Managing lymphedema

Managing lymphedema

ELSEVIER Managing Lymphedema PETER S. MORTIMER, MD, FRCP rice established, lymphedema is to all intents and purposes an incurable problem and, th...

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ELSEVIER

Managing Lymphedema PETER

S. MORTIMER,

MD,

FRCP

rice established, lymphedema is to all intents and purposes an incurable problem and, therefore, all treatment is essentially palliative. That is not to say nothing can be done. The rather nihilistic approach to lymphedema treatment is both unacceptable and unfounded. The management of lymphedema varies greatly around the world. In developed countries the emphasis is more on physical forms of therapy involving compression, whereas in poorer, hotter countries, where hosiery and appropriate bandages are too costly, surgery may be the mainstay of treatment. There is therefore a need to consider treatment based on the underlying cause(s), the problems created for the patient, and the circumstances of that patient. The three main (known) consequences of lymphatic failure are swelling (lymphoedema), infection (e.g., cellulitis), and, rarely, cancer (e.g., lymphangiosarcoma). They may occur as isolated events or in combination. The diagnosis of lymphedema is frequently delayed and may go unrecognized altogether. One of the reasons is the lack of readily available, easy-to-use investigative methods for confirming lymphatic failure unlike, for example, in venous disease. Equally, many clinicians and even dermatologists are unaware of the many cutaneous signs of lymphatic failure that help point to a diagnosis.

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Basic Principles Lymphedema is perceived as a rare condition and yet chronic edema is common, What is the difference? Medical textbooks tend to refer to edema of heart failure, nephrotic syndrome, hypoproteinemia, venous disease, and so on. This classification of edemas according to the underlying medical condition detracts from the fact that all edemas, whatever the underlying cause, are due to an imbalance between capillary filtration and lymph drainage.’ Edema represents an increase in extracellular fluid volume to the point of swelling. Control of extracellular volume is predominantly by the lymphatic, which normally compensates for any increase in capillary filFrom the Department of Dermatology, St. George’s and Royal Marsden Hospitals, and the University of London, London, United Kingdom. Address correspondence to Peter S. Mortimer, MD, Department of Dermatology, OQT, UK.

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tration by increasing lymph flow. This “safety valve” role therefore implicates the lymphatic in the development of all edemas. That is not to say all edema is lymphedema, because lymphedema occurs strictly when tissue swelling develops through a failure of lymph drainage in the face of a normal load (capillary filtration). Most edemas are due to increased capillary filtration overwhelming lymph drainage. Were clinicians to approach the problem of edema from a “physiologic” rather than a “disease” viewpoint, they would be much less likely to miss (or misunderstand) lymphedema.

Limb Lymphedema Historical Aspects to Diagnosis Most cases of lymphedema affect limbs. Presumably this is because there are reduced options for collateral (escape) lymph drainage owing to the relatively narrow exit from the root of a limb. Swelling results in discomfort, heaviness, reduced mobility, and impaired function. Moderate to severe pain is not a feature of lymphedema per se and, if present, should suggest alternative pathology. Nevertheless, the size and weight of some limbs soon produce secondary complications such as musculoskeletal problems .’ Edema that easily subsides with overnight elevation suggests increased capillary filtration is the predominant mechanism. Conversely, edema that does not change much overnight suggests lymphedema. The reason is simply that elevation, by lowering venous pressure, reduces capillary filtration but makes little difference to lymph drainage. This has implications for treatment. Difficulty in the clinical diagnosis of lymphedema is likely to be encountered in the early stages before the characteristic skin and subcutaneous tissue changes ensue. Indeed, it is not uncommon for lymphedema to present with swelling which comes and goes. Cancer rarely presents in the first instance with lymphedema because lymph flow is maintained remarkably well through malignant nodes. Lymphoma, for example, does not produce lymphedema, and it is only in circumstances of advanced cancer (e.g., carcinoma of the prostate) where swelling can be a presenting feature. Nevertheless it is incumbent on alI clinicians to consider underlying tumor in anyone presenting with peripheral edema, particularly if it is 0738-081 X/95/$32.00 SSDZ 0738-081X(9.5~00095-W

