title: Development of a disease severity score for newborns with collodion membrane.
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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Section of Dermatology, Hospital for Sick Children, and University of Toronto, Toronto, Ontario, Canada.
authors.forename: Gustavo A
authors.initials: GA
authors.lastname: Rubio-Gomez
authors.affiliation: Section of Dermatology, Hospital for Sick Children, and University of Toronto, Toronto, Ontario, Canada.
authors.forename: Miriam
authors.initials: M
authors.lastname: Weinstein
authors.affiliation: Section of Dermatology, Hospital for Sick Children, and University of Toronto, Toronto, Ontario, Canada. Electronic address: elena.pope@sickkids.ca.
authors.forename: Elena
authors.initials: E
authors.lastname: Pope
mesh_terms: D015331:Cohort Studies; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D016113:Ichthyosiform Erythroderma, Congenital / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics*; D017490:Ichthyosis, Lamellar / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics*; D015994:Incidence; D007231:Infant, Newborn; D008297:Male; D009864:Ontario; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D062606:Tertiary Care Centers
keywords: collodion baby; collodion membrane; congenital ichthyosis; disorders of cornification; neonatal diseases; nonsyndromic ichthyosis; syndromic ichthyosis