Dramatic improvement of pyoderma gangrenosum with infliximab in a patient with PAPA syndrome

Dorothee S. Stichweh, Marilynn Punaro, Virginia Pascual

Research output: Contribution to journalArticle

93 Scopus citations


Infliximab, a chimeric antitumor necrosis factor alpha monoclonal antibody (anti-TNF α), has been recently shown to have a beneficial effect on pyoderma gangrenosum associated with inflammatory bowel disease. Patients with the syndromic triad of pyogenic sterile arthritis, pyoderma gangrenosum, and acne, an autoinflammatory process caused by mutations in the CD2 binding protein-1 (CD2BP1) gene, can have severe pyoderma gangrenosum. We describe a 14-year-old patient with this syndrome who was unresponsive to multiple therapies. A dramatic improvement in his pyoderma gangrenosum was observed after one infusion of infliximab, and a second infusion led to its resolution. Our observation extends the therapeutic use of infliximab to this component of PAPA syndrome.

Original languageEnglish (US)
Pages (from-to)262-265
Number of pages4
JournalPediatric Dermatology
Issue number3
StatePublished - May 1 2005


ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Dermatology

Cite this