Angiopathic Pathogenesis of Clinical Manifestations in Prolidase Deficiency

Abstract

To the Editor.— We examined mild initial cutaneous lesions in a prolidase-deficient girl by light and electron microscopy. Her brain was examined by magnetic resonance imaging and electroencephalography. Report of a Case.— The patient, now 19 years old, is the younger of the two prolidase-deficient sisters we reported in 1979. 1 At that time she was free of clinical manifestations, although her prolidase activity was as low as that in her elder sister, with full-blown clinical signs. She has been developing crops of annular erythematous macules with marginal scales on her thighs and buttocks (Fig 1), fine purpuric lesions, reticulate erythema, and atrophies blanches or small ulcers with a radial violaceous halo on her lower legs (Fig 2). The ulcers healed readily with topical application of 5% Fig 1.— Specimen from annular erythema showed thickened capillary wall and perivascular edema in deep dermis, and thrombosis with red blood cells and