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unilateral, and to perform the necessary full clinical examination and appropriate investigations. Lymphedema, however, may be a manifestation of relapsed cancer because of direct infiltration of collateral lymph drainage routes. Once main lymphatic channels have been damaged by surgery or radiation, it becomes easier for malignant cells to infiltrate and thus block small collateral lymphatics within the tissues. Patients living in or traveling from tropical countries should be questioned about filariasis. A detailed account of the natural history and other aspects of filariasis is beyond the scope of this article, but confirmation of infection should be made and the appropriate treatment initiated. Swelling, however, will remain a permanent sequela and needs to be treated as for any other cause. A condition often confused with filarial elephantiasis is podoconiosis3 or nonfilarial elephantiasis. The clinical manifestations of swollen feet and legs are no different from that of any other cause, but the cause in this condition is uptake of microparticles of silica through the soles of the feet. It exists in nonfilarial areas of tropical Africa, Central America, and, to some extent, the Indian subcontinent, and occurs only in barefoot walkers. One complication of poor lymph drainage regardless of cause is recurrent inflammatory episodes of cellulitis. These can be so characteristic as to suggest lymphedema from the history even though swelling may be minimal. Certain forms of primary lymphedema are hereditary, and a history of more than one family member with chronic asymmetric leg swelling would be strongly suggestive of lymphedema.

Making the Diagnosis Lymphedema should be considered in any chronically swollen limb without pain or inflammation. The diagnosis depends on the history and characteristic changes in the skin and subcutaneous tissues. Traditionally, lymphedema is described as a brawny edema that does not readily pit. This is in part true but not a reliable sign. Although most swelling occurs in the subcutaneous layer, it is the skin that exhibits the most changes. It becomes thicker as demonstrated by the Kaposi-Stemmer sign, a failure to pick up or pinch a fold of skin at the base of the second toe.* Skin creases become enhanced and hyperkeratosis develops. As dermal lymph stasis progresses, elephantiasis changes become more marked. Dilation of upper dermal lymphatics with consequent organization and fibrosis of tissue gives rise to further hyperkeratosis and papillomatosis. Such skin changes occur most frequently in

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the lower limb, owing to the compounding effects of gravity. It should be borne in mind that edema may be absent in cases where fibrosis predominates. When advanced, the term mossy foot is used, and secondary bacterial and fungal infection becomes common. Confirmation should be made by isotopic lymphoscintigraphy5 (Figure 1) or, if necessary, by radiocontrast lymphography. Exclusion of general medical central causes of edema should be paramount. Some form of venous assessment is desirable because of the frequent coexistence of venous and lymphatic disease. PhleboFigure 2. Isotope lymphoscintigraphy. No discernible lymph drainage in the right leg. Uptake is normal in the left iliofemoral nodes, although minor structural abnormalities giving dermal backflow are demonstrable in peripheral lymphatics.

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graphy provides anatomic but not functional information, and a physiologic venous test such as photoplethysmography or Doppler assessment is preferable.

General Consider-ations Unlike all other forms of edema, lymphedema is not simply fluid accumulation and herein lies the difficulty for treatment. The “solid” component is due not just to the accumulation of macromolecules, particularly protein and lipid, that cannot escape from the tissues by any other way, but to the changes within the tissue that these materials provoke. Such changes as demonstrated by histopathology are those of chronic inflammation and fibrosis. Two particular problems need to be overcome with lymphedema: the swelling and the predisposition to infections, particularly recurrent cellulitis. Treatment of the edema may be by physical or surgical means. Drug treatment for lymphedema is generally disappointing.

