Joint pain daily.
GU: Still having monthly PMS. No bladder sensation of fullness – started on August 18th 2010 lost sensation in her bladder. Neurologist said not neurological due to normal bowel movement and sensation. Urologist thinks it is neurological. Decreased libido. Pain with intercourse.
Neuro: Most of the time her hand is numb lateral (ulnar) and stops above wrist. Poor concentration, confusion, headaches, head pressure, indecisive. Can’t go to sleep nightly.
Vascular: Sensitive to heat. Hands and feet “go to sleep” feel numb. High blood pressure. Dizzyness on arising. Irregular heartbeat.
HEENT: Wears glasses; Flushed face; Small hard nodule right pre-auricular. Increased fat on head; mild superior increasing posterior.
Neck: Supraclavicular fat bilateral. Fat on neck is grainy especially on the anterior surface, superior and lateral, bilateral. The grainy nodules are very small
Arms: Increased fat on upper arms; nodular, thick fat; some increase in weight to the fat of the arms. Nodular fat on lower arms, nodular diminishing mid-way to hand. Stemmer’s sign negative on hand. Increased fat between thumb and index fingers bilateral, dorsal surface of hands
Chest: Large breasts. Nodular fat under bra – not large and fibrotic
Abdomen: Large pendulous abdominal fat with petechiae and silver striae
Back: Tender low back; striae along back (silver)
Hips: Tender hips bilateral
Legs: Cellulite of thighs. Nodular fat around and in popliteal area extending down on leg. Increased fat around ankle bilateral R>L. Stemmer’s negative on feet
Bleeding time normal
EKG, stress test and ECHO normal.
Angioedema labs: C1q level normal; C2 level normal; C1-INH level high (28H – normal 11-26 mg/dl); C1-INH activity >100%. CRP <0.1.>
CT angiogram left extremity normal 10/7/10.
Lipedema/Other Lymphedema 457.1
Adiposis dolorosa 272.8
Postural orthostatic tachycardia syndrome (autonomic dysfunction) 337.8
Chronic pain syndrome 338.4
Chronic fatigue syndrome 780.71
1) From her family history and the feel of Lori’s tissue, she has lipedema. Lipedema is a congenital fatty enlargement of the legs almost exclusively seen in women by the third decade; two cases have been reported in men. According to an epidemiologic study by Földi E and Földi M, lipedema affects 11% of the female population. Of women with lymphedema, estimates are that up to 17% have lipedema as well. Lipedema was initially described by Allen and Hines in 1940; its etiology remains unknown and it remains under-diagnosed. In 1951 Wold et al. analyzed 119 cases and provided the diagnostic criteria for lipedema:
· Almost exclusive occurrence in women
· Bilateral and symmetrical manifestation with minimal involvement of the feet
· Minimal pitting edema; the Kaposi–Stemmer sign is negative
· Pain, tenderness on pressure
· Increased vascular fragility; easy bruising
· Persistent enlargement after elevation of the extremities or weight loss
· Arms are affected 30% of the time
· Hypothermia of the skin
· Swelling worsens with orthostasis in summer
· Unaffected by caloric restriction
Synonyms of lipedema include lipalgia, adiposalgia/adipoalgesia, adiposis dolorosa, lipomatosis dolorosa of the legs, lipohypertrophy dolorosa, painful column leg, painful lipedema syndrome.
In lipedema, there are increased macrophages in tissue, a microangiopathy (leading to increased bruising), dilation of subdermal capillaries which can be seen as telangiectasias and petechiae on the skin, dilation and leakage of lymphatic vessels in the subcutaneous fat - leaking lymphatics into subcutaneous fat increases growth of adipose tissue in mouse models. Because Lori’s lipedema is more widespread, i.e. to the neck on the abdomen, on the lower arms, we call this adiposis dolorosa or Dercum’s disease type 2.
The mainstays of treatment for lipedema and Dercum’s disease are as follows:
A) Manual lymphatic drainage (also known as complete decongestive therapy). This includes decongestive massage, wrapping, garments, kinesiotape and a sequential compression device. A trained therapist in your area can be found here: http://www.vodderschool.com/ and here: http://www.lymphnet.org/resourceGuide/manualDrainage.htm#CA
B) Selenium - Selenium has been shown to decrease edema in tissue in multiple placebo controlled trials and reduces the incidence of erysipelas infections in patients with chronic lymphedema at various sites; it lowers oxygen radical production in part by increasing glutathione peroxidase and thioredoxin reductase; it dcreases glycoprotein adhesion molecules (eg, P-selectin, ICAM-1, VCAM-1, ELAM-1) in a dose-dependent manner which may unclog lymphatic capillaries; it significantly enhanced cellular immunologic reactions improving efficacy of cytotoxic T-lymphocytes and stimulating macrophages degradation of excess tissue proteins. The US National Research Council has defined the individual maximum safe dietary intake for selenium as 600 μg daily and the no adverse effect level as 800 μg daily. Anticipate a possible increase in pain or decreased well-being as the toxins come out of the tissue. However, if you don’t feel good taking the selenium, then stop it. You can get sodium selenite solution from Life Extension at 50 mcg per drop (you take 5-10 drops). Many other internet sites also sell the liquid. You can buy 250 mcg tablets from twin labs (try iHerb).
