Hereditary Lymphedema of the Leg – A Case Report
Identifieur interne : 002358 ( Pmc/Curation ); précédent : 002357; suivant : 002359Hereditary Lymphedema of the Leg – A Case Report
Auteurs : Birgit Heinig ; Torello Lotti ; Georgi Tchernev ; Uwe WollinaSource :
- Open Access Macedonian Journal of Medical Sciences [ 1857-9655 ] ; 2017.
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.
Url:
DOI: 10.3889/oamjms.2017.082
PubMed: 28785330
PubMed Central: 5535655
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<front><div type="abstract" xml:lang="en"><p>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.</p>
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<front><journal-meta><journal-id journal-id-type="nlm-ta">Open Access Maced J Med Sci</journal-id>
<journal-id journal-id-type="iso-abbrev">Open Access Maced J Med Sci</journal-id>
<journal-title-group><journal-title>Open Access Macedonian Journal of Medical Sciences</journal-title>
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<article-id pub-id-type="doi">10.3889/oamjms.2017.082</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>Case Report</subject>
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<title-group><article-title>Hereditary Lymphedema of the Leg – A Case Report</article-title>
</title-group>
<contrib-group><contrib contrib-type="author"><name><surname>Heinig</surname>
<given-names>Birgit</given-names>
</name>
<xref ref-type="aff" rid="aff1">1</xref>
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<contrib contrib-type="author"><name><surname>Lotti</surname>
<given-names>Torello</given-names>
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<xref ref-type="aff" rid="aff2">2</xref>
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<contrib contrib-type="author"><name><surname>Tchernev</surname>
<given-names>Georgi</given-names>
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<xref ref-type="aff" rid="aff3">3</xref>
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<contrib contrib-type="author"><name><surname>Wollina</surname>
<given-names>Uwe</given-names>
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<xref ref-type="aff" rid="aff4">4</xref>
<xref ref-type="corresp" rid="cor1">*</xref>
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<aff id="aff1"><label>1</label>
<italic>Center of Physical and Rehabilitative Medicine, Academic Teaching Hospital Dresden-Friedrichstadt, Friedrichstrasse 41, 01067, Dresden, Germany</italic>
</aff>
<aff id="aff2"><label>2</label>
<italic>Department of Dermatology, University of Rome “G. Marconi”, Rome, Italy; Department of Biotechnology, Delft University of Technology, 2628 BC, Delft, The Netherlands</italic>
</aff>
<aff id="aff3"><label>3</label>
<italic>Department of Dermatology, Venereology and Dermatologic Surgery, Medical Institute of Ministry of Interior, and Onkoderma Policlinic for Dermatology and Dermatologic Surgery, Sofia, Bulgaria</italic>
</aff>
<aff id="aff4"><label>4</label>
<italic>Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Friedrichstrasse 41, 01067, Dresden, Germany</italic>
</aff>
<author-notes><corresp id="cor1"><label>*</label>
<bold>Correspondence:</bold>
Uwe Wollina. Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Friedrichstrasse 41, 01067, Dresden, Germany. E-mail: <email xlink:href="uwollina@googlemail.com">uwollina@googlemail.com</email>
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<pub-date pub-type="collection"><day>25</day>
<month>7</month>
<year>2017</year>
</pub-date>
<pub-date pub-type="epub"><day>19</day>
<month>7</month>
<year>2017</year>
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<volume>5</volume>
<issue>4</issue>
<fpage>451</fpage>
<lpage>453</lpage>
<history><date date-type="received"><day>25</day>
<month>5</month>
<year>2017</year>
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<date date-type="rev-recd"><day>21</day>
<month>4</month>
<year>2017</year>
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<date date-type="accepted"><day>26</day>
<month>4</month>
<year>2017</year>
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<permissions><copyright-statement>Copyright: © 2017 Birgit Heinig, Torello Lotti, Georgi Tchernev, Uwe Wollina.</copyright-statement>
<copyright-year>2017</copyright-year>
<license license-type="open-access" xlink:href="http://creativecommons.org/licenses/CC BY-NC/4.0"><license-p>This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0).</license-p>
</license>
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<abstract><p>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.</p>
</abstract>
<kwd-group><kwd>Primary lymphedema</kwd>
<kwd>Secondary lymphedema</kwd>
<kwd>Complex decongestive therapy</kwd>
<kwd>Immunocompromised district</kwd>
<kwd>Erysipelas</kwd>
<kwd>Fibrosis</kwd>
<kwd>Elephantiasis nostra</kwd>
</kwd-group>
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