POMP-related autoinflammation and immune dysregulation disease
Alerts and Notices
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
ICD10CM:M04.9 – Autoinflammatory syndrome, unspecified
SNOMEDCT:
724834006 – Neutrophilic dermatosis
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Chronic neutrophilic dermatosis, lipodystrophy, and elevated temperature (CANDLE) syndrome
- Severe combined immunodeficiency disease (SCID)
- Keratosis linearis with ichthyosis congenita and sclerosing keratoderma (KLICK) syndrome
- Sweet syndrome
- Nakajo-Nishimura syndrome
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Management Pearls
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Therapy
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References
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Last Reviewed:08/14/2022
Last Updated:08/15/2022
Last Updated:08/15/2022