Development of a disease severity score for newborns with collodion membrane

J Am Acad Dermatol. 2014 Mar;70(3):506-11. doi: 10.1016/j.jaad.2013.11.002. Epub 2013 Dec 24.

Abstract

Background: Collodion membrane in the neonate may be the initial presentation of a number of different conditions. There is a lack of data correlating the extent of clinical involvement to the underlying disease and prognosis.

Objective: We sought to identify features predictive of the final outcome and complications in a cohort of patients with collodion membrane, using a disease severity score.

Methods: This was a retrospective cohort study of newborns with collodion membrane at a tertiary care institution over a period of 31 years. We designed and applied a 0- to 15-point severity score and correlated the results with the final diagnoses and complications. Data on demographics, membrane shedding, and treatment were collected.

Results: We identified 29 cases. Congenital ichthyosiform erythroderma and lamellar ichthyosis were the most common final diagnoses with 7 of 29 cases (24%) each; 3 patients were given the diagnosis of a syndromic ichthyosis. The classic nonsyndromic ichthyoses had higher average score results (7.33) than the syndromic ichthyoses (2.0) and other presentations (4.0), (P = .0097). Patients with major complications had higher, but nonsignificant, average severity scores (P = .64).

Limitations: The retrospective design and small number of patients with a syndromic ichthyosis are limitations.

Conclusions: Prospective studies are required to validate the proposed disease severity score.

Keywords: collodion baby; collodion membrane; congenital ichthyosis; disorders of cornification; neonatal diseases; nonsyndromic ichthyosis; syndromic ichthyosis.

Publication types

  • Comparative Study

MeSH terms

  • Cohort Studies
  • Female
  • Genetic Predisposition to Disease*
  • Humans
  • Ichthyosiform Erythroderma, Congenital / diagnosis*
  • Ichthyosiform Erythroderma, Congenital / epidemiology
  • Ichthyosiform Erythroderma, Congenital / genetics*
  • Ichthyosis, Lamellar / diagnosis*
  • Ichthyosis, Lamellar / epidemiology
  • Ichthyosis, Lamellar / genetics*
  • Incidence
  • Infant, Newborn
  • Male
  • Ontario
  • Prognosis
  • Retrospective Studies
  • Risk Assessment
  • Severity of Illness Index
  • Tertiary Care Centers