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

• Autores: Angelo V. Marzano, Valentina Trevisan
• Localización: JAMA Dermatology, ISSN 2168-6068, Vol. 149, Nº. 6, 2013, págs. 762-764
• Idioma: inglés
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• Resumen

  • 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.