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Topical Treatment of Skin Ulcers in Prolidase Deficiency
Author(s) -
Jemec Gregor B. E.,
Moe April T. T.
Publication year - 1996
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1996.tb01191.x
Subject(s) - medicine , proline , glycine , inborn error of metabolism , enzyme deficiency , enzyme , amino acid , gastroenterology , dermatology , biochemistry , chemistry
Prolidase deficiency is a hereditary enzyme deficiency characterized dermatologically by chronic recurrent ulcers and scarring due to increased skin fragility. It has been speculated that the enzyme deficiency causes a relative deficiency of proline in the wounds of these patients and negatively affects clinical healing. Two ulcers in a 17‐year‐old girl with established prolidase deficiency were treated for 12 weeks with ointments containing amino acids in an open study comparing the effects of 5% proline and a combination of 5% proline plus 5% glycine. Both ointments caused significant reduction of the ulcer size (p < 0.02), but the 5% proline‐5% glycine mixture caused a more rapid reduction (0.01 < p < 0.02). The results confirm previous findings in this rare inborn error of metabolism.