What is the relationship between acne conglobata (AC) and PAPA syndrome?

Updated: Apr 24, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Answer

Pyogenic arthritis, pyoderma gangrenosum, and acne conglobata are clinically distinct inflammatory disorders that may be seen rarely in the same patient in a syndrome known as PAPA Syndrome. It was originally reported in a 3-generation kindred with autosomal dominant transmission The PAPA syndrome is related to the triad of pyoderma gangrenosum, acne conglobata, and suppurative hidradenitis, known as the PASH syndrome. [2] The simultaneous presence of pyoderma gangrenosum, acne conglobata, suppurative hidradenitis, and seronegative spondyloarthritis has been suggested as a new linkage with the designation being PASS syndrome. [3] Micali and Lacarrubba adroitly observed that secondary comedones in acne conglobata may correlate with double-ended pseudocomedones in hidradenitis suppurativa. [4]


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