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A case report of RASA1-associated inherited lymphoedema with recurrent life-threatening lymphangitis.


ABSTRACT:

Background

Most cases of lymphoedema are secondary to other causes, while cases of primary lymphoedema, in particular that of congenital origin, are uncommon. Limited genetic disorders are so far known to be associated with lymphatic malformation including mutations in RASA1. This clinical case highlights the possible complications of RASA1-associated lymphatic malformation in a female suffering from recurrent life-threatening septic lymphangitis.

Case summary

A 23-year-old female patient presented with congenital lymphoedema of the lower right extremity. At the age of eight, she first suffered from an episode of lymphangitis. Thereafter, she developed recurrent episodes of lymphangitis predominately occurring during menstruation and culminating into severe and

SUBMITTER: Westphal DS 

PROVIDER: S-EPMC8633724 | biostudies-literature | 2021 Nov

REPOSITORIES: biostudies-literature