Sections

Lymphedema

Medication

Medication Summary

As previously mentioned, few pharmacologic therapies have been found to be effective in the treatment of lymphedema. Case reports have suggested, however, that chronic lymphedematous changes (eg, elephantiasis nostra verrucosa [ENV]) can be treated with oral and topical retinoids. These therapies are thought to help normalize keratinization and decrease inflammatory and fibrotic changes.^{[79, 80]}

Topical emollients and keratolytics, such as ammonium lactate, urea, and salicylic acid, have been recommended to improve secondary epidermal changes.

Patients experiencing recurrent lymphangitis or cellulitis may require long-term, prophylactic treatment with antimicrobial agents such as penicillin, cephalexin, or erythromycin.^{[73, 74]}

Filariasis has been treated with the anthelmintic agents diethylcarbamazine and albendazole.^[75]

Future therapeutic interventions will hopefully facilitate lymphatic regeneration and function, with both preclinical studies and clinical trials currently ongoing.^[93]

Class Summary

These agents are thought to help normalize keratinization and decrease inflammatory and fibrotic changes.

Acitretin (Soriatane)

View full drug information

Acitretin, a metabolite of etretinate, is related to retinoic acid and retinol (vitamin A). Its mechanism of action is unknown; however, the drug is thought to exert its therapeutic effect by modulating keratinocyte differentiation, keratinocyte hyperproliferation, and tissue infiltration by inflammatory cells.

Tazarotene, topical (Tazorac)

View full drug information

Topical tazarotene (topical gel 0.1%) is a retinoid prodrug with an active metabolite that modulates the differentiation and proliferation of epithelial tissue. It may also have anti-inflammatory and immunomodulatory properties. Ensure that the skin is dry before the gel is applied.

Class Summary

Filaria can cause lymphedema by obstruction.

Albendazole (Albenza)

View full drug information

Albendazole is a benzimidazole carbamate drug that inhibits tubulin polymerization, resulting in degeneration of cytoplasmic microtubules. It is converted in the liver to its primary metabolite, albendazole sulfoxide. Less than 1% of the primary metabolite is excreted in urine.

The plasma level is noted to rise significantly (as much as 5-fold) when albendazole is ingested after a high-fat meal. Experience with patients under age 6 years is limited.

To avoid an inflammatory response in the central nervous system (CNS), patients must also be started on anticonvulsants and high-dose glucocorticoids.

Class Summary

Topical emollients and keratolytics may improve secondary epidermal changes.

Ammonium lactate lotion (AmLactin, AmLactin XL, Lac-Hydrin, Gen-Hydrolac)

View full drug information

This agent is believed to act as a humectant when applied to skin. This may influence hydration of the stratum corneum. In addition, when applied to the skin, ammonium lactate lotion may decrease corneocyte cohesion. The mechanism by which this is accomplished is not yet known.

Found in a variety of topical emollient lotions, ammonium lactate is used to decrease scaling and pruritus. Ammonium lactate 5% lotion is available over the counter; lactic acid 12% cream and lotion are available by prescription.

Urea, topical (Carmol, Keralac, Ureacin)

View full drug information

Topical urea promotes hydration and removal of excess keratin in conditions of hyperkeratosis.

Class Summary

Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of this clinical setting.

Cefazolin

View full drug information

Cefazolin is a first-generation, semisynthetic cephalosporin that arrests bacterial cell wall synthesis, inhibiting bacterial growth. It is primarily active against skin flora, including Staphylococcus aureus. This agent is typically used alone for skin and skin-structure coverage. The intravenous and intramuscular dosing regimens are similar.

Clindamycin (Cleocin)

View full drug information

Clindamycin is a lincosamide used for the treatment of serious skin and soft tissue staphylococcal infections. It is also effective against aerobic and anaerobic streptococci (but not enterococci). Clindamycin inhibits bacterial growth, possibly by blocking dissociation of peptidyl transfer ribonucleic acid (t-RNA) from ribosomes, causing RNA-dependent protein synthesis to arrest.

Penicillin G benzathine (Bicillin L-A)

View full drug information

This agent interferes with the synthesis of cell wall mucopeptides during active multiplication, resulting in bactericidal activity. It is used to treat syphilis and for prophylaxis against recurrent streptococcal infections.

