Congenital sclerema neonatorum associated with chronic fetal compromise and acute peripartum deterioration

Abstract Sclerema neonatorum (SN) is a rare and frequently fatal panniculitis of critically ill neonates characterized by diffuse woody induration of the skin and subcutaneous tissue. SN is a rare and severe form of panniculitis, affecting critically ill neonates. SN presents as woody hardening of the neonatal skin and subcutaneous tissues and is considered a late-onset postnatal disorder. An increasing number of case reports describe the presence of skin induration and contractures at birth, which suggests an etiology before birth. Due to the low number of reported cases, this Perspective seeks to increase awareness and prompt further focused studies. The purpose of this Perspective is to present chronic fetal compromise and superimposed acute peripartum deterioration as an antenatal-postnatal continuum and to propose congenital SN as one possible presentation along this spectrum. The literature is limited to unrelated case reports and small case series. A causative relationship has not yet been established. This Perspective presents clinically reported cases, fetal surveillance, placenta evaluation, and neonatal assessment to construct a plausible relationship between chronic fetal hypoxia and congenital SN. Early-onset and strictly congenital presentations of SN should be differentiated since their respective etiologies may be interrelated but distinct. Fetal Movement Observation, Amsterdam Placental Workshop Group Consensus criteria placenta review, a pathology review and perinatal multidisciplinary assessment are recommended. This Perspective outlines the speculative association of SN as a disease of congenital origin. It is hoped that this will increase diagnostic focus, stimulate placental evaluation and research in this largely unexplored area.