Lipedema, Lymphedema, Lipolymphedema

Updated 3/2017-- photo and all links removed as many no longer active. and it was easier than checking each one.

There is an interesting article (full reference below)on lipedema.  If you have an interest in the subject, then read the full article.  The terms are so similar that I found myself having to be careful (often deliberately speaking the terms out loud) when I read the article.  Even though the topic is “no laughing matter”  you could easily make a tongue-tying word game out of the words:  lipedema, lymphedema, lipolymphedema.

Lipedema is a condition in which there is a pathological deposition of fatty tissue, usually below the waist, leading to progressive leg enlargement.  The diagnosis of lipedema is a clinical one, often challenging in patients who are overweight or obese, based on the examination and history. 

Distinguishing obesity from lipedema can be accomplished by noting (a) whether the fat deposits in the arms and legs spare the feet and hands, (b) whether the adiposity is predominantly in the extremities or it exists in the extremities and the trunk, and (c) by whether diet and/or physical activities reduce truncal adiposity but not extremity adiposity.   Easy bruising is often noted. Pain upon pressure is universal in patients with lipedema and is commonly described an "aching dysesthesia.

Lymphedema is a condition in which edema leads to inflammation and fibrosis as a result of reduced lymphatic return.

Lipolymphedema is the condition in which lipedema acquires a lymphedema component and is thus differentiated from pure lipedema by the presence of Stemmer's sign.

The Stemmer's sign is the inability to pinch a fold of dorsal skin at the base of the toes and will be positive in patients who have developed secondary lymphedema.   In patients with pure lipedema, the Stemmer's sign is negative. In lipedema patients, edema is minimal and relieved by elevation. In lymphedema patients, the edema is persistent.

Photo credit




REFERENCE
Lipedema: A Frequently Misdiagnosed and Misunderstood Fatty Deposition Syndrome; Advances in Skin & Wound Care, February 2010 - Volume 23 - Issue 2 - pp 81-92; Fife, Caroline E. MD; Maus, Erik A. MD; Carter, Marissa J. PhD, MA

A Guide to Lymphedema; Medscape article, 01/23/2008; Kristiana D Gordon; Peter S Mortimer

Lipedema and Lymphedema; LymphNotes.com
Lipedema; Lymphedema Therapy Website

Microcystic Lymphatic Malformations of the Tongue – an Article Review

Updated 3/2017-- photos and all links (except to my own posts) removed as many no longer active. and it was easier than checking each one.

I stumbled across this article while previewing JAMA & Archives CME articles (full reference below). The article gives an overview of lymphatic malformations, noting that both sexes are equally affected, and there is no predilection for any race.

Lymphatic malformations are vascular malformations with an unknown cause. They are estimated to make up 6% of all benign soft-tissue tumors in children. While they may be rare, 50% of all lymphatic malformations are already obvious at the time of birth. Most (90%) are diagnosed by the end of the second year of life owing to clinical symptoms.

About 60% of all lymphatic malformations are found in the head and neck region. Regarding the mouth, the tongue is most commonly affected.

When the malformations occur in the tongue, the symptoms may include hemorrhage, excessive salivation, speech disturbances, difficulties chewing and swallowing, airway obstruction, and orthodontic abnormalities such as mandibular prognathism and malocclusion. Functional impairment and cosmetic deformity significantly affect the quality of life of patients with lymphatic malformations of the tongue.

Along with the overview, the authors present the review of their patients between January 1, 1998, through December 31, 2008, with respect to age and sex distribution, symptoms, clinical presentation, management, treatment outcome, and follow-up.

Twenty patients (13 male and 7 female) with microcystic lymphatic malformations of the tongue were included in the evaluation. Their ages at initial presentation ranged from newborn to 20 years (mean age, 7.4 years). Thirteen of them had been treated at another hospital before the initial presentation at our department. The treatment methods included surgical reduction, laser therapy, corticosteroid therapy, and OK-432 (Picibanil; Chugai Pharmaceutical Co, Ltd, Tokyo, Japan) injections.

The authors present the classification of lymphatic malformations (photo credit)

• Isolated superficial microcystic lymphatic malformations of the tongue (stage I)
• Isolated lymphatic malformations of the tongue with muscle involvement (stage II; stage IIA, involving a part of the tongue; stage IIB, involving the entire tongue)
• Microcystic lymphatic malformations of the tongue and the floor of mouth (stage III)
• Extensive microcystic lymphatic malformations involving the tongue, floor of mouth, and further cervical structures (stage IV)

• 
  

The article discusses treatment options:

In the present series of patients with microcystic lymphatic malformations of the tongue, it was possible to perform complete excision with a CO₂ laser in all patients with stage I disease and in 3 patients with stage IIA disease. …….. The advantages of the CO₂ laser compared with conventional surgery include less postoperative edema, tissue trauma, and blood loss……... For stages I and IIA microcystic lymphatic malformations of the tongue, CO₂ laser surgery seems to be an excellent curative treatment option. In stages IIB, III, and IV disease, CO₂ laser surgery seems to be useful as a part of a combined or staged approach.

Other treatment modalities discussed include radiofrequency ablation, sclerotherapy (specifically OK-432 injections), and other surgical options, including wedge resection, bilateral marginal resection, U-shaped resection, and Jian or Dingman glossectomy.

Treatment of infected cysts before surgery:

The combination of antibiotics and short-duration systemic corticosteroids usually leads to a reduction of symptoms and a decrease of swelling and inflammation as described in patient 2.

I think the article is well written and well worth reading.

REFERENCE

Microcystic Lymphatic Malformations of the Tongue: Diagnosis, Classification, and Treatment; Arch Otolaryngol Head Neck Surg. 2009;135(10):976-983; Susanne Wiegand, MD; Behfar Eivazi, MD; Annette P. Zimmermann, MD; Andreas Neff, MD, PhD; Peter J. Barth, MD, PhD; Andreas M. Sesterhenn, MD, PhD; Robert Mandic, MD, PhD; Jochen A. Werner, MD, PhD

My post: Vascular Birthmarks (July 15, 2007)