Abstract
Hematopoietic stem cell transplantation has been used as an effective therapy for selected autosomal disease providing patients prolonged long-term survival and optimizing quality of life. PAPA syndrome is an autosomal dominant disease derived from the mutations in the PSTPIPI gene. Furthermore, the PSTPIPI gene is expressed in hematopoietic cells and encodes a protein that binds to pyrin and controls the production of interleukin-1. The main clinical manifestations of PAPA syndrome are pyogenic sterile arthritis, pyoderma gangrenosum and cystic acne.
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