Clinical presentations of lymphatic dysfunction

    Chronic, venous and drug‐induced oedema

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    • 2  Willenberg T, Schumacher A, Amann‐Vesti B, et al. Impact of obesity on venous hemodynamics of the lower limbs. J Vasc Surg 2010;52(3):664–8. Cross Ref link Pubmed link
    • 3  Dreifuss SE, Manders EK. Massive scrotal edema: an unusual manifestation of obstructive sleep apnea and obesity‐hypoventilation syndrome. Case Rep Med 2013;2013:685–716.
    • 4  Moffatt CJ, Franks PJ, Doherty DC, et al. Lymphoedema: an underestimated health problem. Q J Med 2003;96(10):731–8. Cross Ref link
    • 5  Levick JR, Michel CC. Microvascular fluid exchange and the revised Starling principle. Cardiovasc Res 2010;87(2):198–210. Cross Ref link Pubmed link
    • 6  Proby CM, Gane JN, Joseph AE, et al. Investigation of the swollen limb with isotope lymphography. Br J Dermatol 1990;123:29–38. Cross Ref link Pubmed link

    Chronically swollen leg

    • 7  Strømgaard S, Rasmussen SW, Schmidt TA. Brief hospitalizations of elderly patients: a retrospective, observational study. Scand J Trauma Resusc Emerg Med 2014;22:17. Cross Ref link Pubmed link
    • 8  Levick JR. An Introduction to Cardiovascular Physiology, 5th edn. London: Arnold, 2010.
    • 9  Lymphoedema Framework. Best Practice for the Management of Lymphoedema. International Consensus. London: Medical Education, 2006. http://www.lympho.org/mod_turbolead/upload/file/Lympho/Best_practice_20_July.pdf (last accessed August 2015).

    Phleboedema and mixed lymphovenous disease

    • 10  Kaipainen A, Korhonen J, Mustonen T, et al. Expression of the fms‐like tyrosine kinase FLT4 gene becomes restricted to lymphatic endothelium during development. Proc Natl Acad Sci USA 1995;92:3566–70. Cross Ref link Pubmed link
    • 11  Levick JR, Michel CC. Microvascular fluid exchange and the revised Starling principle. Cardiovasc Res 2010;87(2):198–210. Cross Ref link Pubmed link
    • 12  Rabe E, Guex JJ, Puskas A, Scuderi A, Fernandez Quesada F; VCP Coordinators. Epidemiology of chronic venous disorders in geographically diverse populations: results from the Vein Consult Program. Int Angiol 2012;31(2):105–15. Pubmed link
    • 13  Telinius N, Mohanakumar S, Majgaard J, et al. Human lymphatic vessel contractile activity is inhibited in vitro but not in vivo by the calcium channel blocker nifedipine. J Physiol 2014;592(2):4697–714. Cross Ref link Pubmed link
    • 14  Levick JR. An Introduction to Cardiovascular Physiology, 5th edn. London: Arnold, 2010.
    • 15  Mellor RH, Brice G, Stanton AW, et al; Lymphoedema Research Consortium. Mutations in FOXC2 are strongly associated with primary valve failure in veins of the lower limb. Circulation 2007;115(14):1912–20 . Cross Ref link Pubmed link
    • 16  Dean SM, Zirwas MJ, Horst AV. Elephantiasis nostras verrucosa: an institutional analysis of 21 cases. J Am Acad Dermatol 2011; 64(6):1104–10. Cross Ref link Pubmed link
    • 17  Lymphoedema Framework. Best Practice for the Management of Lymphoedema. International Consensus. London: Medical Education, 2006. http://www.lympho.org/mod_turbolead/upload/file/Lympho/Best_practice_20_July.pdf (last accessed August 2015).
    • 18  Zaleska M, Olszewski WL, Durlik M. The effectiveness of intermittent pneumatic compression in long‐term therapy of lymphedema of lower limbs. Lymphat Res Biol 2014;12(2):103–9. Cross Ref link Pubmed link
    • 19  Brittenden J, Cotton SC, Elders A, et al. A randomized trial comparing treatments for varicose veins. N Engl J Med 2014;371(13):1218–27. Cross Ref link Pubmed link

    Lipodermatosclerosis (chronic cellulitis)

    • 20  Strømgaard S, Rasmussen SW, Schmidt TA. Brief hospitalizations of elderly patients: a retrospective, observational study. Scand J Trauma Resusc Emerg Med 2014;22:17. Cross Ref link Pubmed link
    • 21  Herouy Y, May AE, Pornschlegel G, et al. Lipodermatosclerosis is characterized by elevated expression and activation of matrix metalloproteinases: implications for venous ulcer formation. J Invest Dermatol 1998;111(5):822–7. Cross Ref link Pubmed link
    • 22  Caggiati A, Rosi C, Casini A, et al. Skin iron deposition characterises lipodermatosclerosis and leg ulcer. Eur J Vasc Endovasc Surg 2010;40(6):777–82. Cross Ref link Pubmed link
    • 23  Hirschmann JV, Raugi GJ. Lower limb cellulitis and its mimics: part II. Conditions that simulate lower limb cellulitis. J Am Acad Dermatol 2012;67(2):177. Cross Ref link Pubmed link
    • 24  Lymphoedema Framework. Best Practice for the Management of Lymphoedema. International Consensus. London: Medical Education, 2006. http://www.lympho.org/mod_turbolead/upload/file/Lympho/Best_practice_20_July.pdf (last accessed August 2015).
    • 25  National Institute for Health and Care Excellence (NICE). Varicose Veins in the Legs: the Diagnosis and Management of Varicose Veins. NICE Guidelines No. CG168. NICE, 2013. http://www.nice.org.uk/guidance/CG168 (last accessed August 2015).