Questions

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].
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].
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].
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].
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].
Elgendy IY, Lo MC. Unilateral lower extremity swelling as a rare presentation of non-Hodgkin's lymphoma. BMJ Case Rep. 2014. [QxMD MEDLINE Link].
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].
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].
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].
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].
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].
Lambert PC, Micali G, Schwartz RA. Lymphedema. Lebwohl M, ed. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. 5th ed. Elsevier; 2018. 470-71.
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].
Connell F, Brice G, Mortimer P. Phenotypic characterization of primary lymphedema. Ann N Y Acad Sci. 2008. 1131:140-6. [QxMD MEDLINE Link].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
Aghajan Y, Diaz J, Sladek E. Mysteriously puffy hand: puffy hand syndrome. BMJ Case Rep. 2018 Dec 22. 11 (1):[QxMD MEDLINE Link].
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].
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].
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].
Babu S, Nutman TB. Immunology of lymphatic filariasis. Parasite Immunol. 2014 Aug. 36(8):338-46. [QxMD MEDLINE Link].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
Azurdia RM, Guerin DM, Verbov JL. Chronic lymphoedema and angiosarcoma. Clin Exp Dermatol. 1999 Jul. 24(4):270-2. [QxMD MEDLINE Link].
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].
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].
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].
Sharma A, Schwartz RA. Stewart-Treves syndrome: Pathogenesis and management. J Am Acad Dermatol. 2012 Jun 7. [QxMD MEDLINE Link].
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].
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].
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].
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].
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].
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].
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].
Samlaska CP. Congenital lymphedema and distichiasis. Pediatr Dermatol. 2002 Mar-Apr. 19(2):139-41. [QxMD MEDLINE Link].
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].
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].
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].
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].
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].
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].
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].
Vignes S. [Lipedema: a misdiagnosed entity]. J Mal Vasc. 2012 Jul. 37(4):213-8. [QxMD MEDLINE Link].
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].
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].
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].
Mayrovitz HN. The standard of care for lymphedema: current concepts and physiological considerations. Lymphat Res Biol. 2009. 7(2):101-8. [QxMD MEDLINE Link].
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].
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].
Lerner R. What's New in Lymphedema Therapy in America?. Int J Angiol. 1998 May. 7(3):191-6. [QxMD MEDLINE Link].
Beninson J, Redmond MJ. Mossy leg--an unusual therapeutic success. Angiology. 1986 Sep. 37(9):642-6. [QxMD MEDLINE Link].
King B. Toe bandaging to prevent and manage oedema. Nurs Times. 2007 Oct 23-29. 103(43):44, 47. [QxMD MEDLINE Link].
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].
