Lymphedema Pathophysiology and clinical manifestations

Ayman A. Grada, MD, and Tania J. Phillips, MD Boston, Massachusetts. J Am Acad Dermatol 2017;77:1009-20

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Lymphedema Pathophysiology and clinical manifestations

Ayman A. Grada, MD, and Tania J. Phillips, MD Boston, Massachusetts. J Am Acad Dermatol 2017;77:1009-20

Lymphedema is a localized form of tissue swelling resulting from excessive retention of lymphatic fluid in the interstitial compartment and caused by impaired lymphatic drainage. Lymphedema is classified as primary or secondary. Primary lymphedema is caused by developmental lymphatic vascular anomalies. Secondary lymphedema is acquired and arises as a result of an underlying systemic disease, trauma, or surgery. We performed PubMed and Google Scholar searches of the English-language literature (1966-2017) using the terms lymphedema, cancer-related lymphedema, and lymphatic complications. Relevant publications were manually reviewed for additional resources. This progressive chronic disease has serious implications on patients’ quality of life. It is often misdiagnosed because it mimics other conditions of extremity swelling. There is no definitive cure for lymphedema. However, with proper diagnosis and management, its progression and potential complications may be limited.

Main findings

This article provides an overview of the pathophysiology and clinical manifestations of lymphoedema and useful for someone new to the area. It provides key points for each section. These include:

Pathophysiology:

  • Lymphedema is caused by impaired lymphatic drainage in the presence of normal capillary

Function.

  • Lymphatic congestion induces chronic inflammation, which leads to fibrosis and further lymphatic damage.
  • Primary lymphedema is caused by genetic mutation or developmental abnormalities.
  • Filariasis is the most common cause of secondary (acquired) lymphedema worldwide.
  • Cancer-related treatment is the most common cause of secondary lymphedema in the United. States

Epidemiology:

  • Lymphedema is more common in females than males.
  • Lower extremity lymphedema is much more common than upper extremity lymphedema.

Clinical manifestations:

  • Early-stage lymphedema mimics other causes of tissue swelling, such as venous edema.
  • Chronic lymphedema is characterized by skin thickening and tissue fibrosis.
  • Elephantiasis verrucosa nostras is a severe manifestation of chronic lymphedema.
  • It is important to rule out venous edema, lipedema, obesity, and drug-induced swelling before making a diagnosis.

Potential complications:

  • Chronic limb swelling causes discomfort and functional impairment.
  • Recurrent bacterial and fungal infections are common.
  • Recurrent cellulitis and lymphangitis contribute to further lymphatic damage.
  • Ulceration is common and can be difficult to treat.
  • Cutaneous angiosarcoma is a rare and lethal complication; look for red-purple discoloration.
  • Serious psychosocial implications because of cosmetic impairment and activity restriction.