Leg ulceration with histological features of pseudoxanthoma elasticum

Abstract

Pseudoxanthoma elasticum (PXE) is an inherited connective tissue disorder with extensive degeneration and calcification of elastic tissue. Histology demonstrates calcified elastic fibers in the mid to lower dermis with a characteristic “steel wool” appearance. A limited number of published cases have described various inflammatory skin diseases presenting with PXE-like histology without clinical manifestations of PXE. Several other conditions associated with abnormal calcium metabolism have also been documented, in which coincidental histological features of PXE have been observed in patients without clinical findings. We present a case of ulceration and lipodermatosclerosis in the setting of venous insufficiency alongside histological features of PXE.

Keywords:

• Calcification
• elastic fibers
• histology
• lipodermatosclerosis
• pseudoxanthoma elasticum
• stasis
• ulceration