Ichthyosiform Erythroderma, Congenital, Nonbullous, 1

Alternative Names

  • NCIE1
  • IECN1
  • Ichthyosiform Erythroderma, BROCQ Congenital, Nonbullous Form
  • Nonbullous Congenital Ichthyosiform Erythroderma 1
  • NCIE
  • CIE
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WHO-ICD-10 version:2010

Congenital malformations, deformations and chromosomal abnormalities

Other congenital malformations

OMIM Number

242100

Mode of Inheritance

Autosomal recessive

Gene Map Locus

17p13.1

Description

Nonbullous congenital ichthyosiform erythroderma is an inflammatory form of ichthyosis characterized by prominent erythroderma and fine white, superficial, semiadherent scales. At birth, 90% of affected individuals present as collodion babies. Palmoplantar keratoderma, often with painful fissures, digital contractures, and loss of pulp volume are also present. 

Epidemiology in the Arab World

View Map
Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
242100.1.1LebanonMaleYes Congenital nonbullous ichthyosiform eryt...NM_001139.3:c.1294C>THomozygousAutosomal, RecessiveKurban et al, 2010
242100.2.1United Arab EmiratesFemaleYes Congenital nonbullous ichthyosiform eryt...NM_001139.3:c.944T>CHomozygousAutosomal, RecessiveBastaki et al. 2017

Other Reports

Saudi Arabia

Al-Zayir and Al-Amro, 2006, conducted a study between January 1990 and December 1995 in King Fahad hospital to document the clinical and epidemiological features of patients with primary hereditary ichthyosis (PHI).  Out of 10455 dermatology patients, a total of 71 individuals (44 males and 27 females) were diagnosed with PHI.  Nonbullous Ichthyosiform Erythroderma was diagnosed in 29.6% of these cases.  

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