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Patiens with Primary Lymphoedema Followed in the Lymphology Center of the Department of Dermatology of the 2nd Medical School and Bulovka University Hospital in the Years 2000–2006


Authors: N. Vojáčková;  L. Šipulová;  R. Schmiedbergerová;  J. Hercogová
Authors‘ workplace: Dermatovenerologická klinika UK 2. LF a FN Na Bulovce přednostka prof. MUDr. Jana Hercogová CSc.
Published in: Čes-slov Derm, 83, 2008, No. 4, p. 187-190
Category: Clinical and laboratory Research

Overview

Primary lymphoedema is a chronic disease caused by abnormalities of lymphatics, mostly involving lower legs. According to the age of onset it is divided into lymphoedema congenitum, preacox and tardum. From pathophysiological point of view it can be divided into the hypoplastic and hyperplastic hereditary type (Nonne-Milroy syndrome and Meige syndrome, respectively), and idiopathic type. In diagnostics history and physical examination helps to exclude other causes of oedema, in more difficult cases lymphoscintigraphy is performed. The therapy is based on the complex decongestion therapy using combination of manual and instrumental lymphodrainage and compression bandage, in some cases surgical and pharmacological treatment is indicated. In the Lymphology Center of the Department of Dermatology of the 2nd Medical School and Bulovka University Hospital 44 patients with diagnosis of primary lymphoedema were newly treated from January 2000 to December 2006. Diagnosis was made on the basis of history, clinical picture and lymphoscintigraphy. Most patiens underwent the complex decongestion therapy in the phase of oedema reduction. Maintenance therapy is always compressive (bandage, compressive stockings or leggings of II – IV class) including home instrumental therapy in a number of patients. The therapeutical effect is asessed by a regular measurement of extremity perimeter according to the established scheme. Each patient with primary lymphoedema is regularly followed up.

Key words:
primary lymphoedema – lymphoscintigraphy – complex decongestion therapy


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Labels
Dermatology & STDs Paediatric dermatology & STDs
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