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Isolation of Human Lymphatic Endothelial Cells by Multi-parameter Fluorescence-activated Cell Sorting

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Angioedema Presenting as Infant-Onset Primary Lymphedema.

Alekha Kolli¹, Daniel B Techabunyart¹, Craig Platt¹

¹Department of Plastic and Oral Surgery, Lymphedema Program, Boston Children's Hospital, Harvard Medical School, Boston, MA.

Journal of Vascular Anomalies

|June 18, 2026

Summary

Primary lymphedema causes chronic swelling. A case study reveals idiopathic spontaneous angioedema can mimic lymphedema, emphasizing accurate diagnosis for effective treatment.

Keywords:

angioedema idiopathic lymphedema spontaneous

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Area of Science:

Medical diagnosis
Vascular disorders
Immunology

Background:

Primary lymphedema is a chronic, progressive condition causing extremity swelling.
Differentiating lymphedema from other swelling disorders is clinically significant.
Idiopathic spontaneous angioedema presents with acute, recurrent swelling, often affecting the face and extremities.

Purpose of the Study:

To present a case of misdiagnosed primary lymphedema.
To highlight the key differences between primary lymphedema and idiopathic spontaneous angioedema.
To emphasize the importance of considering idiopathic spontaneous angioedema in the differential diagnosis of lymphedema.

Main Methods:

Case report presentation.
Review of patient's clinical presentation and diagnostic workup.
Comparison of clinical features of primary lymphedema and idiopathic spontaneous angioedema.

Main Results:

The patient presented with four-limb and facial swelling, initially suspected as primary lymphedema.
Diagnostic evaluation revealed the patient's condition was idiopathic spontaneous angioedema.
Distinct clinical characteristics were identified that differentiated the two conditions.

Conclusions:

Idiopathic spontaneous angioedema can present with symptoms mimicking primary lymphedema.
Accurate differentiation is crucial for appropriate management and patient outcomes.
Clinicians should include idiopathic spontaneous angioedema in the differential diagnosis for patients presenting with unexplained chronic swelling.