Successful combined antibiotic therapy with oral clindamycin and oral rifampicin for pyoderma gangrenosum in patient with PASH syndrome.

摘要: Pyoderma gangrenosum (PG) is a neutrophilic dermatitis, the prevalence of which unknown. The only reported incidence in literature estimates that there are 3 to 10 cases per million annually United Kingdom.1 association with digestive tract and inflammatory rheumatic disease, neoplasia, endocrinopathies well known.2 New autoinflammatory syndromes PG have been described: PAPA syndrome, combining pyogenic sterile arthritis cystic acne; PASH acne hidradenitis suppurativa (HS); PAPASH arthritis, acne, HS.3 PAPA arise from mutations coding region proline-serine-threonine-phosphatase interacting protein 1 gene (PSTPIP1) resulting loss inhibitory effect on NALP3 inflammasome production interleukin (IL)-1β. For known anomaly an increase number CCTG repetitions PSTPIP1 promoter, no functional impact.4 First-line treatment for generally based systemic corticosteroid therapy or antibiotics an anti-inflammatory action (eg, dapsone tetracyclines) immunosuppressive drugs azathioprine, cyclosporine, mycophenolate mofetil). Also introduced recently anti–tumor necrosis factor-α agents, anti–IL-1 (anakinra), finally anti–IL-12–IL-23 (ustekinumab) anti–IL-17 (ixekizumab). Studies investigating syndrome found cyclosporine biotherapy using factor-α5 (anakinra)4 effective. clinical course is, however, marked by risk repeated relapses resistance conventional treatments PG. Furthermore, any proposed therapeutic strategy should be effective against all entities (ie, PG, HS, acne).