Physical (Decongestive) Therapy Physical methods of treating lymphedema have been practiced in Europe for many years.‘j Therapy aims essentially to control lymph formation (capillary filtration), including inflammatory causes or venous hypertension, and to improve lymph drainage through existing lymphatics and collateral routes by applying normal physiologic procedures that stimulate lymph flow. Physical treatment can, in the majority of cases, improve quality of life considerably (Figure 2). Central to management is getting the patients to understand their condition and know what they can do for themselves.7 Only then can a high level of motivation and compliance with treatment be generated. It is important to explain to patients that unlike the blood, which is propelled by the heart, lymph drainage relies on changes in tissue pressure to generate flow. It is through local “massaging” of tissues by intermittent heart muscle contraction, passive movements, local arterial pulsation, breathing, and surface massage (pressure, shearing forces) that tissue fluid accesses lymphatics and becomes lymph. Initial lymphatic vessels act as simple conduits transporting lymph in response to local pressure changes,8 and it is not until lymph enters lymphatic collectors, which contain smooth muscle, that active propulsion of lymph flow occurs. Therefore, physical treatment exploits these physiologic principles, enhancing lymph flow as much as possible within the limits of a compromised drainage. After all, lymph flow still exists in lymphedema; otherwise, swelling would be a relentlessly progressive process. As it is, the natural history of lymphedema is for swelling to develop and then remain in a steady

Figure 2. Resultsof physicalfdecongestive) therapywith reductionof postmastectomy edrma.A. Bejoretreatment.B. After treatment.

state9 for months to years, with stepwise deterioration according to compounding influences (e.g., trauma, infection).

Minimum Provision of Care The cornerstone of limb lymphedema treatment is elastic hosiery. lo Such garments (below-knee or full-length stockings, half or full tights or sleeves) are not designed simply to “squeeze” edema but to limit capillary filtration by opposing capillary pressure and act as a counterforce to striated muscle contractions, which act as the most powerful stimulus to lymph drainage. Exercise with an emphasis on movement rather than vigor is therefore not only an additional and complementary treatment component to hosiery, but without

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it the hosiery is ineffective. Dynamic muscle contractions (isotonic exercises) encourage both passive (movement of lymph along tissue planes or through open lymphatics) and active (increased contractility and therefore propulsion of lymph within contractile lymphatics) phases of lymph drainage. Overexertion of excessive static (isometric, e.g., gripping) exercise will, by increasing blood flow, tend to increase edema, not help it. Exercise, breathing, and posture are important for clearance of central dilated and obstructed lymphatics in the thorax and abdomen, without the dispersal of which more peripheral edema will persist. Massage is a contentious issue despite being an accepted treatment for lymphedema in continental Europe. The problem is that there are different forms of massage, used mostly for increasing blood flow and therefore of a vigorous nature. Tissue movement, if gentle, is a stimulus to lymph flow without increasing blood flow (and, therefore, capillary filtration). *’ If performed correctly, simple skin surface massage encourages the movement of lymph either along tissue planes or through the intercommunicating network of skin and subcutaneous lymphatics to normally drained areas, from the leg to the axilla or from one side of the body to another. 6 Even in an obstructed system there should be sufficient reserve transport capacity to allow drainage by collateral routes as occurs in the venous system. Manual lymphatic drainage (MLD) therapy is a specific form of manual massage that is directed primarily at normally draining lymph node regions, thereby “siphoning” or “milking” lymph away from congested areas and complementing attempts to improve drainage “upstream” generated by muscle pumps. i* MLD is the only effective treatment for midline lymphedema (i.e., trunk, face, genitalia) where external compression is impractical. Prevention of infection, particularly lymphangitis/ cellulitis, is crucial to control of lymphedema. Recurrent cellulitis can be debilitating and lead to deteriorating swelling. The cause is unknown, but as prophylactic penicillin is effective in the majority of patients, it would appear that a failure of local immune mechanisms to contain bacterial and probably streptococcal antigen seems likely. Care of the skin, good hygiene, control of tinea pedis, chronic dermatitis, and other inflammatory dermatoses, as well as good antisepsis following abrasions and minor wounds, are as important as in diabetes. Administration of antibiotics at the time of an attack of cell&is must be very prompt; otherwise, as in outbreaks of herpes simplex, they do not significantly influence the course of the illness. Indeed, there are striking parallels between recurrent cellulitis and recurrent herpes simplex. The only effective treatment is phenoxymethyl penicillin 500 mg daily prophylactically for an indefinite period. Control of the edema should help reduce antibiotic requirements.