    Recurrent cellulitis (erysipelas)

    • 26  Thomas KS, Crook AM, Nunn AJ, et al; UK Dermatology Clinical Trials Network's PATCH I Trial Team. Penicillin to prevent recurrent leg cellulitis. N Engl J Med 2013;368(18):1695–703. Cross Ref link Pubmed link
    • 27  Jeng A, Beheshti M, Li J, Nathan R. The role of beta‐hemolytic streptococci in causing diffuse, nonculturable cellulitis: a prospective investigation. Medicine (Baltimore) 2010;89:217–26. Cross Ref link Pubmed link
    • 28  Damstra RJ, van Steensel MA, Boomsma JH, et al. Erysipelas as a sign of sub‐clinical primary lymphoedema: a prospective quantitative scintigraphic study of 40 patients with unilateral erysipelas of the leg. Br J Dermatol 2008;158:1210–15. Cross Ref link Pubmed link
    • 29  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;154(5):933–41. Cross Ref link Pubmed link
    • 30  Dupuy A, Benchikhi H, Roujeau JC, et al. Risk factors for erysipelas of the leg (cellulitis): case control study. BMJ 1999;318:591–4. Cross Ref link
    • 31  Cox NH. Oedema as a risk factor for multiple episodes of cellulitis/erysipelas of the lower leg: a series with community follow‐up. Br J Dermatol 2006;155:947–50. Cross Ref link Pubmed link
    • 32  Moffatt CJ, Franks PJ, Doherty DC, et al. Lymphoedema: an underestimated health problem. Q J Med 2003;96:731–8. Cross Ref link
    • 33  Mortimer PS, Rockson SG. New developments in clinical aspects of lymphatic disease. J Clin Invest 2014;124(3):915–21. Cross Ref link Pubmed link
    • 34  Chira S, Miller LG. Staphylococcus aureus is the most common identified cause of cellulitis: a systematic review. Epidemiol Infect 2010;138(3):313–17. Cross Ref link Pubmed link
    • 35  Chlebicki MP, Oh CC. Recurrent cellulitis: risk factors, etiology, pathogenesis and treatment. Curr Infect Dis Rep 2014;16(9):422. Cross Ref link Pubmed link
    • 36  Tay EY, Thirumoorthy T, Pang SM, Lee HY. Clinical outcomes of bacteraemia in cellulitis of the leg. Clin Exp Dermatol 2014;39(6):683–8. Cross Ref link Pubmed link
    • 37  Levell NJ, Wingfield CG, Garioch JJ. Severe lower limb cellulitis is best diagnosed by dermatologists and managed with shared care between primary and secondary care. Br J Dermatol 2011;164(6):1326–8. Cross Ref link Pubmed link
    • 38  British Lymphology Society (BLS). Consensus Document on the Management of Cellulitis in Lymphoedema. BLS, 2013. http://www.thebls.com/docs/Cellulitis_Consensus_2013.pdf (last accessed August 2015).

    Swollen arm

    • 39  DiSipio T, Rye S, Newman B, Hayes S. Incidence of unilateral arm lymphoedema after breast cancer: a systematic review and meta‐analysis. Lancet Oncol 2013;14(6):500–15. Cross Ref link Pubmed link
    • 40  Levick JR. An Introduction to Cardiovascular Physiology, 5th edn. London: Arnold, 2010.
    • 41  Fletcher PG, Sterling JC. Recurrent herpes simplex virus type 2 infection of the hand complicated by persistent lymphoedema. Australas J Dermatol 2005;46(2):110–13. Cross Ref link Pubmed link
    • 42  Pearce VJ, Mortimer PS. Hand dermatitis and lymphoedema. Br J Dermatol 2009;161(1):177–80. Cross Ref link Pubmed link
    • 43  Kiely PD, Bland JM, Joseph AE, et al. Upper limb lymphatic function in inflammatory arthritis. J Rheumatol 1995;22:214–17. Pubmed link
    • 44  Mulherin DM, Fitzgerald O, Bresnihan B. Lymphoedema of the upper limb in patients with psoriatic arthritis. Semin Arthritis Rheum 1993;22:350–6. Cross Ref link Pubmed link
    • 45  Keppler‐Noreuil KM, Sapp JC, Lindhurst MJ, et al. Clinical delineation and natural history of the PIK3CA‐related overgrowth spectrum. Am J Med Genet A 2014;164(7):1713–33. Cross Ref link
    • 46  Stanton A, Modi S, Mellor R, Levick R, Mortimer P. Diagnosing cancer‐related lymphoedema in the arm. J Lymphoedema 2006;1:12–15.
    • 47  Lymphoedema Framework. Best Practice for the Management of Lymphoedema. International Consensus. London: Medical Education, 2006. http://www.lympho.org/mod_turbolead/upload/file/Lympho/Best_practice_20_July.pdf (last accessed August 2015).
    • 48  British Lymphology Society (BLS). What is Lymphoedema? www.thebls.com/cellulitis (last accessed August 2015).
    • 49  Bosma J, Vahl AC, Coveliers HME, Rauwerda JA, Wisselink W. Primary subclavian vein thrombosis and its long‐term effect on quality of life. Vascular 2011;8:327–32. Cross Ref link

