Lymphedema Questions & Answers

1. Arellano J, Gonzalez R, Corredoira Y, Nuñez R. Diagnosis of elephantiasis nostras verrucosa as a clinical mani-festation of Kaposi's sarcoma. Medwave. 2020 Jan 20. 20 (1):e7767. [QxMD MEDLINE Link].

2. Karlsson K, Nilsson-Wikmar L, Brogårdh C, Johansson K. Palpation of Increased Skin and Subcutaneous Thickness, Tissue Dielectric Constant, and Water Displacement Method for Diagnosis of Early Mild Arm Lymphedema. Lymphat Res Biol. 2019 Oct 9. [QxMD MEDLINE Link].

3. Sudduth CL, Greene AK. Primary Lymphedema: Update on Genetic Basis and Management. Adv Wound Care (New Rochelle). 2022 Jul. 11 (7):374-381. [QxMD MEDLINE Link].

4. Pereira de Godoy JM, Azoubel LM, de Fatima Guerreiro de Godoy M. Intensive treatment of leg lymphedema. Indian J Dermatol. 2010 Apr-Jun. 55(2):144-7. [QxMD MEDLINE Link]. [Full Text].

5. Yimer M, Hailu T, Mulu W, Abera B. Epidemiology of elephantiasis with special emphasis on podoconiosis in Ethiopia: A literature review. J Vector Borne Dis. 2015 Jun. 52 (2):111-5. [QxMD MEDLINE Link].

6. Elgendy IY, Lo MC. Unilateral lower extremity swelling as a rare presentation of non-Hodgkin's lymphoma. BMJ Case Rep. 2014. [QxMD MEDLINE Link].

7. Fife C. Massive localized lymphedema, a disease unique to the morbidly obese: a case study. Ostomy Wound Manage. 2014 Jan. 60(1):30-5. [QxMD MEDLINE Link].

8. Chopra K, Tadisina KK, Brewer M, Holton LH, Banda AK, Singh DP. Massive localized lymphedema revisited: a quickly rising complication of the obesity epidemic. Ann Plast Surg. 2015 Jan. 74 (1):126-32. [QxMD MEDLINE Link].

9. Greene AK, Sudduth CL. Lower extremity lymphatic function predicted by body mass index: a lymphoscintigraphic study of obesity and lipedema. Int J Obes (Lond). 2021 Feb. 45 (2):369-373. [QxMD MEDLINE Link].

10. Prakash J, Kumar M, Singh V, Sankhwar S. Giant penile elephantiasis after circumcision: a devastating complication. BMJ Case Rep. Sep 16;2013. bcr2013200780. [QxMD MEDLINE Link].

11. Namnyak S, Adhami Z, Toms G, Jenks P. Pasteurella multocida septicaemia in Milroy's disease. J Infect. 1995 Sep. 31(2):175-6. [QxMD MEDLINE Link].

12. Lambert PC, Micali G, Schwartz RA. Lymphedema. Lebwohl M, ed. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. 5th ed. Elsevier; 2018. 470-71.

13. Connell F, Brice G, Jeffery S, Keeley V, Mortimer P, Mansour S. A new classification system for primary lymphatic dysplasias based on phenotype. Clin Genet. 2010 May. 77(5):438-52. [QxMD MEDLINE Link].

14. Connell F, Brice G, Mortimer P. Phenotypic characterization of primary lymphedema. Ann N Y Acad Sci. 2008. 1131:140-6. [QxMD MEDLINE Link].

15. Mellor RH, Hubert CE, Stanton AW, et al. Lymphatic dysfunction, not aplasia, underlies Milroy disease. Microcirculation. 2010 May. 17(4):281-96. [QxMD MEDLINE Link].

16. Sheng J, Zeng F, Li C, Liu J, Wang Q, Liu M. [Identification of VEGFR3 gene mutation in a Chinese family with autosomal dominant primary congenital lymphoedema.]. Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2010 Aug. 27(4):371-5. [QxMD MEDLINE Link].