C) Enzymes such as serrapeptase and wobenzyme have also been shown to decrease lymphedema (www.lipomadoc.org). Take 20,000 international units of serrapeptase or one tablet of wobenzyme or fibrozyme in-between meals or 100,000 international units serrapeptase at bedtime.
D) Matrix metalloproteinase (MMP) inhibitors - To strengthen blood vessels, horse chestnut seed extract as an MMP inhibitor or other MMP inhibitors (see www.lipomadoc.org) May be helpful taken as 250-300 mg with meals. You can also eat one square of dark chocolate every day (>70% cocoa or more). Choose only one bioflavonoid as if you take more bioflavonoids, the effect is lost.
2) Autonomic dysfunction – I am very concerned that Lori has autonomic dysfunction similar to POTS. I am sure this has been assessed previously. I think the flushing is part of this dysfunction. To help us understand what is going on, she should have an EMG of her arms, bilateral. In addition, if she continues without a diagnosis, she may benefit from seeing Dr. David C. Agnew, Neurology and Pain Specialist; 533 East Micheltorena Street, Suite 202, Santa Barbara, California, 93103; 805-962-1116. He should evaluate Lori for autonomic dysfunction and help her seek a diagnosis. He has seen another patient with Dercum’s disease and I have spoken to him so this will not come out of left field for him. Dr. Agnew may be able to tell Lori if thoracic outlet syndrome exercises may help her. She can also get an MRI, MRA, MRV with Dr. James Collins at UCLA, even though I recognize that the CT angiogram on the left was normal. There is more than one thoracic outlet.
3) The edematous pannus that Lori has is very concerning. It is large and if the edema continues in this area, then she is at risk for developing a larger and larger pannus, which, since it is accompanied by pain, will decrease her quality of life further; in the least, it is not helping Lori to feel better. Edema in tissue is stagnant, becomes inflammatory and eventually there is liposclerosis (fibrosis of fat tissue). This can already be felt in the pannus. I recommend that Lori undergo manual lymphatic drainage by a certified therapist (Lymphedema Clinic in Stockton) that can also recommend compression garments for Lori to help with the drainage. If after 3 months there is no improvement, then I recommend Lori have a pannectomy with good compression for 6 months after.
4) Complement labs. Because of the elevation in C1-INH, I would like the labs to be repeated at National Jewish Hospital. I will send the form along with this note. Just C1-INH level and function need to be rechecked.
5) Prevention: To improve the health of the fat tissue, I suggest Lori get some light compression garments such as those by UnderArmour. It must say “Compression”. Lori can shop for these locally and should wear these daily. She also should have an air cushion on which she sits at home, in the car and elsewhere. The air cushions tend to be better than gel cushions. Also, do not cross the legs and prevent lymph flow!
6) Exercises: The best exercises to get lymphatics flowing include swimming, whole body vibration (you can buy your own or try first at a local chiropractor or wellness center), elliptical, stationary bike and walking. Wear compression when exercising (except in the pool). Avoid exercises that build up lactic acid quickly and/or to a great extent.
7) I think Lori has a central hypersensitivity syndrome. The body is over-responding to the inflammatory lumps in her tissue and she is building a framework to continue in this pattern for her lifetime. Once thing that may help decrease her pain is counseling for her past history of abuse. The type of therapy can vary and should be dictated by Lori’s comfort. This is one of the most important things Lori can do right now as she has been retaining this pain for a long time and it may be showing itself as Dercum’s disease. The nervous system is likely now working in tangent with the inflamed fat, encouraging its growth.
******* I meet with a plastic surgeon on the 8th for the lumps I have on the back of my leg.
******* Cardiologist on the 1st for the tilt table test that should show if I have POTS and Orthostatic Hypertension
*******Calling for appointment with a neurologist to test for something called "thorasic outlet syndrom"
Okay, that is it for now. i will do my very best to update this as I can. It may not be easy to do right now, but I will try.