Autosomal recessive prolidase deficiency. Three patients with recalcitrant ulcers

Abstract

Three patients had prolidase deficiencies. The family pedigree of these 3 patients suggests that this rare disorder is inheritied through an autosomal recessive gene. This genodermatosis is characterized by signs and symptoms of the skin, CNS, teeth, ears, nose, throat, eyes, bones and joints. Among the skin changes, recalcitrant leg ulcers are the most characteristic. There is no established method of treatment of this rare disorder, but the use of dapsone was helpful in the treatment of 1 patient.