Purpura fulminans (PF) is a rare and fatal complication of septic shock or diffuse intravascular coagulation (DIC) resulting in skin and soft tissue necrosis. PF can be caused by congenital or acquired protein C (PC) or protein S (PS) deficiency. The most common cause of PF in a neonate is sepsis. In our extremely low birth weight preterm case, due to PF that started in the right-hand fingers, examination was made and protein S deficiency was detected as well as MTHFR (A1298C) and Factor V Leiden (R506Q) homozygous mutations. While being unresponsive to fresh frozen plasma (FFP) and unfractionated heparin (UFH) therapy, we want to highlight the curative treatment with hyperbaric oxygen (HBOT), which has not previously been used in extremely low birth weight preterm infants for this purpose.
PriceVE, LedinghamDL, KrümpelA, ChanAK. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med. 2011;16(6):318–22.
2.
FindleyT, PatelM, ChapmanJ, BrownD, DuncanAF. Acquired versus congenital neonatal purpura fulminans: A case report and literature review. J Pediatr Hematol Oncol. 2018;40(8):625–7.
3.
ZencirogluA, IpekMS, AydinM, KaraA, OkumusN, KilicM. Purpura fulminans in a newborn infant with galactosemia. Eur J Pediatr. 2010;169(7):903–6.
4.
RosenthalE, BenderlyA, Monies-ChassI, FishmanJ, LevyJ, BialikV. Hyperbaric oxygenation in peripheral ischaemic lesions in infants. Arch Dis Child. 1985;60(4):372–4.
5.
WiebersJ, PurdyI, LieberM, MilisavljevicV. Hyperbaric oxygen in treatment of neonatal arterial thromboembolism of lower extremities. J Perinatol. 2006;26(12):777–9.
6.
MillarDS, AllgroveJ, RodeckC, KakkarVV, CooperDN. A homozygous deletion/insertion mutation in the protein C (PROC) gene causing neonatal purpura fulminans: Prenatal diagnosis in an at-risk pregnancy. Blood Coagul Fibrinolysis. 1994;5(4):647–9.
7.
BarnesC, NewallF, HigginsS, CardenS, MonagleP. Perinatal management of patients at high risk of homozygous protein C deficiency. Thromb Haemost. 2002;88(2):370–1.
8.
ZencirogluA, KaragolBS, IpekMS, OkumusN, YaraliN, AydinM. Neonatal purpura fulminans secondary to group B streptococcal infection. Pediatr Hematol Oncol. 2010;27(8):620–5.
9.
NicolasL, PhilipJ, LarsonS, IslamS, LewJF, GlavinFL, et al. Severe neonatal purpura fulminans caused by staphylococcus aureus. Clin Pediatr (Phila). 2017;56(8):776–80.
10.
AlbarrakM, Al-MataryA. Neonatal purpura fulminans manifestation in early-onset group B Streptococcal infection. Am J Case Re. 2013;14, 315–7.
11.
GoodwinAJ, RosendaalFR, Kottke-MarchantK, BovillEG. A review of the technical, diagnostic, and epidemiologic considerations for protein S assays. Arch Pathol Lab Med. 2002;126(11):1349–66.
12.
KhorB, VanCott EM. Laboratory tests for protein C deficiency. Am J Hematol. 2010;85(6):440–2.
13.
LiX, LiX, LiX, ZhuangY, KangL, JuX. Genotypic and phenotypic character of Chinese neonates with congenital protein C deficiency: A case report and literature review. Thromb J. 2019;17, 19.
14.
SalonvaaraM, KuismanenK, MononenT, RiikonenP. Diagnosis and treatment of a newborn with homozygous protein C deficiency. Acta Paediatr. 2004;93(1):137–9.
15.
IrfanKazi SG, SiddiquiE, HabibI, TabassumS, AfzalB, KhanIQ. Neonatal purpura fulminans, a rare genetic disorder due to protein C deficiency: A case report. J Pak Med Assoc. 2018;68(3):463–5.
16.
KimSS, ParkIS, HongHS. Neonatal arterial thromboembolism and limb loss following respiratory distress syndrome: Case report. Arch Argent Pediatr. 2015;113(3):e157–e160.
17.
ThomSR. Hyperbaric oxygen: Its mechanisms and efficacy. Plast Reconstr Surg. 2011; 127Suppl 1(Suppl 1):131S–141S.
18.
MathieuD, MarroniA, KotJ. Tenth European Consensus Conference on hyperbaric medicine: Recommendations for accepted and non-accepted clinical indications and practice of hyperbaric oxygen treatment [published correction appears in Diving Hyperb Med. 131-132]. Diving Hyperb Med. 2017;47(1):24–32.
19.
MemarMY, YekaniM, AlizadehN, BaghiHB. Hyperbaric oxygen therapy: Antimicrobial mechanisms and clinical application for infections. Biomed Pharmacother. 2019;109, 440–7.
20.
MihaljevićS, MihaljevićL, Majerić-KoglerV, OremusK. Hyperbaric oxygenation combined with streptokinase for treatment of arterial thromboembolism of the lower leg. Wien Klin Wochenschr. 2004;116(4):140–2.
21.
PlafkiC, PetersP, AlmelingM, WelslauW, BuschR. Complications and side effects of hyperbaric oxygen therapy. Aviat Space Environ Med. 2000;71(2):119–24.
