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PAPASH syndrome
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PAPASH syndrome

Contributors: Boya Abudu MD, MPH, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

PAPASH syndrome is a rare genetic autoinflammatory condition consisting of the following tetrad: pyogenic arthritis, pyoderma gangrenosum (PG), acne vulgaris, and hidradenitis suppurativa (HS). It is thought to be due to a mutation in the PSTPIP1 gene that affects the innate immune system.

PAPASH should be distinguished from the following PG syndromes: PAPA (pyogenic arthritis, PG, and acne), PASH (PG, acne, and HS), and PsAPASH (psoriatic arthritis, PG, acne, and HS). These rare inflammatory syndromes are thought to share a common mechanism of interleukin (IL)‐1‐driven episodes of neutrophil‐predominated sterile inflammation.

Codes

ICD10CM:
M04.9 – Autoinflammatory syndrome, unspecified

SNOMEDCT:
363138005 – Hereditary disorder of immune system

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Last Reviewed:06/12/2019
Last Updated:08/13/2019
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PAPASH syndrome
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PAPASH syndrome
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