    Swollen face, head and neck

    • 50  Deng J, Murphy BA, Dietrich MS, et al. Impact of secondary lymphedema after head and neck cancer treatment on symptoms, functional status, and quality of life. Head Neck 2013;35(7):1026–35. Cross Ref link Pubmed link
    • 51  Dozsa A, Karoli ZS, Degrell P. Bilateral blepharochalasis. J Eur Acad Dermatol Venereol 2005;19:725–8. Cross Ref link Pubmed link
    • 52  Carlson JA, Mazza J, Kircher K, Tran TA. Otophyma: a case report and review of the literature of lymphedema (elephantiasis) of the ear. Am J Dermatopathol 2008;30(1):67–72 . Cross Ref link Pubmed link
    • 53  Buntzel J, Glatzel M, Mucke R, et al. Influence of amifostine on late radiation‐toxicity in head and neck cancer – a follow‐up study. Anticancer Res 2007;27:1953–6. Pubmed link
    • 54  Levick JR. An Introduction to Cardiovascular Physiology, 5th edn. London: Arnold, 2010.
    • 55  Connelly MG, Winkelmann RK. Solid facial edema as a complication of acne vulgaris. Arch Dermatol 1985;121:87–90. Cross Ref link Pubmed link
    • 56  Nagasaka T, Koyama T, Matsumura K, Chen KR. Persistent lymphoedema in Morbihan disease: formation of perilymphatic epithelioid cell granulomas as a possible pathogenesis. Clin Exp Dermatol 2008;33:764–7. Cross Ref link Pubmed link
    • 57  Manzoon S, Azadeh B. Elephantiasis of external ear: a rare manifestation of pediculosis capitis. Acta Derm Venereol (Stockh) 1983;63:363–5 .
    • 58  Worm AM, Staberg B, Thomsen K. Persistent oedema in allergic contact dermatitis. Contact Dermatitis 1983;9:517–18. Cross Ref link Pubmed link
    • 59  Nozicka Z. Endovasal granulomatous lymphangitis as a pathogenetic factor in cheilitis granulomatosa. J Oral Pathol 1985;14:363–5. Cross Ref link Pubmed link
    • 60  Salles AG, Lotierzo PH, Gemperli R, et al. Complications after polymethylmethacrylate injections: report of 32 cases. Plast Reconstr Surg 2008;121:1811–20. Pubmed link
    • 61  Lymphoedema Framework. Best Practice for the Management of Lymphoedema. International Consensus. London: Medical Education, 2006. http://www.lympho.org/mod_turbolead/upload/file/Lympho/Best_practice_20_July.pdf (last accessed August 2015).
    • 62  Odom R, Dahl M, Dover J, et al; National Rosacea Society Expert Committee on the Classification and Staging of Rosacea. Standard management options for rosacea, part 2: options according to subtype. Cutis 2009;84(2):97–104. Pubmed link
    • 63  Al Johani KA, Moles DR, Hodgson TA, Porter SR, Fedele S. Orofacial granulomatosis: clinical features and long‐term outcome of therapy. J Am Acad Dermatol 2010;62:611–20. Cross Ref link Pubmed link
    • 64  Smith BG, Hutcheson KA, Little LG, et al. Lymphedema outcomes in patients with head and neck cancer. Otolaryngol Head Neck Surg 2015;152(2):284–91. Cross Ref link Pubmed link

    Swollen genitalia and mons pubis

    • 65  Daroczy j. Pathology of lymphoedema. Clin Dermatol 1995;13:433–44. Cross Ref link Pubmed link
    • 66  Browse NL. Management of genital lymphoedema. In: Browse NL, Burnand KG, Mortimer PS, eds. Diseases of the Lymphatics. London: Arnold, 2003:217–30.
    • 67  Dandapat MC, Mohapatro SK, Patro SK. Elephantiasis of the penis and scrotum. A review of 350 cases. Am J Surg 1985;149:686–90. Cross Ref link Pubmed link
    • 68  Narang T, Kanwar AJ. Genital elephantiasis due to donovanosis: forgotten but not gone yet … Int J STD AIDS 2012;23(11):835–6. Cross Ref link Pubmed link
    • 69  Mansour S, Connell F, Steward C, et al; Lymphoedema Research Consortium. Emberger syndrome – primary lymphedema with myelodysplasia: report of seven new cases. Am J Med Genet A 2010;152A(9):2287–96. Cross Ref link Pubmed link
    • 70  Brice G, Mansour S, Bell R, et al. Analysis of the phenotypic abnormalities in lymphoedema‐distichiasis syndrome in 74 patients with FOXC2 mutations or linkage to 16q24. J Med Genet 2002;39(7):478–83. Cross Ref link Pubmed link
    • 71  Alders M, Al‐Gazali L, Cordeiro I, et al. Hennekam syndrome can be caused by FAT4 mutations and be allelic to Van Maldergem syndrome. Hum Genet 2014;133(9):1161–7. Cross Ref link Pubmed link
    • 72  Smpokou P, Tworog‐Dube E, Kucherlapati RS, Roberts AE. Medical complications, clinical findings, and educational outcomes in adults with Noonan syndrome. Am J Med Genet A 2012;158A(12):3106–11. Cross Ref link Pubmed link
    • 73  British Lymphology Society (BLS). What is Lymphoedema? www.thebls.com/cellulitis (last accessed August 2015).
    • 74  Brewer MB, Singh DP. Massive localized lymphedema: review of an emerging problem and report of a complex case in the mons pubis. Ann Plast Surg 2012;68(1):101–4. Cross Ref link Pubmed link
    • 75  Lymphoedema Framework. Best Practice for the Management of Lymphoedema. International Consensus. London: Medical Education, 2006. http://www.lympho.org/mod_turbolead/upload/file/Lympho/Best_practice_20_July.pdf (last accessed August 2015).
    • 76  Garaffa G, Christopher N, Ralph DJ. The management of genital lymphoedema. BJU Int 2008;102(4):480–4. Cross Ref link Pubmed link
    • 77  Thomas CL, Gordon KD, Mortimer PS. Rapid resolution of hidradenitis suppurativa after bariatric surgical intervention. Clin Exp Dermatol 2014;39(3):315–17. Cross Ref link Pubmed link