17. Irrthum A, Karkkainen MJ, Devriendt K, Alitalo K, Vikkula M. Congenital hereditary lymphedema caused by a mutation that inactivates VEGFR3 tyrosine kinase. Am J Hum Genet. 2000 Aug. 67(2):295-301. [QxMD MEDLINE Link].

18. Karkkainen MJ, Ferrell RE, Lawrence EC, et al. Missense mutations interfere with VEGFR-3 signalling in primary lymphoedema. Nat Genet. 2000 Jun. 25(2):153-9. [QxMD MEDLINE Link].

19. Butler MG, Dagenais SL, Rockson SG, Glover TW. A novel VEGFR3 mutation causes Milroy disease. Am J Med Genet A. 2007 Jun 1. 143A(11):1212-7. [QxMD MEDLINE Link].

20. Connell FC, Ostergaard P, Carver C, Brice G, Williams N, Mansour S, et al. Analysis of the coding regions of VEGFR3 and VEGFC in Milroy disease and other primary lymphoedemas. Hum Genet. 2009 Jan. 124(6):625-31. [QxMD MEDLINE Link].

21. Ghalamkarpour A, Holnthoner W, Saharinen P, Boon LM, Mulliken JB, Alitalo K, et al. Recessive primary congenital lymphoedema caused by a VEGFR3 mutation. J Med Genet. 2009 Jun. 46(6):399-404. [QxMD MEDLINE Link].

22. Evans AL, Brice G, Sotirova V, Mortimer P, Beninson J, Burnand K, et al. Mapping of primary congenital lymphedema to the 5q35.3 region. Am J Hum Genet. 1999 Feb. 64(2):547-55. [QxMD MEDLINE Link]. [Full Text].

23. Berry FB, Tamimi Y, Carle MV, Lehmann OJ, Walter MA. The establishment of a predictive mutational model of the forkhead domain through the analyses of FOXC2 missense mutations identified in patients with hereditary lymphedema with distichiasis. Hum Mol Genet. 2005 Sep 15. 14(18):2619-27. [QxMD MEDLINE Link].

24. Salim A, Pike M, Turner R, Mortimer P. Lymphedema: an additional finding in the charge association. Pediatr Dermatol. 2003 Nov-Dec. 20(6):547-8. [QxMD MEDLINE Link].

25. Aghajan Y, Diaz J, Sladek E. Mysteriously puffy hand: puffy hand syndrome. BMJ Case Rep. 2018 Dec 22. 11 (1):[QxMD MEDLINE Link].

26. Hoerauf A, Pfarr K, Mand S, Debrah AY, Specht S. Filariasis in Africa-treatment challenges and prospects. Clin Microbiol Infect. 2011 Jul. 7:977-85. [QxMD MEDLINE Link].

27. Srivastava PK, Dhillon GP. Elimination of lymphatic filariasis in India--a successful endeavour. J Indian Med Assoc. 2008 Oct. 106(10):673-4, 676-7. [QxMD MEDLINE Link].

28. Debrah AY, Mand S, Marfo-Debrekyei Y, Batsa L, Albers A, Specht S, et al. Macrofilaricidal Activity in Wuchereria bancrofti after 2 Weeks Treatment with a Combination of Rifampicin plus Doxycycline. J Parasitol Res. 2011. 2011:201617. [QxMD MEDLINE Link].

29. Babu S, Nutman TB. Immunology of lymphatic filariasis. Parasite Immunol. 2014 Aug. 36(8):338-46. [QxMD MEDLINE Link].

30. Lee R, Saardi KM, Schwartz RA. Lymphedema-related angiogenic tumors and other malignancies. Clin Dermatol. 2014 Sep-Oct. 32 (5):616-20. [QxMD MEDLINE Link].

31. Piccolo V, Baroni A, Russo T, Schwartz RA. Ruocco's immunocompromised cutaneous district. Int J Dermatol. 2016 Feb. 55 (2):135-41. [QxMD MEDLINE Link].

32. Butler DF, Malouf PJ, Batz RC, Stetson CL. Acquired lymphedema of the hand due to herpes simplex virus type 2. Arch Dermatol. 1999 Sep. 135(9):1125-6. [QxMD MEDLINE Link].

