[Skin ulcerations due to CINCA syndrome and its successful treatment with prostaglandin E1]

Abstract

Chronic infantile neurological cutaneous and articular syndrome (CINCA) is a disorder with a defect in the CIAS1 (NLRP3) gene and the altered gene product cryopyrin leads to inflammasome activation with increased IL-1beta synthesis. The activation pathway of the transcription factor NF-κB is also affected, which plays a role in angiogenesis. With respect to the angiogenesis stimulating ability of prostaglandin E₁, we treated a female patient with CINCA syndrome and conventionally non-responsive skin ulcers with prostaglandin E₁ infusions (6 μg/kg bw/24 h/5 day) followed by wound healing lasting over 3 weeks. After 1 year of periodic infusions, the skin defects were permanently closed.

Keywords: Angiogenesis; CINCA; Prostaglandin E; Skin ulcer; Wound healing.