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Lymphedema in Adult
See also in: Cellulitis DDx
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Lymphedema in Adult

See also in: Cellulitis DDx
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Contributors: Vivian Wong MD, PhD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Lymphedema refers to the abnormal accumulation of protein-rich interstitial fluid secondary to lymphatic dysfunction. It may be primary, referring to a developmental abnormality of the lymphatic system, or secondary (acquired). Primary forms of lymphedema include congenital (Milroy disease), lymphedema praecox (onset in teenage years), and lymphedema tarda (onset after age 30).

Secondary lymphedema is usually the result of filariasis, compression of lymphatic structures by a neoplasm, or destruction by surgery, radiation, or infection. Secondary lymphedema is typically diagnosed between 50 and 58 years of age.

In the United States and other developed nations, lymphedema most commonly occurs following breast cancer treatment. Major risk factors include axillary lymph-node dissection and adjuvant radiation therapy.

Lymphedema often presents clinically as painless swelling of an extremity that may grow over time to elephantiasic size. A lower extremity is the most common location. Elephantiasis nostras verrucosa refers to a rare group of cutaneous changes comprising dermal fibrosis and hyperkeratotic, verrucous, and papillomatous lesions arising after chronic secondary (nonfilarial) lymphedema.

Many types of lymphedema are more common in women.

Lymphedema is a chronic ailment that is generally incurable. Treatment is supportive. Lymphedematous limbs are often complicated by infection (eg, cellulitis, onychomycosis) and have considerable impact on quality of life (ie, impede daily activities, create negative self-esteem). The incidence of cellulitis in children with primary lymphedema begins early in life, with a median age of 11.5 years at first episode.

Various syndromes are associated with primary lymphedema such as Turner syndrome, Noonan syndrome, and yellow nail syndrome.

Codes

ICD10CM:
I89.0 – Lymphedema, not elsewhere classified

SNOMEDCT:
234097001 – Lymphedema

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Underlying causes of chronic lymphedema:
Other causes of edema must be ruled out:

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 12/14/2017
Last Updated: 12/05/2018
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Lymphedema in Adult
See also in: Cellulitis DDx
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Lymphedema : Hand edema, Lower extremity edema, Foot edema, Upper extremity edema
Clinical image of Lymphedema
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