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Additional Treatments Pneumatic compression therapy (e.g., Flowtron) or intermittent/sequential pneumatic compression is widely employed.i3 Consisting of an inflatable sleeve or boot connected to a motor driven pump, these machines reduce limb volume by displacement of fluid and help soften a limb by disrupting tissue fibrosis. If external support (e.g., hosiery) is not fitted immediately following compression therapy, a limb readily reswells. Care must be taken to ensure that fluid is not forcibly displaced from the limb to the adjoining quadrant of the trunk to create swelling where it was not previously a problem. Furthermore, edema and distended lymphatits proximal to the pump must be managed before the more distal effects of the massage are activated. Multilayer bandaging can be used for compression therapy in limb reduction.6 The technique involves the application of layers of strong nonelastic (short stretch) bandages to generate a high pressure during muscular contractions but low pressure at rest. The use of foam or Velband helps distribute pressure more evenly as well as protect the skin (Figure 3). The strategic positioning of rubber pads helps “iron out” pockets of swelling and restore shape so that subsequently, a hose fits better and therefore is more effective at maintaining the volume reductions. Multilayer bandaging is a skill that takes time to learn and should not be undertaken by any professional without appropriate training. Drug therapy is, generally speaking, disappointing. Diuretics remain the most commonly used treatment because, to most doctors, edema is an indication for such drugs. Diuretics alone have very little benefit in lymphedema simply because their main action is to limit capillary filtration by reducing circulating blood volume. Indeed, improvement from diuretics suggests that the predominant cause of the edema is not lympathic. Such causes should be treated as well if lymphedema is to be optimally managed. The Rutoside group of drugs (e.g., oxerutins) have been advocated for use in lymphedema, and a recent placebo-controlled trial of benzopyrones showed benefit, only after several months, in a variety of forms of lymphedema, but further confirmation is required.14 Microwave heat therapy has been popular in China. l5 Possibly it activates macrophage clearance of macromolecules and reduces fibrosis by stimulating collagenase or altering the viscosity and stiffness of the tissues. Surgery for lymphedema can be of two types: reconstructive and debulking. Lymphatic microsurgery may be of value in j circumstances of obstructive lymphedema. Direct lymph-venous shunts or interposition of autologous vessel transplantation has been described, but long-term results are lacking. More con-

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Figure 4. Faciallymphedema.Often the underlying causeis unknown.

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Figure 3. Multilayer bandagingis a skilledprocedureinvolving carefulapplicationofprotective Velband,to distributepressure with compression “nonelastic”bandages on top.

ventional debulking operations are still performed if all else fails, to reduce the size of the swollen part.i6 Difficulties with wound healing can create short- and long-term problems.

Midline

Lymphedema

Chronic edema affecting the face and genitalia presents a particularly difficult therapeutic problem. There may be many different underlying causes, but the common denominator is usually damage to local skin and subcutaneous lymphatics.

Facial swelling can coexist with obvious primary lymphedema of one or more limbs, suggesting that there is a widespread congenitally determined lymphatic insufficiency, but more commonly the problem occurs later in life secondary to a variety of inflammatory or traumatic mechanisms (Figure 4). Facial lymphedema has been well described in association with acne and, especially, rosacea. Here the clinical findings are redness and swelling, whereas inflammatory papules/pustules may be hard to find. Treatment includes controlling the rosacea. It is hoped that by decreasing lymph load through a reduction in inflammation, edema will subside. Edema of the upper or lower lip (or both) may start with intermittent bouts of swelling resembling angiodema, but with time the condition becomes more chronic and persistent. Extension of edema within the oral cavity is common and is the explanation for the rugose changes of buccal mucosae and tongue (scrotal tongue). Biopsy may or may not reveal the presence of granulomata. If present, a diagnosis of orofacial granulomatosis (granulomatous cheilitis, Melkerson-Rosenthal syndrome) is made, but it remains unclear if the granulomata are cause or effect despite the occasional documentation of Crohn’s disease or sarcoid elsewhere. Chronic edema of the eyelids is common but may result from dermatomyositis, thyroid disease, or even yellow nail syndrome. Recurrent inflammatory pro-

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cesses (e.g., contact

allergy or angioedema) may slowly undermine lymphatic function. Equally, one severe attack of facial erysipelas or cellulitis may damage lymphatics sufficiently to induce lymphedema. Management of facial lymphedema must start with a thorough search for underlying causes and treatment of any inflammatory process. Lymphedema of external genitalia may develop from lymphatic obstruction due to advanced pelvic/ abdominal cancer or its treatment. In these circumstances, swelling of one or both legs usually coexists. In an otherwise healthy patient with edema confined to the penis, scrotum, and mons pubis, the cause is usually a primary abnormality of local lymphatics. Recurrent cellulitis is frequently associated and may antedate the swelling. Treatment includes control of infection, good scrotal support, manual lymphatic drainage therapy, and, if necessary, plastic surgery.