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].
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].
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].
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].
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].
Al-Kubati AS, Al-Samie AR, Al-Kubati S, Ramzy RMR. The story of Lymphatic Filariasis elimination as a public health problem from Yemen. Acta Trop. 2020 Dec. 212:105676. [QxMD MEDLINE Link].
Milton P, Hamley JID, Walker M, Basáñez MG. Moxidectin: an oral treatment for human onchocerciasis. Expert Rev Anti Infect Ther. 2020 Nov. 18 (11):1067-1081. [QxMD MEDLINE Link].
Feind-Koopmans A, van de Kerkhof PC. Successful treatment of papillomatosis cutis lymphostatica with acitretin. Acta Derm Venereol. 1995 Sep. 75(5):411. [QxMD MEDLINE Link].
Boyd J, Sloan S, Meffert J. Elephantiasis nostrum verrucosa of the abdomen: clinical results with tazarotene. J Drugs Dermatol. 2004 Jul-Aug. 3(4):446-8. [QxMD MEDLINE Link].
Warren AG, Brorson H, Borud LJ, Slavin SA. Lymphedema: a comprehensive review. Ann Plast Surg. 2007 Oct. 59(4):464-72. [QxMD MEDLINE Link].
Drobot A, Bez M, Abu Shakra I, Merei F, Khatib K, Bickel A, et al. Microsurgery for management of primary and secondary lymphedema. J Vasc Surg Venous Lymphat Disord. 2021 Jan. 9 (1):226-233.e1. [QxMD MEDLINE Link].
Onoda S, Nishimon K. The utility of surgical and conservative combination therapy for advanced stage lymphedema. J Vasc Surg Venous Lymphat Disord. 2021 Jan. 9 (1):234-241. [QxMD MEDLINE Link].
Salgado CJ, Sassu P, Gharb BB, Spanio di Spilimbergo S, Mardini S, Chen HC. Radical reduction of upper extremity lymphedema with preservation of perforators. Ann Plast Surg. 2009 Sep. 63(3):302-6. [QxMD MEDLINE Link].
Narushima M, Mihara M, Yamamoto Y, Iida T, Koshima I, Mundinger GS. The intravascular stenting method for treatment of extremity lymphedema with multiconfiguration lymphaticovenous anastomoses. Plast Reconstr Surg. 2010 Mar. 125(3):935-43. [QxMD MEDLINE Link].
van der Walt JC, Perks TJ, Zeeman BJ, Bruce-Chwatt AJ, Graewe FR. Modified Charles procedure using negative pressure dressings for primary lymphedema: a functional assessment. Ann Plast Surg. 2009 Jun. 62(6):669-75. [QxMD MEDLINE Link].
Borst GM, Goettler CE, Kachare SD, Sherman RA. Maggot Therapy for Elephantiasis Nostras Verrucosa Reveals New Applications and New Complications: A Case Report. Int J Low Extrem Wounds. 2014 May 25. 13(2):135-139. [QxMD MEDLINE Link].
Devoogdt N, Christiaens MR, Geraerts I, Truijen S, Smeets A, Leunen K, et al. Effect of manual lymph drainage in addition to guidelines and exercise therapy on arm lymphoedema related to breast cancer: randomised controlled trial. BMJ. 2011 Sep 1. 343:d5326. [QxMD MEDLINE Link]. [Full Text].
Torres Lacomba M, Yuste Sánchez MJ, Zapico Goñi A, Prieto Merino D, Mayoral del Moral O, Cerezo Téllez E, et al. Effectiveness of early physiotherapy to prevent lymphoedema after surgery for breast cancer: randomised, single blinded, clinical trial. BMJ. 2010 Jan 12. 340:b5396. [QxMD MEDLINE Link]. [Full Text].
Armer JM, Stewart BR, Shook RP. 30-Month Post-Breast Cancer Treatment Lymphedema. J Lymphoedema. 2009 Apr 1. 4 (1):14-18. [QxMD MEDLINE Link]. [Full Text].
Lowry F. Study finds genetic link to lymphedema. Medscape Medical News. Available at http://www.medscape.com/viewarticle/802874. April 22, 2013; Accessed: April 29, 2013.
Miaskowski C, Dodd M, Paul SM, West C, Hamolsky D, Abrams G, et al. Lymphatic and Angiogenic Candidate Genes Predict the Development of Secondary Lymphedema following Breast Cancer Surgery. PLoS One. 2013. 8(4):e60164. [QxMD MEDLINE Link]. [Full Text].
Brown S, Dayan JH, Coriddi M, et al. Pharmacological Treatment of Secondary Lymphedema. Front Pharmacol. 2022. 13:828513. [QxMD MEDLINE Link]. [Full Text].

