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June 2013

Pyogenic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa (PAPASH): A New Autoinflammatory Syndrome Associated With a Novel Mutation of the PSTPIP1 Gene

Author Affiliations

Author Affiliations: U. O. Dermatologia, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti Universitá degli Studi di Milano, Milan, Italy (Drs Marzano, Trevisan, and Crosti); Pediatria II, Istituto Giannina Gaslini, Genoa, Italy (Dr Gattorno); Laboratorio di Genetica Molecolare, Istituto Giannina Gaslini, Genoa (Dr Ceccherini); and Dipartimento di Dermatologia, Universitá Cattolica del Sacro Cuore, Rome, Italy (Dr De Simone).

JAMA Dermatol. 2013;149(6):762-764. doi:10.1001/jamadermatol.2013.2907

The PASH triad (pyoderma gangrenosum, acne, and hidradenitis suppurativa) has recently been described in 2 unrelated patients as a new entity within the spectrum of autoinflammatory syndromes.1,2 PASH syndrome is similar to PAPA syndrome (pyogenic arthritis, acne, and pyoderma gangrenosum) but differs insofar as it lacks the associated arthritis and has a different genetic basis.3 PAPA syndrome is caused by mutations in the proline-serine-threonine-phosphatase protein 1 (PSTPIP1) gene (OMIM: 606347) that is involved in regulating innate immune responses, whereas no mutations have yet been detected in PASH syndrome.