Archives of Disease in Childhood - Fetal and Neonatal Edition | 2021
Nicolau syndrome or ‘embolia cutis medicamentosa’ in a newborn: successful treatment with a surgical intervention
Abstract
A 3dayold male newborn was referred to a quaternary hospital with pain, pallor and cyanosis of the left lower limb for the past 2 days of admission. These signs and symptoms started immediately after intramuscular injection of 50 000 IU of benzathine penicillin G at the lateral face of his left thigh to treat possible congenital syphilis. After diagnosis of acute compartment syndrome secondary to Nicolau syndrome (NS) (figure 1A), the assistant team immediately performed a fasciotomy of the left lower limb (figure 1B). After the procedure, unfractionated heparin, prostaglandin and antibiotics were administered, and the patient was discharged after 22 days of hospitalisation with complete pain resolution and adequate peripheral perfusion and movements. Neither amputation nor skin debridement was needed up to 60 days of followup (figure 2). NS, ‘livedoid dermatitis’ or ‘embolia cutis medicamentosa’ was first described almost 100 years ago. However, even now, its aetiology and best treatments are still under debate. NS is a rare and severe condition related to the intramuscular, subcutaneous or intraarticular injection of a wide range of drugs, and after hyaluronic acid soft tissue fillers. Patients with NS present hyperaemia, skin discolouration, livedoid dermatitis and a haemorrhagic patch on the injection site. Only a few NS cases have been reported to date, and less than five were reported in newborns. Although there is no specific treatment for NS, early diagnosis, recognition and additional surgical interventions such as fasciotomy should also be considered for limb preservation when treating NS.