Elastosis perforans serpiginosa in a case of pseudoxanthoma elasticum: A rare association

Abstract

Elastosis perforans serpiginosa (EPS), characterized by transepidermal elimination of fragmented elastic fibers, clinically presents as hyperkeratotic papules. EPS is classified into three types: (1) Idiopathic; (2) reactive, with associated connective tissue diseases such as pseudoxanthoma elasticum (PXE), Ehlers–Danlos syndrome, cutis laxa, Marfan syndrome, osteogenesis imperfecta, Down's syndrome; (3) the one that is induced by D-penicillamine. A rare association of EPS with PXE, which is primarily a defect of transmembrane transporter protein with accumulation of certain metabolic compounds and secondary calcification of elastic fibers has been documented in the literature. We report a case of PXE with associated lesions that were histopathologically compatible with EPS.

A Systematic Review of Case Reports of a Rare Dermatological Condition: Elastosis Perforans Serpiginosa

“…Based on the clinical and histological results, EPS was diagnosed. Histopathological analysis of EPS revealed localized or perforated epidermal hyperplasia, eosinophilic deteriorated elastic fibers, and a thin vertical transepidermal channel that included nuclear debris, granular basophilic debris, scant mononuclear inflammatory infiltrate, and bumble brush appearance of elastic fibers [3,[6][7][8][9][10][12][13][14][15]. Histological evaluation of EPS specimens is usually performed with Verhoeff-Van Gieson staining, Gomori methenamine silver staining, and periodic acid-Schiff with diastase [1].…”