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Published on: December 9, 2022
Massive vulvar edema revealing idiopathic systemic capillary leak syndrome
Nabil Belfeki1, Gabrielle Postic1, Nabil Abdelouahab Hadji2
1Department of Internal Medicine, Groupe Hospitalier Sud Ile de France, 77000, Melun, France.
Abstract:
Idiopathic systemic capillary leak syndrome is a rare disease characterized by recurrent episodes of hypotension, hypoalbuminemia and peripheral edema caused by capillary hyperpermeability with approximately 350 documented cases worldwide. We report herein the case of a 22-year-old primiparous patient with an unusual compartment syndrome represented with spontaneous massive vulvar edema. Treatment consisted of volume replacement and intravenous polyvalent immunoglobulins perfusions. Vulvar edema was treated surgically by incision and drainage with favorable outcome. The pregnancy follow-up did not show any fetal abnormalities. The childbirth at 35 weeks was natural and the newborn was healthy.
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