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Hz/Hc can be distinguished from PAPA syndrome based on clinical symptoms and laboratory parameters. A, Characteristic clinical symptoms and laboratory parameters present or absent in patients with Hz/Hc and those with PAPA syndrome. B-I, Palpebral lesions (Fig 1, B), pyoderma gangrenosum–like ulcers (Fig 1, C) or ulcerative dermatitis (Fig 1, D and E), and MRP14 expression in affected (Fig 1, F and G) and unaffected (Fig 1, H and I) skin of patient 1.