33. Nikitenko LL, Shimosawa T, Henderson S, Mäkinen T, Shimosawa H, Qureshi U, et al. Adrenomedullin Haploinsufficiency Predisposes to Secondary Lymphedema. J Invest Dermatol. 2013 Jan 30. [QxMD MEDLINE Link].

34. Thielitz A, Bellutti M, Bonnekoh B, Franke I, Wiede A, Lotzing M, et al. Progressive lipo-lymphedema associated with increased activity of dermal fibroblasts in monoclonal gammopathy of undetermined significance: is there a causal relationship?. Lymphology. 2012 Sep. 45:124-9. [QxMD MEDLINE Link].

35. Boneti C, Badgwell B, Robertson Y, Korourian S, Adkins L, Klimberg V. Axillary reverse mapping (ARM): initial results of phase II trial in preventing lymphedema after lymphadenectomy. Minerva Ginecol. 2012 Oct. 64(5):421-30. [QxMD MEDLINE Link].

36. McPherson T, Persaud S, Singh S, et al. Interdigital lesions and frequency of acute dermatolymphangioadenitis in lymphoedema in a filariasis-endemic area. Br J Dermatol. 2006 May. 154(5):933-41. [QxMD MEDLINE Link].

37. Levinson KL, Feingold E, Ferrell RE, Glover TW, Traboulsi EI, Finegold DN. Age of onset in hereditary lymphedema. J Pediatr. 2003 Jun. 142(6):704-8. [QxMD MEDLINE Link].

38. Dürr HR, Pellengahr C, Nerlich A, Baur A, Maier M, Jansson V. Stewart-Treves syndrome as a rare complication of a hereditary lymphedema. Vasa. 2004 Feb. 33(1):42-5. [QxMD MEDLINE Link].

39. Chopra S, Ors F, Bergin D. MRI of angiosarcoma associated with chronic lymphoedema: Stewart Treves syndrome. Br J Radiol. 2007 Dec. 80(960):e310-3. [QxMD MEDLINE Link].

40. Aguiar Bujanda D, Camacho Galan R, Bastida Inarrea J, et al. Angiosarcoma of the abdominal wall after dermolipectomy in a morbidly obese man. A rare form of presentation of Stewart-Treves syndrome. Eur J Dermatol. 2006 May-Jun. 16(3):290-2. [QxMD MEDLINE Link].

41. Azurdia RM, Guerin DM, Verbov JL. Chronic lymphoedema and angiosarcoma. Clin Exp Dermatol. 1999 Jul. 24(4):270-2. [QxMD MEDLINE Link].

42. Komorowski AL, Wysocki WM, Mitus J. Angiosarcoma in a chronically lymphedematous leg: an unusual presentation of Stewart-Treves syndrome. South Med J. 2003 Aug. 96(8):807-8. [QxMD MEDLINE Link].

43. Shehan JM, Ahmed I. Angiosarcoma arising in a lymphedematous abdominal pannus with histologic features reminiscent of Kaposis sarcoma: report of a case and review of the literature. Int J Dermatol. 2006 May. 45(5):499-503. [QxMD MEDLINE Link].

44. Offori TW, Platt CC, Stephens M, Hopkinson GB. Angiosarcoma in congenital hereditary lymphoedema (Milroy's disease)--diagnostic beacons and a review of the literature. Clin Exp Dermatol. 1993 Mar. 18(2):174-7. [QxMD MEDLINE Link].

45. Sharma A, Schwartz RA. Stewart-Treves syndrome: Pathogenesis and management. J Am Acad Dermatol. 2012 Jun 7. [QxMD MEDLINE Link].

46. Atillasoy ES, Santoro A, Weinberg JM. Lymphoedema associated with Kaposi sarcoma. J Eur Acad Dermatol Venereol. 2001 Jul. 15(4):364-5. [QxMD MEDLINE Link].

47. Torres-Paoli D, Sanchez JL. Primary cutaneous B-cell lymphoma of the leg in a chronic lymphedematous extremity. Am J Dermatopathol. 2000 Jun. 22(3):257-60. [QxMD MEDLINE Link].

