POMP-related autoinflammation and immune dysregulation disease
Synopsis
POMP expression is particularly important for the function of immune cells because without protein degradation, antigens cannot be presented on major histocompatibility complex (MHC) class I receptors.
Individuals with PRAID are susceptible to a multitude of viral, bacterial, and fungal infections, including opportunistic organisms such as Pneumocystis jirovecii and mycobacteria, as a result of dysregulated immune functioning.
Codes
M04.9 – Autoinflammatory syndrome, unspecified
SNOMEDCT:
724834006 – Neutrophilic dermatosis
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Last Updated:08/15/2022