    Obesity‐related lymphoedema

    • 78  Disipio T, Rye S, Newman S, Hayes S. Incidence of unilateral arm lymphoedema after breast cancer: a systematic review and meta‐analysis. Lancet Oncol 2013;14(6):500–15. Cross Ref link Pubmed link
    • 79  Shaw C, Mortimer P, Judd PA. A randomized controlled trial of weight reduction as a treatment for breast cancer‐related lymphedema. Cancer 2007;110:1868–74. Cross Ref link Pubmed link
    • 80  Todd M. Managing chronic oedema in the morbidly obese patient. Br J Nurs 2009;18(18):1120–4. Cross Ref link Pubmed link
    • 81  Randolph GJ, Miller NE. Lymphatic transport of high‐density lipoproteins and chylomicrons. J Clin Invest 2014;124(3):929–35. Cross Ref link Pubmed link
    • 82  Lim HY, Thiam CH, Yeo KP, et al. Lymphatic vessels are essential for the removal of cholesterol from peripheral tissues by SR‐BI‐mediated transport of HDL. Cell Metab 2013;17(5):671–84. Cross Ref link Pubmed link
    • 83  Harvey NL, Srinivasan RS, Dillard ME, et al. Lymphatic vascular defects promoted by Prox1 haploinsufficiency cause adult‐onset obesity. Nat Genet 2005;37(10):1072–81. Cross Ref link Pubmed link
    • 84  O'Malley E, Ahern T, Dunlevy C, Lehane C, Kirby B, O'Shea D. Obesity related chronic lymphoedema‐like swelling and physical function. Q J Med 2015;108(3):183–7. Cross Ref link
    • 85  Greene AK, Grant FD, Slavin SA. Lower‐extremity lymphedema and elevated body‐mass index. N Engl J Med 2012;366:2136–7. Cross Ref link Pubmed link
    • 86  Arngrim N, Simonsen L, Holst JJ, Bulow J. Reduced adipose tissue lymphatic drainage of macromolecules in obese subjects: a possible link between obesity and local tissue inflammation. Int J Obes (Lond) 2013;37(5):748–50. Cross Ref link Pubmed link
    • 87  Arfvidsson B, Eklof B, Balfour J. Iliofemoral venous pressure correlates with intraabdominal pressure in morbidly obese patients. Vasc Endovascular Surg 2005;39:505–9. Cross Ref link Pubmed link
    • 88  Willenberg T, Schumacher A, Amann‐Vesti B, et al. Impact of obesity on venous hemodynamics of the lower limbs. J Vasc Surg 2010;52(3):664–8. Cross Ref link Pubmed link
    • 89  Lymphoedema Framework. Best Practice for the Management of Lymphoedema. International Consensus. London: Medical Education, 2006. http://www.lympho.org/mod_turbolead/upload/file/Lympho/Best_practice_20_July.pdf (last accessed August 2015).
    • 90  British Lymphology Society (BLS). What is Lymphoedema? www.thebls.com/cellulitis (last accessed August 2015).

    Abdominal wall lymphoedema

    • 91  Buyuktas D, Arslan E, Celik O, Tasan E, Demirkesen C, Gundogdu S. Elephantiasis nostras verrucosa on the abdomen of a Turkish female patient caused by morbid obesity [Letter]. Dermatol Online J 2010;16(8):14. Pubmed link
    • 92  Bull RH, Mortimer PS. Acute lipodermatosclerosis in a pendulous abdomen. Clin Exp Dermatol 1993;18(2):164–6. Cross Ref link Pubmed link
    • 93  British Lymphology Society (BLS). What is Lymphoedema? www.thebls.com/cellulitis (last accessed August 2015)..
    • 94  Lymphoedema Framework. Best Practice for the Management of Lymphoedema. International Consensus. London: Medical Education, 2006. http://www.lympho.org/mod_turbolead/upload/file/Lympho/Best_practice_20_July.pdf (last accessed August 2015).
    • 95  Ollapallil J, Koong D, Panchacharavel G, Butcher C, Yapo B. New method of abdominoplasty for morbidly obese patients. ANZ J Surg 2004;74(6):504–6. Cross Ref link Pubmed link

    Cancer‐related lymphoedema

    • 96  DiSipio T, Rye S, Newman B, Hayes S. Incidence of unilateral arm lymphoedema after breast cancer: a systematic review and meta‐analysis. Lancet Oncol 2013;14(6):500–15. Cross Ref link Pubmed link
    • 97  Jung SY, Shin KH, Kim M, et al. Treatment factors affecting breast cancer‐related lymphedema after systemic chemotherapy and radiotherapy in stage II/III breast cancer patients. Breast Cancer Res Treat 2014;148(1):91–8. Cross Ref link Pubmed link
    • 98  Snijders‐Keilholz A, Hellebrekers BW, Zwinderman AH, et al. Adjuvant radiotherapy following radical hysterectomy for patients with early‐stage cervical carcinoma (1984–1996). Radiother Oncol 1999;51:161–7. Cross Ref link Pubmed link
    • 99  Chatani M, Nose T, Masaki N, Inoue T. Adjuvant radiotherapy after radical hysterectomy of the cervical cancer. Prognostic factors and complications. Strahlenther Onkol 1998;174:504–9 . Cross Ref link Pubmed link
    • 100  Gaarenstroom KN, Kenter GG, Trimbos JB, et al. Postoperative complications after vulvectomy and inguinofemoral lymphadenectomy using separate groin incisions. Int J Gynecol Cancer 2003;13:522–7 . Cross Ref link Pubmed link
    • 101  Keegan KA, Cookson MS. Complications of pelvic lymph node dissection for prostate cancer. Curr Urol Rep 2011;12(3):203–8. Cross Ref link Pubmed link
    • 102  Pilepich MV, Krall J, George FW, et al. Treatment‐related morbidity in phase III RTOG studies of extended‐field irradiation for carcinoma of the prostate. Int J Radiat Oncol Biol Phys 1984;10(10):1861–7. Cross Ref link Pubmed link
    • 103  Bevan‐Thomas R, Slaton JW, Pettaway CA. Contemporary morbidity from lymphadenectomy for penile squamous cell carcinoma: the M.D. Anderson Cancer Center Experience. J Urol 2002;167:1638–42. Cross Ref link Pubmed link
    • 104  Hyngstrom JR, Chiang YJ, Cromwell KD, et al. Prospective assessment of lymphedema incidence and lymphedema‐associated symptoms following lymph node surgery for melanoma. Melanoma Res 2013;23(4):290–7. Pubmed link
    • 105  Friedmann D, Wunder JS, Ferguson P, et al. Incidence and severity of lymphoedema following limb salvage of extremity soft tissue sarcoma. Sarcoma 2011;2011:289673. Cross Ref link Pubmed link
    • 106  Donker M, van Tienhoven G, Straver ME, et al. Radiotherapy or surgery of the axilla after a positive sentinel node in breast cancer (EORTC 10981‐22023 AMAROS): a randomised, multicentre, open‐label, phase 3 non‐inferiority trial. Lancet Oncol 2014;15(12):1303–10. Cross Ref link Pubmed link
    • 107  Marneros AG, Blanco F, Husain S, et al. Classification of cutaneous intravascular breast cancer metastases based on immunolabeling for blood and lymph vessels. J Am Acad Dermatol 2009;60:633–8. Cross Ref link Pubmed link
    • 108  Finkel LJ, Griffiths CEM. Inflammatory breast carcinoma (carcinoma erysipeloides), an easily overlooked diagnosis. Br J Dermatol 1993;129:324–6. Cross Ref link Pubmed link

