Hereditary Lymphedema of the Leg – A Case Report

  • Birgit Heinig Center of Physical and Rehabilitative Medicine, Academic Teaching Hospital Dresden-Friedrichstadt, Friedrichstrasse 41, 01067, Dresden
  • Torello Lotti Department of Dermatology, University of Rome “G. Marconi”, Rome, Italy; Department of Biotechnology, Delft University of Technology, 2628 BC, Delft
  • Georgi Tchernev Department of Dermatology, Venereology and Dermatologic Surgery, Medical Institute of Ministry of Interior, and Onkoderma Policlinic for Dermatology and Dermatologic Surgery, Sofia
  • Uwe Wollina Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Friedrichstrasse 41, 01067, Dresden
Keywords: Primary lymphedema, Secondary lymphedema, Complex decongestive therapy, Immunocompromised district, Erysipelas, Fibrosis, Elephantiasis nostra

Abstract

Primary of hereditary lymphedema is a rare but progressive disease. It is yet not curable. We present a 48-year-old male patient with hereditary lymphedema of his left leg, that was realised by minor trauma (able twist) when he was seven years old. He had never been treated for lymphedema but experienced multiple erysipelas during his life. After diagnostic procedures to exclude other causes of leg swelling, the diagnosis of hereditary lymphedema of the leg, stage III was confirmed. We initialized complex decongestive therapy. During two weeks of intensive treatment, the circumference of the left leg could be reduced by 10 cm. This case illustrates the "natural course" hereditary lymphedema. But it raises the hope that even after decades of ignorance, the patients benefits from complex decongestive treatment. Therapeutic nihilism is unnecessary and poses lymphedema patients to risks of infection and secondary malignancies like Stewart-Trewes syndrome.

Downloads

Download data is not yet available.

Metrics

Metrics Loading ...

Plum Analytics Artifact Widget Block

References

Schook CC, Mulliken JB, Fishman SJ, Grant FD, Zurakowski D, Greene AK. Primary lymphedema: clinical features and management in 138 pediatric patients. Plast Reconstr Surg. 2011;127:2419–2431. https://doi.org/10.1097/PRS.0b013e318213a218 PMid:21617474

Soran A, Ozmen T, McGuire KP, Diego EJ, McAuliffe PF, Bonaventura M, et al. The importance of detection of subclinical lymphedema for the prevention of breast cancer-related clinical lymphedema after axillary lymph node dissection; a prospective observational study. Lymphat Res Biol. 2014;12:289-294. https://doi.org/10.1089/lrb.2014.0035 PMid:25495384

Lindqvist E, Wedin M, Fredrikson M, Kjølhede P. Lymphedema after treatment for endometrial cancer - A review of prevalence and risk factors. Eur J Obstet Gynecol Reprod Biol. 2017;211:112-121. https://doi.org/10.1016/j.ejogrb.2017.02.021 PMid:28242470

Ruocco E, Puca RV, Brunetti G, Schwartz RA, Ruocco V. Lymphedematous areas: privileged sites for tumors, infections, and immune disorders. Int J Dermatol. 2007;46:662. https://doi.org/10.1111/j.1365-4632.2007.03244.x PMid:17550576

Brouillard P, Boon L, Vikkula M. Genetics of lymphatic anomalies. J Clin Invest. 2014;124:898-904. https://doi.org/10.1172/JCI71614 PMid:24590274 PMCid:PMC3938256

Cohen MD. Complete decongestive physical therapy in a patient with secondary lymphedema due to orthopedic trauma and surgery of the lower extremity. Phys Ther. 2011;91:1618-1626. https://doi.org/10.2522/ptj.20100101 PMid:21868611

Heinig B, Wollina U. Complex decongestive therapy. Hautarzt. 2015;66:810-818. https://doi.org/10.1007/s00105-015-3674-1 PMid:26315101

Carl HM, Walia G, Bello R, Clarke-Pearson E, Hassanein AH, Cho B, Pedreira R, Sacks JM. Systematic review of the surgical treatment of extremity lymphedema. J Reconstr Microsurg. 2017; doi: 10.1055/s-0037-1599100. https://doi.org/10.1055/s-0037-1599100

Wollina U, Heinig B, Schönlebe J, Nowak A. Debulking surgery for elephantiasis nostras with large ectatic podoplanin-negative lymphatic vessels in patients with lipo-lymphedema. Eplasty. 2014;14:e11. PMid:24741382 PMCid:PMC3944717

Published
2017-07-19
How to Cite
1.
Heinig B, Lotti T, Tchernev G, Wollina U. Hereditary Lymphedema of the Leg – A Case Report. Open Access Maced J Med Sci [Internet]. 2017Jul.19 [cited 2020Oct.26];5(4):451-3. Available from: https://www.id-press.eu/mjms/article/view/oamjms.2017.082

Most read articles by the same author(s)

1 2 > >>