Prolidase deficiency: its dermatological manifestations and some additional biochemical studies

Abstract

Prolidase deficiency occurred in a 13 yr old girl. Determinations were made of prolidase and prolinase activities in cultured fibroblasts, and TLC studies of skin prolidase were performed. The patient had chronic, recurrent ulcers on the legs and feet, diffuse telangiectasia, shallow scarlike atrophic lesions on the face and arms, soft and thin abdominal skin, and premature gray hairs. Prolidase in the patint''s skin fibroblasts was absent. Greatly reduced prolidase activity was demonstrated in the patient''s skin. A review of hitherto reported cases of this disease showed such skin manifestations as skin ulceration or skin fragility with scar formation, purpuric lesions, telangiectasia and/or photosensitivity, and thickening of the skin with lymphedema.