title: [Neonatal purpura fulminans without sepsis due to a severe congenital protein C deficiency].
abstract: Severe congenital protein C deficiency is a rare life-threatening coagulopathy. In the early hours of life, the neonate presents with extensive purpura fulminans and substantial skin necrosis contrasting with a preserved general state and a negative infectious exam. Disseminated intravascular coagulation sets in secondarily. Prenatal outset of thrombotic events is a rare situation that worsens the prognosis, especially protein C replacement in utero is not available. We report a case of a male newborn of consanguineous parents who were asymptomatic carriers of heterozygous protein C deficiency. This infant presented prenatal ventricular hemorrhage with hydrocephalus and rapidly extensive postnatal skin necrosis that was not regressive in spite of fresh frozen plasma administrated after 24h of life. Prenatal diagnosis, early recognition, and urgent therapy with protein C replacement and anticoagulant treatment are crucial to improve the prognosis, avoid further damage after delivery, and prevent the devastating consequences of severe protein C deficiency.
authors.affiliation: Service de néonatologie et réanimation néonatale, CHU Hassan II, 30000 Fès, Maroc; Faculté de médecine et de pharmacie, université Sidi-Mohammed-Ben-Abdellah, 30000 Fès, Maroc. Electronic address: fhmami@hotmail.com.
authors.forename: F
authors.initials: F
authors.lastname: Hmami
authors.affiliation: Service de chirurgie pédiatrique, CHU Hassan II, 30000 Fès, Maroc; Faculté de médecine et de pharmacie, université Sidi-Mohammed-Ben-Abdellah, 30000 Fès, Maroc.
authors.forename: H
authors.initials: H
authors.lastname: Cherrabi
authors.affiliation: Service de néonatologie et réanimation néonatale, CHU Hassan II, 30000 Fès, Maroc; Faculté de médecine et de pharmacie, université Sidi-Mohammed-Ben-Abdellah, 30000 Fès, Maroc.
authors.forename: A
authors.initials: A
authors.lastname: Oulmaati
authors.affiliation: Service de chirurgie pédiatrique, CHU Hassan II, 30000 Fès, Maroc; Faculté de médecine et de pharmacie, université Sidi-Mohammed-Ben-Abdellah, 30000 Fès, Maroc.
authors.forename: Y
authors.initials: Y
authors.lastname: Bouabdallah
authors.affiliation: Service de néonatologie et réanimation néonatale, CHU Hassan II, 30000 Fès, Maroc; Faculté de médecine et de pharmacie, université Sidi-Mohammed-Ben-Abdellah, 30000 Fès, Maroc.
authors.forename: A
authors.initials: A
authors.lastname: Bouharrou
mesh_terms: D003241:Consanguinity; D004211:Disseminated Intravascular Coagulation / Q000209:etiology; D017809:Fatal Outcome; D006801:Humans; D006849:Hydrocephalus / Q000150:complications; D007231:Infant, Newborn; D008297:Male; D020151:Protein C Deficiency / Q000150:complications* / Q000151:congenital; D055665:Purpura Fulminans / Q000209:etiology*; D012720:Severity of Illness Index