Media Gallery

Lymphedema in a patient with hypertension, diabetes, and impaired cardiac function.
Morbidly obese patient with lymphedema.
The body quadrants of superficial lymph drainage.
(1) Normal lymphatic flow in (a) deep systems and (b) superficial systems. Note the small collateral vessels interconnecting the 2 systems. (2) Lymphedema develops from obstruction, dilation of valves, valvular insufficiency, and subsequent reversal of lymphatic flow.
Lymphatic system, anterior view.

of 5

Author

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

Disclosure: Nothing to disclose.

Chief Editor

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.

Additional Contributors

Rajendra Kapila, MD, MBBS † Professor, Department of Medicine, Rutgers New Jersey Medical School

Rajendra Kapila, MD, MBBS is a member of the following medical societies: American College of Physicians, American Medical Association, Infectious Diseases Society of America, Infectious Diseases Society of New Jersey

Disclosure: Nothing to disclose.

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

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Kathleen M Rossy, MD Princeton Dermatology Associates

Disclosure: Nothing to disclose.

Acknowledgements

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

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD Herman Beerman Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System

Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology and American Society of Dermatopathology

Disclosure: Lippincott Williams Wilkins Royalty Textbook editor; DLA Piper Consulting fee Consulting

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

of New York Upstate Medical University College of Medicine, Syracuse

Amy L Friedman, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, American Medical Women's Association, American Society for Artificial Internal Organs, American Society of Transplant Surgeons, American Society of Transplantation, Association for Academic Surgery, Association of Women Surgeons, International College of Surgeons, International Liver Transplantation Society, NewYork Academy of Sciences, Pennsylvania Medical Society, Philadelphia County Medical Society, Society of Critical Care Medicine, and Transplantation Society

Disclosure: Nothing to disclose.

John Geibel, MD, DSc, MA Vice Chair and Professor, Department of Surgery, Section of Gastrointestinal Medicine, and Department of Cellular and Molecular Physiology, Yale University School of Medicine; Director, Surgical Research, Department of Surgery, Yale-New Haven Hospital

John Geibel, MD, DSc, MA is a member of the following medical societies: American Gastroenterological Association, American Physiological Society, American Society of Nephrology, Association for Academic Surgery, International Society of Nephrology, New York Academy of Sciences, and Society for Surgery of the Alimentary Tract

Disclosure: AMGEN Royalty Consulting; ARdelyx Ownership interest Board membership

Raphael J Kiel, MD Associate Professor of Medicine, Wayne State University School of Medicine; Associate Professor of Medicine, Oakland University William Beaumont School of Medicine; Consulting Staff, Infectious Diseases Division, William Beaumont Hospital; Consulting Staff, Infectious Diseases Division Providence Hospital

Raphael J Kiel, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine and American Geriatrics Society

Disclosure: Nothing to disclose.

Zab Mosenifar, MD Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Professor and Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society

Disclosure: Nothing to disclose.

Don R Revis Jr, MD Consulting Staff, Department of Surgery, Division of Plastic and Reconstructive Surgery, University of Florida College of Medicine

Don R Revis Jr, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, American Society for Aesthetic Plastic Surgery, and American Society of Plastic Surgeons

Disclosure: Nothing to disclose.

Sat Sharma, MD, FRCPC Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St Boniface General Hospital

Sat Sharma, MD, FRCPC is a member of the following medical societies: American Academy of Sleep Medicine, American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Thoracic Society, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Royal Society of Medicine, Society of Critical Care Medicine, and World Medical Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Lymphedema Medication

Medication Summary

Retinoid-like Agents

Class Summary

Acitretin (Soriatane)

Acitretin (Soriatane)

Tazarotene, topical (Tazorac)

Tazarotene, topical (Tazorac)

Anthelmintics

Class Summary

Albendazole (Albenza)

Albendazole (Albenza)

Topical Skin Products

Class Summary

Ammonium lactate lotion (AmLactin, AmLactin XL, Lac-Hydrin, Gen-Hydrolac)

Ammonium lactate lotion (AmLactin, AmLactin XL, Lac-Hydrin, Gen-Hydrolac)

Urea, topical (Carmol, Keralac, Ureacin)

Urea, topical (Carmol, Keralac, Ureacin)

Antibiotics

Class Summary

Cefazolin

Cefazolin

Clindamycin (Cleocin)

Clindamycin (Cleocin)

Penicillin G benzathine (Bicillin L-A)

Penicillin G benzathine (Bicillin L-A)

Lymphedema Medication

Medication Summary

Retinoid-like Agents

Class Summary

Acitretin (Soriatane)

Tazarotene, topical (Tazorac)

Anthelmintics

Class Summary

Albendazole (Albenza)

Topical Skin Products

Class Summary

Ammonium lactate lotion (AmLactin, AmLactin XL, Lac-Hydrin, Gen-Hydrolac)

Urea, topical (Carmol, Keralac, Ureacin)

Antibiotics

Class Summary

Cefazolin

Clindamycin (Cleocin)

Penicillin G benzathine (Bicillin L-A)

References

Tables

Contributor Information and Disclosures

What would you like to print?