Reumatologia: pesquisas atuais
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Generalization of Pyogenic Arthritis, Pyoderma Gangrenosum and Acne (PAPA)

Fatemah Baroun

PAPA syndrome (Pyogenic Arthritis Pyoderma gangrenosum) is an autoinflammatory disorder, a hereditary ailment caused due to changes in mutations within the PSTPIP1/CD2BP1 gene on a chromosome. These mutations produce a hyper-phosphorylated PSTPIP1 protein and modify its involvement inactivation of the “inflammasomes” effect in interleukin-1 (IL-1β) production. PA syndrome is an acronym for Pyogenic Arthritis, Pyoderma gangrenous, and pimples. It is a rare genetic autoinflammatory disease characterized by effects on pores, skin, and joints. It is also referred to as PAPGA syndrome (Pyogenic Arthritis Pyoderma Gangrenosum and acne).PAPA syndrome is hereditary autosomal dominant style, this shows a 50% threat that a toddler will inherit the disease from an affected determine. It generally starts with arthritis at a younger age, with the pores and skin changes greater outstanding from the time of puberty.