Common symptoms of SCPCD

Purpura fulminans is a rapidly-progressing thrombotic disorder involving haemorrhagic infarction of the skin and disseminated intravascular coagulation.² At first, skin lessions appear dark red and subsequently become purple-black and indurated.³ In SCPCD, these lesions can develop on the lower limbs, male genitalia as well as pressure points, such as the back of the head, buttocks, and heels.^1,2 The image on the left shows a PF lesion on the left hip of a male newborn.

Disseminated Intravascular Coagulation (DIC) is an acquired syndrome characterised by the intravascular activation of coagulation with a loss of localisation arising from different causes. It can both originate from and cause damage to the microvasculature, which, if sufficiently severe, can produce organ dysfunction.⁴
The image on the left shows the typical clinical features of DIC.⁵

Blindness is a common manifestation of severe PC deficiency and may arise from vitreal bleeding, retinal vein, artery or vitreal vein thrombosis with retinal detachment manifesting as leucocoria or ischaemic optic atrophy.² Ophthalmic lesions may occurs before or after birth.⁶

Large vessel venous thrombosis may also occur, e.g. , renal vein thrombosis.³

Affected neonates also commonly show significant neurological injuries resulting from antenatal or early postnatal cerebral venous thrombosis with secondary peri-ventricular haemorrhagic infarction and hydrocephalus.²