BPSU Study - Results of a national research study of newborn babies with collodion membrane.

International guidelines for babies born with collodion membrane (CM) or harlequin ichthyosis (HI) recommend intensive care in a high-humidity incubator. By contrast, at Birmingham Children’s Hospital, we successfully nurse such babies in an open cot, encouraging parental involvement. The guidelines are based on expert consensus with little evidence. So, we investigated this using the British Paediatric Surveillance Unit (BPSU) e-reporting scheme to identify all babies born in the UK and Ireland with CM or HI during a 2-year period 1 November 2018 to 31 October 2020.

There were no new babies with HI but 31 with CM, of whom 26 had autosomal recessive congenital ichthyosis (ARCI). We calculated the birth incidence of CM as 2·05 per 100,000 live births and for ARCI as 1.72 per 100,000 live births, higher than the usual estimate of 1 per 200,000 live births. Interestingly the CM resolved by 12 months in 40% of babies– the usual quote for self-improving CM is 10-20%. Our higher figures reflect our prospective approach: all affected babies in the population were identified at birth and followed up. Most studies are retrospective and will miss the CMs which resolve very quickly.

The 5 non-ARCI babies with CM had additional skin or medical problems; two sadly died in their first year. The 26 ARCI babies all survived and thrived: they had good birth weights (average 3.01kg = 6lb 10oz) despite shortened gestation (average 38 weeks). Their condition measured as APGAR scores at 1 and 5 minutes was 9/10 or 10/10 in all cases. Despite this 76% were kept in an incubator for an average of 9 days, 59% had intravenous antibiotics and 10 were tube-fed. All had emollients.

We conclude that the outlook for otherwise healthy babies born with uncomplicated, typical CM is very good and recommend conservative management. Paediatricians naturally treat this unfamiliar condition intensively, but medical interventions increase the risk of hospital-acquired infections and impede normal parent-baby interactions.

ISG members with babies born during the study period may be surprised that they were not told about the study. In order to get a true picture of all cases we applied, successfully, for permission from the Confidentiality Advisory Group, under Section 251 of the National Health Service Act 2006, to collect anonymised medical data without patient/parent consent (similar to the process used for government health statistics). The research team did not see patient names, photographs or addresses and worked within tight security constraints.

Link to study website: www.rcpch.ac.uk/work-we-do/bpsu/study-congenital-ichthyosis  Link to full report: https://academic.oup.com/bjd/article/188/1/139/6795461

Celia Moss – Professor of Dermatology .