Topical Treatment of Skin Ulcers in Prolidase Deficiency

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Abstract
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.