48. Beloncle F, Sayegh J, Eymerit-Morin C, Duveau A, Augusto JF. AA amyloidosis as a complication of primary lymphedema. Amyloid. 2014 Mar. 21(1):54-6. [QxMD MEDLINE Link].

49. Park G, Jeong HW, Lee J, Mun YC, Sung SH, Han SJ. Lymphedema Associated With Primary Amyloidosis: A Case Study. Ann Rehabil Med. 2017 Oct. 41 (5):887-891. [QxMD MEDLINE Link].

50. Maltese PE, Michelini S, Ricci M, Maitz S, Fiorentino A, Serrani R, et al. Increasing evidence of hereditary lymphedema caused by CELSR1 loss-of-function variants. Am J Med Genet A. 2019 Sep. 179 (9):1718-1724. [QxMD MEDLINE Link].

51. Karg E, Bereczki C, Kovacs J, et al. Primary lymphoedema associated with xanthomatosis, vaginal lymphorrhoea and intestinal lymphangiectasia. Br J Dermatol. 2002 Jan. 146(1):134-7. [QxMD MEDLINE Link].

52. Johnson SM, Kincannon JM, Horn TD. Lymphedema-distichiasis syndrome: report of a case and review. Arch Dermatol. 1999 Mar. 135(3):347-8. [QxMD MEDLINE Link].

53. Samlaska CP. Congenital lymphedema and distichiasis. Pediatr Dermatol. 2002 Mar-Apr. 19(2):139-41. [QxMD MEDLINE Link].

54. Lu S, Tran TA, Jones DM, et al. Localized lymphedema (elephantiasis): a case series and review of the literature. J Cutan Pathol. 2009 Jan. 36(1):1-20. [QxMD MEDLINE Link].

55. Ridner SH, Deng J, Fu MR, Radina E, Thiadens SR, Weiss J, et al. Symptom burden and infection occurrence among individuals with extremity lymphedema. Lymphology. 2012 Sep. 45:113-23. [QxMD MEDLINE Link].

56. Fukuda H, Saito R. Verruciform xanthoma in close association with isolated epidermolytic acanthoma: a case report and review of the Japanese dermatological literature. J Dermatol. 2005 Jun. 32(6):464-8. [QxMD MEDLINE Link].

57. Wu JJ, Wagner AM. Verruciform xanthoma in association with milroy disease and leaky capillary syndrome. Pediatr Dermatol. 2003 Jan-Feb. 20(1):44-7. [QxMD MEDLINE Link].

58. Wu YH, Hsiao PF, Lin YC. Verruciform xanthoma-like phenomenon in seborrheic keratosis. J Cutan Pathol. 2006 May. 33(5):373-7. [QxMD MEDLINE Link].

59. Chavda LK, Vaidya RA, Vaidya AD. Yellow nail syndrome: missed diagnosis of a rare syndrome. J Assoc Physicians India. 2011 Apr. 59:258-60. [QxMD MEDLINE Link].

60. Ghalamkarpour A, Morlot S, Raas-Rothschild A, Utkus A, Mulliken JB, Boon LM, et al. Hereditary lymphedema type I associated with VEGFR3 mutation: the first de novo case and atypical presentations. Clin Genet. 2006 Oct. 70(4):330-5. [QxMD MEDLINE Link].

61. Vignes S. [Lipedema: a misdiagnosed entity]. J Mal Vasc. 2012 Jul. 37(4):213-8. [QxMD MEDLINE Link].

62. Bollinger A, Amann-Vesti BR. Fluorescence microlymphography: diagnostic potential in lymphedema and basis for the measurement of lymphatic pressure and flow velocity. Lymphology. 2007 Jun. 40(2):52-62. [QxMD MEDLINE Link].

63. Kim YB, Hwang JH, Kim TW, Chang HJ, Lee SG. Would complex decongestive therapy reveal long term effect and lymphoscintigraphy predict the outcome of lower-limb lymphedema related to gynecologic cancer treatment?. Gynecol Oncol. 2012 Sep 26. [QxMD MEDLINE Link].