    Swollen breast and breast lymphoedema

    • 109  Boughey JC, Hoskin TL, Cheville AL, et al. Risk factors associated with breast lymphedema. Ann Surg Oncol 2014;21(4):1202–8. Cross Ref link Pubmed link
    • 110  Levick JR. An Introduction to Cardiovascular Physiology, 5th edn. London: Arnold, 2010.
    • 111  Jung SY, Shin KH, Kim M, et al. Treatment factors affecting breast cancer‐related lymphedema after systemic chemotherapy and radiotherapy in stage II/III breast cancer patients. Breast Cancer Res Treat 2014;148(1):91–8. Cross Ref link Pubmed link
    • 112  Boughey JC, Hoskin TL, Cheville AL, et al. Risk factors associated with breast lymphedema. Ann Surg Oncol 2014;21(4):1202–8. Cross Ref link Pubmed link
    • 113  Hille U, Soergel P, Makowski L, Dörk‐Bousset T, Hillemanns P. Lymphedema of the breast as a symptom of internal diseases or side effect of mTor inhibitors. Lymphat Res Biol 2012;10(2):63–73. Cross Ref link Pubmed link
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    Massive localized lymphoedema

    • 115  Lu S, Tran Ta, Jones DM, et al. Localized lymphedema (elephantiasis): a case series and review of the literature. J Cutan Pathol 2008;36:1–20. Cross Ref link Pubmed link
    • 116  Manduch M, Oliveira AM, Nascimento AG, Folpe AL. Massive localised lymphoedema: a clinicopathological study of 22 cases and review of the literature. J Clin Pathol 2009;62(9):808–11. Cross Ref link Pubmed link
    • 117  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;74(1):126–32. Cross Ref link Pubmed link
    • 118  Khanna M, Naraghi AM, Salonen D, et al. Massive localised lymphoedema: clinical presentation and MR imaging characteristics. Skeletal Radiol 2011;40(5):647–52 . Cross Ref link Pubmed link

    Primary lymphoedema

    • 119  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;77(5):438–52. Cross Ref link Pubmed link
    • 120  Ferrell RE, Levinson KL, Esman JH, et al. Hereditary lymphedema: evidence for linkage and genetic heterogeneity. Hum Mol Genet 1998;7(13):2073–8. Cross Ref link Pubmed link
    • 121  Evans AL, Brice G, Sotirova V, et al. Mapping of primary congenital lymphedema to the 5q35.3 region. Am J Hum Genet 1999;64(2):547–55. Cross Ref link Pubmed link
    • 122  Karkkainen MJ, Ferrell RE, Lawrence EC, et al. Missense mutations interfere with VEGFR‐3 signalling in primary lymphoedema. Nature Genet 2000;25(2):153–9. Cross Ref link Pubmed link
    • 123  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;67(2):295–301. Cross Ref link Pubmed link
    • 124  Mangion J, Rahman N, Mansour S, et al. A gene for lymphedema‐distichiasis maps to 16q24.3. Am J Hum Genet 1999;65(2):427–32. Cross Ref link Pubmed link
    • 125  Fang JM, Dagenais SL, Erickson RP, et al. Mutations in FOXC2 (MFH‐1), a forkhead family transcription factor, are responsible for the hereditary lymphedema‐distichiasis syndrome. Am J Hum Genet 2000;67(6):1382–8. Cross Ref link Pubmed link
    • 126  Brice G, Mansour S, Bell R, et al. Analysis of the phenotypic abnormalities in lymphoedema‐distichiasis syndrome in 74 patients with FOXC2 mutations or linkage to 16q24. J Med Genet 2002;39(7):478–83. Cross Ref link Pubmed link
    • 127  Mendola A, Schlogel MJ, Ghalamkarpour A, et al. Mutations in the VEGFR3 signaling pathway explain 36% of familial lymphedema. Mol Syndromol 2013;4(6):257–66. Cross Ref link Pubmed link
    • 128  Connell F, Gordon K, Brice G, et al. The classification and diagnostic algorithm for primary lymphatic dysplasia: an update from 2010 to include molecular findings. Clin Genet 2013;84(4):303–14. Cross Ref link Pubmed link
    • 129  Dale RF. The inheritance of primary lymphoedema. J Med Genet 1985;22(4):274–8. Cross Ref link Pubmed link
    • 130  Moffatt CJ, Franks PJ, Doherty DC, et al. Lymphoedema: an underestimated health problem. Q J Med 2003;96(10):731–8. Cross Ref link
    • 131  Mortimer PS. Managing lymphoedema. Clin Exp Dermatol 1995;20(2):98–106. Cross Ref link Pubmed link
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    Lipoedema

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    Yellow nail syndrome

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    Lymphatic malformation and lymphangioma circumscriptum

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    Lymphoedema as a result of amniotic band constriction

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    Lymphangiomatosis, lymphangioleiomyomatosis and non‐malignant lymphatic tumours

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    Lymphangiectasia

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    • 253  Harwood CA, Mortimer PS. Acquired vulval lymphangiomata mimicking genital warts. Br J Dermatol 1993;129: 34–6. Cross Ref link
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    Lymphocele, seroma and lymph fistula

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    Lymphatic filariasis

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    Podoconiosis

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    • 297  Price EW. The association of endemic elephantiasis of the lower legs in East Africa with soil derived from volcanic rocks. Trans R Soc Trop Med Hyg 1976;70(4):288–95. Cross Ref link Pubmed link
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    Pretibial myxoedema

