POMP-related autoinflammation and immune dysregulation disease
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.
M04.9 – Autoinflammatory syndrome, unspecified
724834006 – Neutrophilic dermatosis
Differential Diagnosis & Pitfalls