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CASE REPORT
Year : 2016  |  Volume : 7  |  Issue : 2  |  Page : 103-106

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


Department of Dermatology, Venereology and Leprosy, Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

Correspondence Address:
Dr. Kavya Chennamsetty
Plot No. 46, Pandurangapuram, Visakhapatnam, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.178078

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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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