    • 303  Fatourechi V, Pajouhi M, Fransway AF. Dermopathy of Graves disease (pretibial myxedema). Review of 150 cases. Medicine (Baltimore) 1994;73:1. Cross Ref link Pubmed link
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    • 311  Davies TF. Trauma and pressure explain the clinical presentation of the Graves' disease triad. Thyroid 2000;10:629–30. Cross Ref link Pubmed link
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    • 314  Wright AL, Buxton PK, Menzies D. Pretibial myxedema localized to scar tissue. Int J Dermatol 1990;29:54–5. Cross Ref link Pubmed link
    • 315  Akasu F, Takazawa K, Akasu R, et al. Localized myxedema on the nasal dorsum in a patient with Graves' disease: report of a case. J Endocrinol Invest 1989;12:717–21. Cross Ref link Pubmed link
    • 316  Noppakun N, Bancheun K, Chandraprasert S. Unusual locations of localized myxedema in Graves' disease. Report of three cases. Arch Dermatol 1986;122:85–8. Cross Ref link Pubmed link
    • 317  Rice SA, Peden NR, McGlynn S, et al. Atypical presentation of infiltrative thyroid dermopathy. Clin Exp Dermatol 2010;35:56–8. Cross Ref link Pubmed link
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    • 319  Takasu N, Higa H, Kinjou Y. Treatment of pretibial myxedema with topical steroid ointment application with sealing cover (steroid occlusive dressing technique: steroid ODT) in Graves' patients. Intern Med 2010;49(7):665–9. Cross Ref link Pubmed link
    • 320  Pingsmann A, Ockenfels HM, Patsalis T. Surgical excision of pseudotumorous pretibial myxedema. Foot Ankle Int 1996;17:107–10. Cross Ref link Pubmed link
    • 321  Deng A, Song D. Multipoint subcutaneous injection of long‐acting glucocorticid as a cure for pretibial myxedema. Thyroid 2011;21:83–5. Cross Ref link Pubmed link
    • 322  Wiersinga WM. Therapy: evidence‐based treatment of Graves ophthalmopathy. Nature Rev Endocrinol 2009;5:653–4. Cross Ref link
    • 323  Dandona P, Marshall NJ, Bidey SP, et al. Successful treatment of exophthalmos and pretibial myxoedema with plasmapheresis. BMJ 1979;1:374–6. Cross Ref link Pubmed link
    • 324  Antonelli A, Navarranne A, Palla R, et al. Pretibial myxedema and high‐dose intravenous immunoglobulin treatment. Thyroid 1994;4:399–408. Cross Ref link Pubmed link
    • 325  Heyes C, Nolan R, Leahy M, Gebauer K. Treatment‐resistant elephantiasic thyroid dermopathy responding to rituximab and plasmapheresis. Australas J Dermatol 2012;53(1):e1–4. Cross Ref link Pubmed link
    • 326  Wemeau JL, Caron P, Beckers A, et al. Octreotide (long‐acting release formulation) treatment in patients with Graves' orbitopathy: clinical results of a four‐month, randomized, placebo‐controlled, double‐blind study. J Clin Endocrinol Metab 2005;90:841–8. Cross Ref link Pubmed link

    Trauma‐induced lymphoedema

    • 327  Ouvry PA, Guenneguez H. Lymphatic complications from variceal surgery. Phlebologie 1993;46:563–8. Pubmed link
    • 328  Pittaluga P, Chastanet S. Lymphatic complications after varicose veins surgery: risk factors and how to avoid them. Phlebology 2012;27(Suppl. 1):139–42. Cross Ref link Pubmed link
    • 329  Disipio T, Rye S, Newman S, Hayes S. Incidence of unilateral arm lymphoedema after breast cancer: a systematic review and meta‐analysis. Lancet Oncol 2013;14(6):500–15. Cross Ref link Pubmed link
    • 330  Nikitenko LL, Shimosawa T, Henderson S, et al. Adrenomedullin haploinsufficiency predisposes to secondary lymphedema. J Invest Dermatol 2013;133(7):1768–76. Cross Ref link Pubmed link
    • 331  Kissin MW, della Rovere GO, Easton D, et al. Risk of lymphoedema following the treatment of breast cancer. Br J Surg 1986;73:580–4. Cross Ref link Pubmed link
    • 332  Unno N, Yamamoto N, Suzuki M, et al. Intraoperative lymph mapping with preoperative vein mapping to prevent postoperative lymphorrhea following paramalleolar bypass surgery in patients with critical limb ischemia. Surg Today 2014;44(3):436–42. Cross Ref link Pubmed link
    • 333  Buchel EW, Dalke KR, Hayakawa TE. The transverse upper gracilis flap: eefficiencies and design tips. Can J Plast Surg 2013;21(3):162–6. Pubmed link
    • 334  Tourani SS, Taylor GI, Ashton MW. Understanding the three‐dimensional anatomy of the superficial lymphatics of the limbs. Plast Reconstr Surg 2014;134(5):1065–74. Cross Ref link Pubmed link
    • 335  Reading G. Secrétan's syndrome: hard edema of the dorsum of the hand. Plast Reconstr Surg 1980;65:182–7. Cross Ref link Pubmed link
    • 336  Nwaejike N, Archbold H, Wilson DS. Factitious lymphoedema as a psychiatric condition mimicking reflex sympathetic dystrophy: a case report. J Med Case Rep 2008;2:216. Cross Ref link Pubmed link
    • 337  Andresz V, Marcantoni N, Binder F, et al. Puffy hand syndrome due to drug addiction: a case–control study of the pathogenesis. Addiction 2006;101(9):1347–51. Cross Ref link Pubmed link
    • 338  Vollum DI. Skin lesions in drug addicts. BMJ 1970;2(7):647–50. Cross Ref link Pubmed link
    • 339  Lymphoedema Framework. Best Practice for the Management of Lymphoedema. International Consensus. London: Medical Education, 2006. http://www.lympho.org/mod_turbolead/upload/file/Lympho/Best_practice_20_July.pdf (last accessed August 2015).
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    Lymphoedema due to immobility

