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Classification by Age of Onset
1. Congenital lymphedema—presents at birth
2. Lymphedema praecox—ages 1 to 35
77%–94% of cases
3. Lymphedema tarda—after age 35
developmental abnormality precipitated by some insult (trauma, illness, physical immobility)
Clues: Facial, conjunctival, and genital lymphedema is typically seen in association with the limbs involved
Emberger syndrome (Primary Lymphedema with
Myelodysplasia)- AD, mutations in GATA2, a transcription factor
Milroy Syndrome - AD, mutation in VEGFR3 (FLT4 gene) disrupts lymphangiogenesis. Females>males
Lymphedema-Distichiasis - AD mutation, transcription factor FoxC2 causing primary valve failure.
Lymphedema: A Comprehensive Review
Warren, Anne G. BA*; Brorson, Håkan MD, PhD‡; Borud, Loren J. MD†; Slavin, Sumner A. MD†
*Harvard Medical School, Boston, MA; †Harvard Medical School, Division of Plastic Surgery, Beth Israel Deaconess Medical Center, Boston, MA; and the ‡Department of Plastic and Reconstructive Surgery, Lund University, Malmö University Hospital, Malmö, Sweden. November 28, 2006. Reprints: Sumner A. Slavin, MD, 1101 Beacon St, Brookline, MA 02446. E-mail: sslavin@bidmc.harvard.edu.
Burkhart CN, Adigun C, Burton CS. Chapter 174. Cutaneous Changes in Peripheral Venous and Lymphatic Insufficiency. In: Wolff K, ed. Fitzpatrick's Dermatology in General Medicine. 8th ed. New York: McGraw-Hill; 2012. http://www.accessmedicine.com/content.aspx?aID=56081150. Accessed November 3, 2013.
HYPOTRICHOSIS–LYMPHEDEMA–TELANGIECTASIA - rare mutation of transcription factor gene SOX18
MEIGE SYNDROME
Definition: Excess fluid in tissues and cells due to lymphatic malformation or malfunction, most common in lower extremities
Clinical Presentation:
Risk factors:
• Trauma, infection, obesity, chronic venous stasis
#1 cause worldwide: filariasis infection with nematode Wusheria banrofti (100 million people)
#1 cause in the U.S.: malignancy or its therapy
Podoconiosis (nonfilarial elephantiasis) - bilateral edematous feet and legs 2/2 chronic inoculation of microparticles of silica in barefoot walkers
• International Society of Lymphology
1. Babu S, Nutman TB. Immunopathogenesis of lymphatic filarial disease. Semin
Immunopathol. 2012 Nov;34(6):847-61. doi: 10.1007/s00281-012-0346-4. Epub 2012
Oct 3. Review. PubMed PMID: 23053393; PubMed Central PMCID: PMC3498535.
2. Burkhart CN, Adigun C, Burton CS. Chapter 174. Cutaneous Changes in Peripheral Venous and Lymphatic Insufficiency. In: Wolff K, ed. Fitzpatrick's Dermatology in General Medicine. 8th ed. New York: McGraw-Hill; 2012. http://www.accessmedicine.com/content.aspx?aID=56081150. Accessed November 3, 2013.
3. Coenenberg J, Altmeyer P, Brockmeyer NH, Potthoff A. ["Who knows
Podoconiosis?" A dermatologic travel report]. Hautarzt. 2013 Aug;64(8):606-10.
doi: 10.1007/s00105-013-2613-2. German. PubMed PMID: 23907173.
4. Gokhale S, Gokhale S. Four Generations of Rare Familial Lymphedema (Milroy
Disease). Med Princ Pract. 2013 Jun 4. [Epub ahead of print] PubMed PMID:
23751338.
5. Kaarthigeyan K, Ramprakash M, Kalpana G. Distichiasis-lymphedema syndrome with
optic disc pit. Indian J Ophthalmol. 2011 Jan-Feb;59(1):71-2. PubMed PMID:
21157084; PubMed Central PMCID: PMC3032255.
6. Mansour S, Connell F, Steward C, Ostergaard P, Brice G, Smithson S, Lunt P, Jeffery S, Dokal I, Vulliamy T, Gibson B, Hodgson S, Cottrell S, Kiely L, Tinworth L, Kalidas K, Mufti G, Cornish J, Keenan R, Mortimer P, Murday V; Lymphoedema Research Consortium. Emberger syndrome-primary lymphedema with myelodysplasia: report of seven new cases. Am J Med Genet A. 2010. Sep;152A(9):2287-96. doi: 10.1002/ajmg.a.33445. PubMed PMID: 20803646.
7. Warren AG, Brorson H, Borud LJ, Slavin SA. Lymphedema: a comprehensive review. Ann Plast Surg. 2007 Oct;59(4):464-72. Review. PubMed PMID: 17901744.
Key component is compression
Pharmacologic Therapy:
Surgical Therapy - once failed conservative therapy, likely due to adipose tissue hypertrophy