Post varicella disseminated intravascular coagulation and transient protein S deficiency in an otherwise healthy 6-year-old boy: a case report

Excerpt

Varicella infections are usually considered to be benign, even though varicella disease may cause serious complications. However, in Europe, the incidence of varicella infections (and complications) has been reported to peak in the spring [1]. An increased risk of complications is well known in immunocompromised individuals and in neonates, if maternal varicella has erupted close to birth. Severe complications (0.82/100,000 children/year) can also occur in previously healthy children, including central nervous system manifestations (61%), secondary bacterial infections (38%), haematological disorders (5%) and occasional fatal outcome (6%) [1, 2]. Purpura fulminans is a thrombotic disease that can occur during infections, disseminated intravascular coagulation (DIC) or in the context of an acquired or congenital protein C or S deficiency. The importance of protein S and C as two inhibitors in haemostasis is well known because severe inherited deficiency is associated with neonatal purpura fulminans and heterozygous protein S or C deficiency is associated with venous thromboembolism. …