    • 341  Garcia AM, Dicianno BE. The frequency of lymphedema in an adult spina bifida population. Am J Phys Med Rehabil 2011;90(2):89–96. Cross Ref link Pubmed link
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    • 343  Geurts AC, Visschers BA, van Limbeek J, Ribbers GM. Systematic review of aetiology and treatment of post‐stroke hand oedema and shoulder‐hand syndrome. Scand J Rehabil Med 2000;32(1):4–10. Cross Ref link Pubmed link
    • 344  Sneddon I, Church R. Practical Dermatology, 4th edn. London: Arnold, 1983:166.
    • 345  Bull RH, Mortimer PS. Acute lipodermatosclerosis in a pendulous abdomen. Clin Exp Dermatol 1993;18:164–6. Cross Ref link Pubmed link
    • 346  Dirette D, Hinojosa J. Effects of continuous passive motion on the edematous hands of two persons with flaccid hemiplegia. Am J Occup Ther 1994;48(5):403–9. Cross Ref link Pubmed link

    Lymphangitis

    • 347  Gill MJ, Arlette J, Tyrrell DL, Buchan KA. Herpes simplex virus infection of the hand. Clinical features and management. Am J Med 1988;85(2A):53–6. Cross Ref link Pubmed link
    • 348  Taylor MJ, Hoerauf A, Bockarie M. Lymphatic filariasis and onchocerciasis. Lancet 2010;376(9747):1175–85. Cross Ref link Pubmed link
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    • 350  Barnes JM, Trueta J. Absorption of bacteria and snake venoms from the tissues: importance of the lymphatic circulation. Lancet 1941;1:623–6. Cross Ref link
    • 351  Pozio E. Venomous snakebites in Italy: epidemiological and clinical aspects. Trop Med Parasitol 1988;39(1):62–6. Pubmed link
    • 352  Anker RL, Straffon WG, Loiselle DS. First aid for snake bite. Med J Aust 1982;1:103. Pubmed link
    • 353  Marsch W, Haas N, Stuttgen G. Mondor's phlebitis: a lymphovascular process. Dermatologica 1986;172:133–8. Cross Ref link Pubmed link
    • 354  Moskovitz AH, Anderson BO, Yeung RS, et al. Axillary web syndrome after axillary dissection. Am J Surg 2001;181:434–9. Cross Ref link Pubmed link
    • 355  Ingram DM, Sheiner HJ, Ginsberg AM. Mondor's disease of the breast resulting from jellyfish sting. Med J Aust 1992;157(11–12):836–7. Pubmed link
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    • 357  Babu AK, Krishnan P, Andezuth DD. Sclerosing lymphangitis of penis – literature review and report of 2 cases. Dermatol Online J 2014;20(7):ii.
    • 358  Marneros AG, Blanco F, Husain S, et al. Classification of cutaneous intravascular breast cancer metastases based on immunolabeling for blood and lymph vessels. J Am Acad Dermatol 2009;60:633–8. Cross Ref link Pubmed link
    • 359  Finkel LJ, Griffiths CEM. Inflammatory breast carcinoma (carcinoma erysipeloides), an easily overlooked diagnosis. Br J Dermatol 1993;129:324–6. Cross Ref link Pubmed link

    Complications of lymphoedema

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    • 362  Mortimer PS, Rockson SG. New developments in clinical aspects of lymphatic disease. J Clin Invest 2014;124(3):915–21. Cross Ref link Pubmed link
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    • 364  Soria X, Bielsa I, Ribera M, et al. Acute dermal abscesses caused by Serratia marcescens. J Am Acad Dermatol 2008;58(5):891–3. Cross Ref link Pubmed link
    • 365  Moffatt CJ, Franks PJ, Doherty DC, et al. Lymphoedema: an underestimated health problem. Q J Med 2003;96(10):731–8. Cross Ref link
    • 366  Tobin MB, Lacey HJ, Meyer L, Mortimer PS. The psychological morbidity of breast cancer‐related arm swelling. Psychological morbidity of lymphoedema. Cancer 1993;72(11):3248–52. Cross Ref link Pubmed link
    • 367  Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphoedema. Cancer 1948;1:64–81. Cross Ref link Pubmed link
    • 368  Lee R, Saardi KM, Schwartz RA. Lymphedema‐related angiogenic tumors and other malignancies. Clin Dermatol 2014;32(5):616–20. Cross Ref link Pubmed link
    • 369  Benson PM, Pessoa CM, Lupton GP, et al. Basal cell carcinomas arising in chronic lymphoedema. J Dermatol Surg Oncol 1988;14:781–3. Cross Ref link Pubmed link
    • 370  Epstein JL, Mendelsohn G. Squamous carcinoma of the foot arising in association with longstanding verrucous hyperplasia in a patient with congenital lymphoedema. Cancer 1984;54:943–7. Cross Ref link Pubmed link
    • 371  Waxman M, Fatteh S, Elias JM, et al. Malignant lymphoma of skin associated with postmastectomy lymphoedema. Arch Pathol Lab Med 1984;108:206–8. Pubmed link
    • 372  Tatnall FM, Mann BS. Non‐Hodgkin's lymphoma of the skin associated with chronic limb lymphoedema. Br J Dermatol 1985;113:751–6. Cross Ref link Pubmed link
    • 373  Hills RJ, Ive FA. Cutaneous secondary follicular centre cell lymphoma in association with lymphoedema praecox. Br J Dermatol 1993;129:186–9. Cross Ref link Pubmed link
    • 374  Sarkany I. Malignant melanomas in lymphoedematous arm following radical mastectomy for breast carcinoma. J R Soc Med 1972;65:253–4.
    • 375  Bartal AH, Pinsky CM. Malignant melanoma appearing in a postmastectomy lymphoedematous arm: a novel association of double primary tumours. J Surg Oncol 1985;30:16–18. Cross Ref link Pubmed link
    • 376  Fergusson CM, Copeland SA, Horton L. Unusual sarcoma arising in lymphoedema. J R Soc Med 1985;78:1497–8.
    • 377  Peterson CM, Lane JE, Guill MA. Merkel cell carcinoma after postmastectomy lymphedema. J Am Acad Dermatol 2003;48(6):983. Cross Ref link Pubmed link
    • 378  Merimsky O, Chaitchik S. Kaposi's sarcoma on a lymphoedematous arm following radical mastectomy. Tumori 1992;78:407–8. Pubmed link
    • 379  Pearson IC, Cox NH, Ostlere LS, Marsden RA, Mortimer PS. Porokeratosis in association with lymphoedema. Clin Exp Dermatol 2005;30(2):152–4. Cross Ref link Pubmed link
    • 380  Tatnall FM, Sarkany I. Primary focal lymphoedema with xanthoma. J R Soc Med 1988;81:113–14. Pubmed link
    • 381  Romaní J, Luelmo J, Sáez A, et al. Localized xanthomas associated with primary lymphedema. Pediatr Dermatol 2012;29(1):113–14. Cross Ref link Pubmed link
    • 382  Callens A, Vaillant L, Machet MC, et al. Localized atypical pemphigoid on lymphoedema following radiotherapy. Acta Derm Venereol (Stockh) 1993;73:461–4.
    • 383  Wilkinson SM, Heagarty AH, Smith AG. Toxic epidermal necrolysis localized to an area of lymphoedema. Clin Exp Dermatol 1991;17:456–7. Cross Ref link
    • 384  Demitsu T, Tadaki T. Atypical neutrophilic dermatosis on the upper extremity affected by post‐mastectomy lymphoedema: report of 2 cases. Dermatologica 1991;183:230–3. Cross Ref link Pubmed link
    • 385  Kaier T, Larsen J. Necrotizing fasciitis in congenital lymphoedema. Int J Dermatol 1990;29:41–4. Cross Ref link Pubmed link

