Contents

SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences
Lymphedema praecox
Other Resources UpToDate PubMed

Lymphedema praecox

Contributors: Ashley Dietrich MD, 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 lymphedema is rare in children, with secondary lymphedema being far more common. Primary forms of lymphedema are categorized by age of onset. These include congenital (onset before age 1 year), lymphedema praecox (onset near puberty), and lymphedema tarda (onset after age 30).

Lymphedema praecox is the most common type of primary lymphedema. This condition primarily affects girls, and its onset at puberty or, less commonly, during pregnancy, leads to speculation that estrogen plays a role in its pathogenesis. It usually presents with unilateral involvement of a lower extremity, but involvement can be bilateral; it can also affect upper extremities. Other, more common, secondary causes of lymphedema should be ruled out.  

It is often sporadic, but it is termed Meige disease (type II) if familial. Meige disease is inherited in autosomal-dominant pattern and is associated with lymphedema-distichiasis (double row of eyelashes) syndrome related to a FOXC2 gene mutation. Familial variants may also be associated with yellow nail syndrome (autosomal dominant FOXC2 mutations) or hypotrichosis-lymphedema-telangiectasia syndrome (autosomal dominant or recessive SOX18 mutations).

Lymphedema predisposes patients to infection; thus, cellulitis and other recurrent infections are common. Lymphedema is a chronic ailment that is generally incurable. Treatment is supportive. However, prognosis is favorable with medical management.

Codes

ICD10CM:
I89.0 – Lymphedema, not elsewhere classified

SNOMEDCT:
77123007 – Lymphedema Praecox

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Primary lymphedema (strongly consider age of onset)
  • Congenital lymphedema – anatomical abnormalities, arms more commonly affected
  • Hereditary (see Congenital lymphedema)
  • History of genodermatoses (see Turner syndrome, Noonan syndrome, RASopathies)
  • Lymphedema tarda – onset after age 30
Secondary lymphedema (overall more common, but often older adults are affected). Thorough history will often elucidate a secondary cause.
  • Recurrent Cellulitis
  • Parasitic infections (ie, Lymphatic filariasis)
  • History of lymph node dissection on the side of lymphedema
  • Malignancy – obstruction of lymphatics
  • History of radiation
  • Obesity
  • Podoconiosis – exposure to mineral microparticles in volcanic soils
  • Granulomatous disease (see Sarcoidosis, Granuloma inguinale)
Other considerations (although more likely in older adults)
  • Lipedema – rare, female predilection, minimal pitting edema, elevation has no effect
  • Myxedema
  • Chronic Venous insufficiency
  • Acute Deep vein thrombosis
  • Limb hypertrophy
Cellulitis is also in the differential diagnosis of lymphedema. The swelling of lymphedema is often more striking than that of cellulitis. Erythema is a consistent feature of cellulitis, but in lymphedema, it may or may not be present. Pain is a feature of cellulitis. Lymphedema predisposes to infection, so the two conditions may exist simultaneously.

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed:02/08/2018
Last Updated:01/19/2022
Copyright © 2024 VisualDx®. All rights reserved.
Lymphedema praecox
Print  
A medical illustration showing key findings of Lymphedema praecox : Unilateral leg edema
Copyright © 2024 VisualDx®. All rights reserved.