Original articlePyoderma gangrenosum, acne, and suppurative hidradenitis (PASH)–a new autoinflammatory syndrome distinct from PAPA syndrome
Section snippets
Case 1
A 34-year-old man (172 cm; 92 kg) of Russian descent had severe cystic acne lesions on his face and back since adolescence, and from clusters of chronic and repeatedly draining sinuses and abscesses in both axillae for 7 years (Fig 1, A to D). During the last 6 months, slowly expanding and painful ulcerations developed on his arms and lower legs. His medical history was otherwise unremarkable in particular for any kind of arthritis, inflammatory bowel disease, or hematologic malignancy; his
Discussion
PAPA syndrome is clinically characterized by pyogenic sterile arthritis, PG, and acne, and has been defined as an autosomal dominant autoinflammatory syndrome caused by mutations in the PSTPIP1 gene. Herein, we present two unrelated patients with a symptom complex very close to, but clearly distinct from, PAPA syndrome. They share the dermatologic signs of PG and acne with PAPA syndrome, but in distinction to PAPA lack the intensive joint inflammation and instead experience severe SH at large
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Funding sources: None.
Conflicts of interest: None declared.