Limb lymphedema as a first manifestation of primary intestinal lymphangiectasia (Waldmann's disease)

Journal des maladies vasculaires
V Boursier, S Vignes

Abstract

Primary intestinal lymphangiectasia (Waldmann's disease) is characterized by protein-losing enteropathy occurring more frequently in childhood. Chronic diarrhea and diffuse edema are the main clinical manifestations. Peripheral lymphedema may also be associated. Lymphedema is usually present at the time of diagnosis or appears later in the course of the disease. We report the observation of a 31-year-old man suffering from an upper, lower limb and genital lymphedema many years before diagnosis of primary intestinal lymphangiectasia was established. Lower limb lymphoscintigraphy confirmed lymphedema and duodenal biopsies lymphangiectasia. Hypoproteinemia, lymphopenia and hypogammaglobulinemia were also noted. Treatment of lymphedema included low stretch bandaging and elastic stocking. No dietary management with a low-fat diet was added. Search for primary intestinal lymphangiectasia with biological parameters would be useful when primary lymphedema is present. Especially since primary intestinal lymphangiectasia may be complicated by occurrence of B cell lymphoma.

Citations

Feb 26, 2008·Orphanet Journal of Rare Diseases·Stéphane Vignes, Jérôme Bellanger
Mar 4, 2010·Digestive Diseases and Sciences·Jie WenWei Cai
Dec 29, 2006·Contrast Media & Molecular Imaging·Tristan BarrettHisataka Kobayashi
Dec 22, 2009·Pathophysiology : the Official Journal of the International Society for Pathophysiology·J S AlexanderMarlys H Witte

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