Conditions

Allied to Lymphedema

Chronic swelling (lymphedema) may arise in circumstances of mixed lymphovenous disease, for example, in congenital syndromes such as Klippel-Trenaunay or in the acquired postphlebitic leg. In such cases, the principles of treatment remain the same: controlling capillary filtration and improving lymph drainage. Another condition in which lymphedema occurs is in the condition “armchair legs.” This term, originally coined by Sneddon and Church,17 and otherwise known as elephantiasis verrucosis no&as, refers to the syndrome of patients who sit in a chair day and night with their legs dependent. No preexisting structural abnormalities of lymphatics exist but the immobility results in minimal lymph drainage, and a “functional lymphedema” compounded by increased capillary filtration from dependency of the limb ensues. The syndrome is not confined to legs but any chronically dependent and immobile part as demonstrated in the pendulous abdomen.” Correction of the underlying cause, namely elevation of the part at night and improved mobility, will resolve the problem.

Skin Problems in Lymphedema The characteristic skin changes that occur in lymphedema (elephantiasis) create their own particular problems. The hyperkeratosis and papillomatosis harbor microbes which produce an odor and increase the risk of infection, particularly when deep folds and crevices appear. Salicylic acid ointment, by removing scale, can help, and the application of materials such as eosin or an antiseptic paint (e.g., brilliant green), applied by a cotton bud into the cracks and folds, has proved through empiric use to be best for controlling surface colonization by bacteria and fungi. Half-strength benzoic acid ointment (Whitfield’s ointment) used prophy-

lactically will help control tinea interdigitale, an almost universal problem in lymphedema, whereas modern antifungal creams tend to increase skin maceration. Compression, either through hosiery or bandaging, will, with time, reverse many of the skin changes secondary to lymphedema. The toes may prove a particular problem area, however, because strong compression stockings have an “open toe” style that can promote, as much as help, lymphedema of these digits. Weeping of lymph from the skin surface (lymphorrhea) serves to soak dressings, clothes, and shoes, creating a source of secondary infection. Although direct leaking of interstitial fluid may occur at points of minor trauma, more often than not lymphorrhea arises from lymphangiomata. Lymphangiomata represent bulging lymphatics, like small blisters, on the skin surface and can be associated with lymphedema, whether congenital or acquired. Acquired lymphangiomata may be easily missed, such as those in the axilla following radiotherapy for breast canceri’ or in the vulva, where they can be misdiagnosed as genital warts.20 If congenital in type and therefore associated with a more widespread network of dilated lymphatics, surgical interference, unless radical, may increase the number of lymphangiomata. Acquired lymphangiomata are often single but may be grouped. Compression may be sufficient treatment, but simple cautery or laser therapy usually “seals” a leaking lesion.

Provision

of Care

The best results in lymphedema treatment are obtained with an interdisciplinary approach to care. Only by implementing all measures known to improve lymph drainage and control excessive capillary filtration will improvement be gained. Each component of treatment is designed, to complement the other. The philosophy of care is to transfer the responsibility for treatment to the patient. Once started, treatment needs to be maintained; otherwise, deterioration will soon set in. Lymphedema invariably progresses, particularly in the lower limb, unless controlled. Influences that are known to hasten this progression must be carefully avoided. Posture and limb positioning are important. Any dependent limb will tend to swell as a result of increased intravascular hydrostatic pressure. Elevation just above heart level is adequate; extreme elevation is unnecessary and probably unwise unless venous hypertension coexists. There is no evidence that the common and uncomfortable practice of hanging a postmastectomy arm overnight from a drip stand does anything to improve lymph drainage. There is some evidence that obesity exacerbates lymphedema particularly in the lower leg, and therefore a weight reduction diet may be needed. Fat deposition makes effective compression more difficult to attain. Psycho-

