Indian Dermatology Online Journal (Jan 2016)

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

  • Konakanchi Venkatachalam,
  • Kavya Chennamsetty

DOI
https://doi.org/10.4103/2229-5178.178078
Journal volume & issue
Vol. 7, no. 2
pp. 103 – 106

Abstract

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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.

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