1. [Skin ulcerations due to CINCA syndrome and its successful treatment with prostaglandin E 1 ].
- Author
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Brochhausen C, Babel M, Schmitt VH, Grevenstein D, Schreml S, Meyer-Scholten C, and Klaus G
- Subjects
- Female, Humans, Inflammasomes, Interleukin-1beta, Alprostadil therapeutic use, Cryopyrin-Associated Periodic Syndromes complications, Skin Ulcer complications, Skin Ulcer drug therapy
- 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 E
1 , 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.- Published
- 2018
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