Pash syndrome M35.8

Acronym for a rare clinical triad consisting of:

• Pyoderma gangraenosum
• acne and
• hidradenitis suppurativa,

whose entity still appears questionable. The clinical picture belongs to the group of autoinflammatory syndromes and must be distinguished from the autosomal dominant inherited PAPA syndrome (pyogenic arthritis, acne conglonbata, pyoderma gangraenosum). The genetic defect in PASH syndrome has not yet been clearly identified. Associations with NOD2, PSTIP1, IL1RN, MEFV and NLRP3 are suspected.

Dapsone, cyclosporine, interleukin-1 blockers, infliximab, adalimumab

1. Braun-Falco M et al (2010) Pyoderma gangrenosum, Acne, and suppurative hidradenitis (PASH)--a new autoinflammatory syndrome distinct from PAPA syndrome. J Am Acad Dermatol 66:409-415
2. Marzano AV et al. (2012) Pyoderma gangrenosum, acne and suppurative hidradenitis syndrome following bowel bypass surgery. Dermatology 225:215-219
3. Menis D et al (2014) Two cases of severe Hidradenitis Suppurativa with failure of anakinra therapy. Br J Dermatol doi: 10.1111/bjd.13292.