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Massive vulvar edema revealing idiopathic systemic capillary leak syndrome


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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This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

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NB and SZ: drafted the manuscript. All authors revised the final manuscript.

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Correspondence to Souheil Zayet.

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Belfeki, N., Postic, G., Hadji, N.A. et al. Massive vulvar edema revealing idiopathic systemic capillary leak syndrome. Arch Gynecol Obstet 306, 1795–1797 (2022).

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  • Idiopathic systemic capillary leak syndrome
  • Intravenous immunoglobulins
  • Shock
  • Critical care
  • Vulvar edema
  • Pregnancy