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Recessive Dystrophic Epidermolysis Bullosa

Disease ID: disease_node_14232

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DbxrefICD10CM:Q81.2, MIM:226600, ORDO:79408
SubclassofDOID_4959, DOID_0050737
Data SourceDOID
SynonymsRDEB, Hallopeau-Siemens type, autosomal recessive dystrophic epidermolysis bullosa generalisata gravis, autosomal recessive dystrophic epidermolysis bullosa, Hallopeau-Siemens type, severe generalized RDEB, severe generalized recessive dystrophic epidermolysis bullosa
Doid Labelrecessive dystrophic epidermolysis bullosa
Doid DescriptionAn epidermolysis bullosa dystrophica characterized by recurrent blistering at the level of the sublamina densa beneath the cutaneous basement membrane, it has_material_basis_in homozygous or compound heterozygous mutation in the gene encoding type VII collagen (COL7A1) on chromosome 3p21.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_14232
Doid IdDOID_0060642
LabelRecessive Dystrophic Epidermolysis Bullosa