    Imaging of the lymphatic system

    • 386  Kinmonth JB. Lymphangiography in man; a method of outlining lymphatic trunks at operation. Clin Sci (Lond) 1952;11(1):13–20. Pubmed link
    • 387  Stewart G, Gaunt JI, Croft DN, Browse NL. Isotope lymphography: a new method of investigating the role of the lymphatics in chronic limb oedema. Br J Surg 1985;72(11):906–9. Cross Ref link Pubmed link
    • 388  Proby CM, Gane JN, Joseph AE, Mortimer PS. Investigation of the swollen limb with isotope lymphography. Br J Dermatol 1990;123(1):29–37. Cross Ref link Pubmed link
    • 389  Modi S, Stanton AW, Mortimer PS, Levick JR. Clinical assessment of human lymph flow using removal rate constants of interstitial macromolecules: a critical review of lymphoscintigraphy. Lymph Res Biol 2007;5(3):183–202. Cross Ref link
    • 390  Mellor RH, Hubert CE, Stanton AW, et al. Lymphatic dysfunction, not aplasia, underlies Milroy disease. Microcirculation 2010;17(4):281–96. Cross Ref link Pubmed link
    • 391  Xiong L, Engel H, Gazyakan E, et al. Current techniques for lymphatic imaging: state of the art and future perspectives. Eur J Surg Oncol 2014;40(3):270–6. Cross Ref link Pubmed link
    • 392  Unno N, Inuzuka K, Suzuki M, et al. Preliminary experience with a novel fluorescence lymphography using indocyanine green in patients with secondary lymphedema. J Vasc Surg 2007;45(5):1016–21. Cross Ref link Pubmed link
    • 393  Yamamoto T, Yamamoto N, Azuma S, et al. Near‐infrared illumination system‐integrated microscope for supermicrosurgical lymphaticovenular anastomosis. Microsurgery 2014;34(1):23–7. Cross Ref link Pubmed link
    • 394  Lohrmann C, Foeldi E, Bartholoma JP, Langer M. Interstitial MR lymphangiography – a diagnostic imaging method for the evaluation of patients with clinically advanced stages of lymphedema. Acta Trop 2007;104(1):8–15. Cross Ref link Pubmed link
    • 395  Costa da Cunha Castro E, Galambos C. Prox‐1 and VEGFR3 antibodies are superior to D2‐40 in identifying endothelial cells of lymphatic malformations – a proposal of a new immunohistichemical panel to differentiate lymphatic from other vascular malformations. Pediatr Dev Pathol 2009;12:187–94. Cross Ref link Pubmed link
    • 396  Connell F, Gordon K, Brice G, et al. The classification and diagnostic algorithm for primary lymphatic dysplasia: an update from 2010 to include molecular findings. Clin Genet 2013;84(4):303–14. Cross Ref link Pubmed link

    Lymphoedema management

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    • 398  Foldi E, Foldi M, Weissleder H. Conservative treatment of lymphoedema of the limbs. Angiology 1985;36:171–80. Cross Ref link Pubmed link
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    • 400  Ikomi F, Schmid‐Schonbein GW. Lymph transport in skin. Clin Dermatol 1995;13:419–27. Cross Ref link Pubmed link
    • 401  Badger CMA, Peacock JL, Mortimer PS. A randomized, controlled, parallel group clinical trial comparing multi‐layer bandaging followed by hosiery versus hosiery alone in the treatment of patients with lymphedema of the limb. Cancer 2000;88:2832–7. Cross Ref link Pubmed link
    • 402  Anonymous. Compression for lymphoedema. Lancet 1986;i:896.
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    • 404  Shaw C, Mortimer P, Judd PA. A randomised controlled trial of weight reduction as a treatment for breast cancer‐related lymphedema. Cancer 2007;110:1868–74. Cross Ref link Pubmed link
    • 405  Shaw C, Mortimer P, Judd PA. Randomised controlled trial comparing a low‐fat diet with a weight‐reduction diet in breast cancer‐related lymphedema. Cancer 2007;109:1949–56. Cross Ref link Pubmed link
    • 406  Gothard L, Stanton A, MacLaren J, et al. Non‐randomised phase II trial of hyperbaric oxygen therapy in patients with chronic arm lymphoedema and tissue fibrosis after radiotherapy for early breast cancer. Radiother Oncol 2004;70:217–24. Cross Ref link Pubmed link
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