title: Penicillamine-induced elastosis perforans serpiginosa with abnormal "lumpy-bumpy" elastic fibers in lesional and non-lesional skin.
abstract: Four types of elastosis perforans serpiginosa (EPS) have been described in literature: 1) idiopathic EPS, 2) reactive perforating elastosis associated with connective tissue disorders, 3) in some instances of pseudoxanthoma elasticum (PXE), disease-specific calcified elastic tissue is extruded, producing a clinical picture indistinguishable from other types, may also be seen in patients undergoing hemodialysis and 4) EPS induced by long-term treatment with D-penicillamine is observed in patients suffering from Wilson's disease. Long term D-penicillamine therapy causes an alteration in the dermal elastic tissue. D-penicillamine induced EPS has a distinctive histopathologic feature - serrated appearance of elastic fibers due to perpendicular budding from their surface giving a "lumpy-bumpy" look. D-penicillamine induced elastic fiber alteration may not always manifest clinically as EPS. We report a case of D-penicillamine induced widespread alteration in skin elastic tissue with distinct histopathologic features.
authors.affiliation: Department of Dermatology, T.N. Medical College and B.Y.L. Nair Ch. Hospital, Mumbai Central, Mumbai - 400 008, India.
authors.forename: Swapna S
authors.initials: SS
authors.lastname: Khatu
authors.forename: Rachita S
authors.initials: RS
authors.lastname: Dhurat
authors.forename: Chitra S
authors.initials: CS
authors.lastname: Nayak
authors.forename: Rickson R
authors.initials: RR
authors.lastname: Pereira
authors.forename: Rucha B
authors.initials: RB
authors.lastname: Kagne
mesh_terms: D000328:Adult; D001707:Biopsy, Needle; D004547:Elastic Tissue / Q000187:drug effects / Q000473:pathology; D005500:Follow-Up Studies; D006527:Hepatolenticular Degeneration / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D007150:Immunohistochemistry; D015552:Injections, Intralesional; D008297:Male; D010396:Penicillamine / Q000009:adverse effects* / Q000627:therapeutic use; D035583:Rare Diseases; D012720:Severity of Illness Index; D012871:Skin Diseases / Q000139:chemically induced / Q000188:drug therapy / Q000473:pathology; D016896:Treatment Outcome; D014221:Triamcinolone / Q000008:administration & dosage
chemical_list: D014221:Triamcinolone; D010396:Penicillamine