2 Epidemiological, Clinical, and Pathophysiological Aspects

Summary

Lymphedema is an inefficiency of the lymphatic system which leads to, clinically, swelling and fibroadipose tissue deformation. Primary lymphedema ensues from a disorder during the development of the lymphatic system. This genetic mutation could be inheritable or occur sporadically. There is a separate group known as lymphatic malformations. Secondary lymphedema constitutes the main reason of lymphedema. Worldwide, most frequently, it is caused by filarial nematodes infection. In high-income countries lymphedema is primarily due to surgical interventions like lymphadenectomy, oncological therapies, and trauma. Prevalence and incidence of primary and secondary lymphedema are discussed in this chapter. The pathophysiological changes of lymphedema are caused by the hyaluronan-rich interstitial fluid accumulation in the interstitium. This activates a complex inflammatory response. Another pathological process in late-stage lymphedema is adipose tissue deposition. Recurrent infections of the lymphedema-affected extremity are a common side effect of lymphedema. This often indicates severity of lymphedema. Long-term tissue changes are thickening of the cutis and subcutis due to accumulation of fatty tissue, and development of fibrosis, lymphatic cysts, and fistulae. Trophic changes in the epidermis are variable. Hyperplasia, hyperkeratosis, hyperpigmentation, minor papillomatosis, and verrucous protuberances may appear.

Key words

etiology of lymphedema - lymphatic malformations - prevalence and incidence - pathophysiology

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