Text and Atlas of Wound Diagnosis and Treatment, 2e _ Chapter 5 Lymphedema

reabsorption or a decrease in transport capacity of the lymphatic system. It can be primary malformation of the lymph system or an acquired condition Caritas Bianchi College of Careers due to obstruction or damage to the system (TABLE 5­1).

Access Provided by:

TABLE 5­1 Primary and Secondary Lymphedema: Causes, Onset, and Characteristics

Primary

Onset

Secondary Lymphedema Causes

Onset

Inherited genetic

Variable age onset

Damage to lymph nodes and/or lymph

Any time after damage to lymph nodes and vessels:

mutations resulting in

depending upon

vessels that results in decreased lymphatic

Either soon after lymph node dissection or months,

abnormal development

the gene involved*

reabsorption and transportation

years, or decades later

(mechanical insufficiency)

Depends on patient medical history, co­morbidities

Hypoplasia

Trauma

(number of lymph nodes removed, radiation therapy,

Hyperplasia

Radiation therapy

chemotherapy, venous pathologies, and obesity), and

Aplasia

Tumor obstruction

general health

Kinmonth

Infection

syndrome (inguinal

CVI

Lymphedema Causes

of lymph vessels:

lymph node fibrosis) Genetic mutations with autosomal dominant pattern: Chromosome 5q34­q35 FLT4 FOXC2 VEGFR3 SOX18

Milroy's disease

Onset during

Surgery

Depends on patient medical history, comorbidities, and

(congenital

infancy*

Trauma

general health

lymphedema)

Radiation therapy

FOXC2

Tumor obstruction (cancer)

VEGEFR3

Infection

SOX18*

CVI

Meige's disease

Onset in childhood

(lymphedema praecox)

or around puberty;

MIM153200*

may begin in early 20s or 30s

Lymphedema tarda

Onset at 35 years or later

*Primary and secondary lymphedema differ in causes and onset, which are used to make a differential diagnosis; however, the characteristics are very similar—an

edematous limb, skin changes, loss of joint spaces. There are some characteristics of hereditary lymphedema, however, that distinguish it from secondary lymphedema. These include but are not limited to hypoparathyroidism, microcephaly, intestinal lymphangiectasia, ptosis, yellow nails, pleural effusions, cerebral arteriovenous anomalies, distichiasis, congenital heart defects, and webbing of the neck.

Data from 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;77:438–452; Levinson KL, et al. Age of onset in hereditary lymphedema. J Pediatr. 2003;142:704–708; Rizzo C. Lymphedema praecox. Derm Online J. 2009;15(8):7.

The lymphatic system is interrelated with all of the other systems of the body. Its primary roles include conducting immune system surveillance, assisting the cardiovascular system7:53 to maintain and aiding the digestive system in the breakdown of long­chain fatty acids. The immunological Downloaded 2022­3­23 A Yourfluid IP ishomeostasis, 165.84.189.140 Page 2 / 47 functions both the immediate responseRose to pathogens Chapter 5:involve Lymphedema, Marisa Perdomo; L. Hammand the long­term resistance to repeated exposure to pathogens. ©2022 McGraw Hill. All Rights Reserved. Terms of Use • Privacy Policy • Notice • Accessibility

ANATOMY OF THE LYMPH SYSTEM