Department of Rehabilitation Medicine, Rehabilitation Hospital, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Gyeongsangnam-do, Republic of Korea.
Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Gyeongsangnam-do, Republic of Korea.
Medicine (Baltimore). 2023 Jul 7;102(27):e34214. doi: 10.1097/MD.0000000000034214.
Primary lymphedema is a form of lymphedema marked by a dysfunction of the lymphatic system without preceding medical conditions. One rare subtype of primary lymphedema, lymphedema tarda, occurs in those older than 35 years and is difficult to diagnose. This paper reports 2 cases of unilateral lymphedema tarda in the lower extremities in South Korea.
The 2 patients complained of worsening swelling in the lower extremity for several months without any direct surgical or traumatic history related to the inguinal or lower extremity lymphatic system.
Primary lymphedema tarda may be determined by ultrasonography. Other vascular or infection-origin causes were excluded from further evaluations.
To confirm primary lymphedema tarda, lymphangiography was performed. In each case, lower extremity lymphangiography indicated dermal backflow and no lymph node uptake at the inguinal node of the affected side, which was compatible with lymphedema.
The patients reported slight improvement in the symptoms after several weeks of rehabilitation.
This paper is the first report of the unilateral primary lymphedema tarda in South Korea. Further investigations are warranted to find the related etiology of this rare disease and a multimodality regimen is needed for improvement of symptoms.
原发性淋巴水肿是一种以淋巴系统功能障碍为特征的淋巴水肿形式，无先前的医疗条件。原发性淋巴水肿的一种罕见亚型——迟发性淋巴水肿，发生在 35 岁以上的人群中，且难以诊断。本文报告了韩国 2 例单侧下肢迟发性淋巴水肿病例。
2 名患者抱怨下肢肿胀数月，且无任何与腹股沟或下肢淋巴系统直接相关的手术或创伤史。
原发性迟发性淋巴水肿可通过超声检查确定。进一步评估排除了其他血管或感染性病因。
为了确认原发性迟发性淋巴水肿，进行了淋巴造影术。在每个病例中，下肢淋巴造影显示真皮逆流，受影响侧腹股沟淋巴结无淋巴结摄取，这与淋巴水肿相符。
患者在康复数周后报告症状略有改善。
本文是韩国首例单侧原发性迟发性淋巴水肿的报告。需要进一步研究以找到这种罕见疾病的相关病因，并需要采用多模式治疗方案来改善症状。
