PAPASH Syndrome: The first case report from Syria

PAPASH syndrome is a rare autoinflammatory consisting of four essential components: pyogenic arthritis (PA), pyoderma gangrenosum (PG), acne (A), and suppurative hidradenitis (SH). The true etiology unknown, yet genetic analysis has shown associations with PSTPIP1 mutations, which ultimately lead to elevations in interleukin-1 activity. Herein, we report case painful ulcers 47-year-old Syrian male located on the legs swelling both ankles, bad condition hyperthermia. He also had history acne, hidradenitis, fistulas, abscesses buttock area. results skin biopsy, laboratory testing, joint aspiration, poor responding antibiotic treatment excluded infective nature case. To our knowledge, this was first diagnosed Syria. Key words: syndrome; Pyoderma gangrenosum; Hidradenitis suppurativa; Hereditary diseases; Acne