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Elastosis perforans serpiginosa in a case of pseudoxanthoma elasticum: A rare association
Konakanchi Venkatachalam1, Kavya Chennamsetty1
1Department of Dermatology, Venereology and Leprosy, Andhra Medical College, Visakhapatnam, Andhra Pradesh, India.
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.
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