64. Schmitz KH, Ahmed RL, Troxel A, Cheville A, Smith R, Lewis-Grant L, et al. Weight lifting in women with breast-cancer-related lymphedema. N Engl J Med. 2009 Aug 13. 361(7):664-73. [QxMD MEDLINE Link].

65. Mayrovitz HN. The standard of care for lymphedema: current concepts and physiological considerations. Lymphat Res Biol. 2009. 7(2):101-8. [QxMD MEDLINE Link].

66. Pereira De Godoy JM, Amador Franco Brigidio P, Buzato E, Fátima Guerreiro De Godoy M. Intensive outpatient treatment of elephantiasis. Int Angiol. 2012 Oct. 31(5):494-9. [QxMD MEDLINE Link].

67. Chang AY, Mungai M, Coates SJ, Chao T, Odhiambo HP, Were PM, et al. Implementing a Locally Made Low-Cost Intervention for Wound and Lymphedema Care in Western Kenya. Dermatol Clin. 2021 Jan. 39 (1):91-100. [QxMD MEDLINE Link].

68. Lerner R. What's New in Lymphedema Therapy in America?. Int J Angiol. 1998 May. 7(3):191-6. [QxMD MEDLINE Link].

69. Beninson J, Redmond MJ. Mossy leg--an unusual therapeutic success. Angiology. 1986 Sep. 37(9):642-6. [QxMD MEDLINE Link].

70. King B. Toe bandaging to prevent and manage oedema. Nurs Times. 2007 Oct 23-29. 103(43):44, 47. [QxMD MEDLINE Link].

71. Gurdal SO, Kostanoglu A, Cavdar I, Ozbas A, Cabioglu N, Ozcinar B, et al. Comparison of intermittent pneumatic compression with manual lymphatic drainage for treatment of breast cancer-related lymphedema. Lymphat Res Biol. 2012 Sep. 10(3):129-35. [QxMD MEDLINE Link].

72. Wang X, Ding Y, Cai HY, You J, Fan FQ, Cai ZF, et al. Effectiveness of modified complex decongestive physiotherapy for preventing lower extremity lymphedema after radical surgery for cervical cancer: a randomized controlled trial. Int J Gynecol Cancer. 2020 Feb 26. [QxMD MEDLINE Link].

73. Olszewski WL, Jamal S, Manokaran G, Tripathi FM, Zaleska M, Stelmach E. The effectiveness of long-acting penicillin (penidur) in preventing recurrences of dermatolymphangioadenitis(DLA) and controlling skin, deep tissues, and lymph bacterial flora in patients with "filarial" lymphedema. Lymphology. 2005 Jun. 38(2):66-80. [QxMD MEDLINE Link].

74. Debrah AY, Mand S, Marfo-Debrekyei Y, et al. Macrofilaricidal effect of 4 weeks of treatment with doxycycline on Wuchereria bancrofti. Trop Med Int Health. 2007 Dec. 12(12):1433-41. [QxMD MEDLINE Link].

75. Yongyuth P, Koyadun S, Jaturabundit N, Sampuch A, Bhumiratana A. Efficacy of a single-dose treatment with 300 mg diethylcarbamazine and a combination of 400 mg albendazole in reduction of Wuchereria bancrofti antigenemia and concomitant geohelminths in Myanmar migrants in Southern Thailand. J Med Assoc Thai. 2006 Aug. 89(8):1237-48. [QxMD MEDLINE Link].

76. Mand S, Debrah AY, Klarmann U, Batsa L, Marfo-Debrekyei Y, Kwarteng A, et al. Doxycycline improves filarial lymphedema independent of active filarial infection: a randomized controlled trial. Clin Infect Dis. 2012 Sep. 55 (5):621-30. [QxMD MEDLINE Link].

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Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

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William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

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Noah S Scheinfeld, JD, MD, FAAD † Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

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David F Butler, MD Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

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Amy L Friedman, MD Professor of Surgery, Director of Transplantation, State University

of New York Upstate Medical University College of Medicine, Syracuse

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