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Autosomal Recessive Congenital Ichthyosis 4B

Disease ID: disease_node_19979

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DbxrefICD10CM:Q80.4, MIM:242500, ORDO:457
SubclassofDOID_0060655
Data SourceDOID
SynonymsARCI4B, harlequin ichthyosis, harlequin type ichthyosis congenita, harlequin type ichthyosis fetalis
Doid Labelautosomal recessive congenital ichthyosis 4B
Doid DescriptionAn autosomal recessive congenital ichthyosis characterized by severe neonatal ichthyosis with bilateral ectropion and eclabium, flattened and rudimentary nose and ears, constricting bands around the extremities and frequently lethality during infancy that has_material_basis_in homozygous or compound heterozygous mutation in the ABCA12 gene on chromosome 2q35.
Disease Node Iddisease_node_19979
Doid IdDOID_0060713
Disease Has Basis InHP_0001197
LabelAutosomal Recessive Congenital Ichthyosis 4B