PA08 Extensive congenital epidermal skin loss with sparing of the palms, soles and head: strikingly similar appearances in four patients with neonatal herpes simplex infection

We present a case series of four preterm neonates from two tertiary centres in the UK. All presented at birth with 90% epidermal skin loss. In all four neonates, there was a strikingly similar presentation, with skin spared on the palms, soles, and areas on either the face or scalp. Three of the neonates tested positive for herpes simplex virus (HSV) type 1 and one tested positive for HSV type 2 on skin swabs taken soon after birth. None of the mothers gave a history of genital HSV infection. In the past, this pattern of skin loss has been attributed to severe congenital erosive and vesicular dermatosis, with a differential diagnosis of aplasia cutis or epidermolysis bullosa. HSV has been recognized in a few cases but there is limited literature available discussing widespread skin loss secondary to HSV infection. In utero HSV is rare in neonates and accounts for 5% of all neonatal herpes infections. Intrauterine HSV has an increased risk of preterm labour and has been reported to occur in asymptomatic mothers with no history of genital herpes. HSV contracted in utero commonly presents with cutaneous lesions in the context of an unwell neonate and carries a high risk of eye involvement, mouth disease and neurological complications such as encephalitis, meningitis and neurodisability. Babies with this suspected diagnosis need urgent skin swabs for viral polymerase chain reaction and bacterial culture. Early treatment with high-dose intravenous antivirals prior to swab results is essential. Full neonatal intensive care support is needed, as well as analgesia, gentle handling and antibiotics. Neonates are best managed in a tertiary neonatal intensive care setting with input from the national epidermolysis bullosa team, due to the extensive nature of the skin loss. Two of our cases healed with severe reticulated scarring and contractures, requiring multiple procedures and extensive physiotherapy. The other two did not survive the neonatal period despite early diagnosis and a multidisciplinary approach. Management of HSV in future pregnancies must be discussed with the obstetric team. This is the first case series describing a characteristic pattern of severe skin loss among neonates with congenital HSV. We recommend early recognition and empirical treatment of HSV, while swabs are awaited.