[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.

• Authors: C Brochhausen, M Babel, V H Schmitt, D Grevenstein, S Schreml, C Meyer-Scholten, G Klaus
• Journal: Zeitschrift fur Rheumatologie (Z Rheumatol) Vol. 77 Issue 7 Pg. 633-636 (Sep 2018) ISSN: 1435-1250 [Electronic] Germany
• Vernacular Title: Kutane Ulzerationen bei CINCA‑Syndrom und ihre erfolgreiche Behandlung durch Prostaglandin E1.
• PMID: 30066026 (Publication Type: Case Reports, Journal Article)

Chemical References

• Inflammasomes
• Interleukin-1beta
• Alprostadil

Topics

• Alprostadil (therapeutic use)
• Cryopyrin-Associated Periodic Syndromes (complications)
• Female
• Humans
• Inflammasomes
• Interleukin-1beta
• Skin Ulcer (complications, drug therapy)