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logical as well as physical handicap occurs as a result of the disfigurement from lymphedema. In a recent casecontrol study comparing breast cancer patients with and without swelling, significant increases in depression and psychosocial maladjustment were found in the lymphedema group.‘l Appropriate counseling may be necessary as part of management. Lymphedema invariably requires the highestcompression-strength (> 40 mm Hg) hosiery, and double hosiery may need to be worn on occasions to maintain control. Most garments last no more than 6 months, less in a active young patient. Two garments (or pairs) should be provided, one to wear and one for the wash. Close collaboration is advised between clinician, fitter, and surgical appliance officer to ensure patient comfort, compliance, and continued improvement. Close monitoring of limb volumes is necessary for the assessment of treatment progress. Although subjective improvement is important for the patient, objective measurement of swelling must be performed if new, or even existing, regimens are to be evaluated properly and the correct hosiery fitted.

References 1. Levick JR. An introduction to cardiovascularphysiology. London: Butterworth-Heinemann, 1995;199. 2. Mortimer ES, Regnard C. Lymphostatic disorders. Br Med J 1986;1:293. 3. Price EW. Podoconiosis. Oxford: Oxford University Press, 1990. 4. Stemmer R. Ein KIinisches zeichen zur fruhund differential diagnose des lymphodems. Vasa 1976;5:261-2. 5. Proby CA, Mortimer PS, Gane J, et al. Investigation of the swollen limb by isotope lymphography Br J Dermatol 1990;123:29-37. 6. Foldi E, Foldi M, Weissleder H. Conservative treatment of lymphoedema of the limbs. Angiology 1985;36:171-80. 7. Regnard C, Badger C, Mortimer I’. Lymphoedema: ad-

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vice on management. Beaconsfield: Beaconsfield Publishers, 1991. 8. Roddie IC. Lymph transport mechanismsin peripheral lymphatics. News Physiol Sci 1990;5:85-9. 9. BatesDO, Levick JR, Mortimer PS. Subcutaneousinterstitial fluid pressure and arm volume in lymphoedema. Int J Micro&c Clin Exp 1992;11:359-73. 10. Mortimer PS. Investigation and management of lymphoedema. Vast Med Rev 1990;1:1-20. 11. Mortimer I’, Simmonds R, Rezvani M, et al. Measurement of skin lymph flow by an isotope clearance technique: Reliability, reproducibility, effect of injection dynamicsand lymph flow enhancement. J Invest Derrnatol 1990;95:677-82. 12. Stijns JJ, Leduc A. The contribution of physical therapy in the treatment of lymphedema. In: Clodius L, ed. Lymphoedema. Stuttgart: Georg Thieme; 1977:27-32. 13. Compressionfor lymphoedema. Lancet 1986;1:896. 14. Casley-Smith JR, Morgan RG, Piller NB. Treatment of lymphoedema of the arms and legs with 5,6-benzo-(x)pyrones. N Engl J Med 1993;329:1158-63. 15. Zhang T, Sheng H W-Y, Han L-Y, Liu W-Y. Heat and bandage treatment for chronic lymphoedema of extremities. Chinese Med J 1984;97:567-77. 16. Browse NL. A colour atlas of reducing operations for lymphoedema of lower limb. Ipswich: Wolfe Medical, 1986. 17. Sneddon I, Church R. Practical dermatology, 4th ed. London: Edward Arnold, 1983. 18. Bull RH, Mortimer PS. Acute lipodermatosclerosisin a pendulous abdomen. Clin Exp Dermatol 1993;18:164-6. 19. Mallett RB, Curley GK, Mortimer I%. Acquired lymphangiomata: report of four casesand a discussionof the pathogenesis.Br J Dermatol 1992;126:380-2. 20. Harwood CA, Mortimer PS. Acquired vulva1 lymphangiomata mimicking genital warts. Br J Dermatol1993;129: 334-6. 21. Tobin M, Mortimer P, Meyer L, et al. The psychological morbidity of breast cancer related arm swelling. Cancer 1993;72:3248-52.