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Proteosome-Associated Autoinflammatory Syndrome 5

Disease ID: disease_node_15595

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DbxrefMIM:619175
SubclassofDOID_0050737, DOID_0060913
Data SourceDOID
SynonymsPRAAS5
Doid Labelproteosome-associated autoinflammatory syndrome 5
Doid DescriptionA proteasome-associated autoinflammatory syndrome that is characterized by recurrent, polymorphic disseminated cutaneous rash with annular lesions, non-specific lymphocytic infiltration, fever, failure to thrive, persistent hepatosplenomegaly, emaciated face, long slender fingers, levated acute-phase reactants and microcytic anemia and that has_material_basis_in homozygous mutation in the PSMB10 gene on chromosome 16q22.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_15595
Doid IdDOID_0060919
LabelProteosome-Associated Autoinflammatory Syndrome 5