April 1987

Prolidase Deficiency in Two Siblings With Chronic Leg UlcerationsClinical, Biochemical, and Morphologic Aspects

Author Affiliations

From the Department of Dermatology, Regional Hospital of Vicenza (Drs Leoni and Bertolini); the Department of Biochemistry, University of Pavia (Drs Cetta, Tenni, and Castellani and Ms Dyne); and the Institute of General Pathology, University of Modena (Drs Pasquali-Ronchetti and Guerra), Italy.

Arch Dermatol. 1987;123(4):493-499. doi:10.1001/archderm.1987.01660280095032

• Prolidase deficiency occurred in two sisters suffering from recurrent leg ulcers that appeared in early childhood. The patients presented the typical clinical symptoms of the disease, including characteristic facies, dermatologic manifestations of the lower extremities, splenomegaly, and hematologic anomalies. Large amounts of iminodipeptides were excreted into the urine, and prolidase activity in their erythrocytes was virtually absent. Changes associated with a connective-tissue disorder were demonstrated by light and electron microscopic studies of the patients' apparently normal skin. Collagen fibers were smaller than in controls and were irregularly packed; the fibrils had normal aspect but were significantly smaller than in one age-matched control. Elastin fibers appeared altered both in size and structure.

(Arch Dermatol 1987;123:493-499)