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Background:Elastosis perforans serpiginosa (EPS) is a reactive perforating de rmatosis characterized by the elimination of abnormal elastic fibers from the up per dermis through the epidermis. In a few cases, it occurs as a side effect of treatment by D-penicillamine (DPA). The first case of EPS induced by DPA was de scribed in 1972 in a patient treated for Wilson’s disease. Subsequently, cutane ous changes resembling pseudoxanthoma elasticum(PXE)wereobservedinpatientstreate dwithDPAand were reported as pseudo-PXE. Case Report:We report herein the clinical, pathological and ultrastructural study of 2 new cases of DPA-induce d EPS and pseudo-PXE. These patients had been treated for Wilson’s disease sin ce 14 and 16 years, respectively. Characteristic abnormal elastic fibers were fo und on histopathological examination of both EPS and pseudo-PXE skin and confir med by an ultrastructural study. There was no ABCC6 mutation. Discussion:Penici llamine is able to induce widespread, cutaneous and systemic, elastic fiber dama ge. Our patients present typical features of DPA-induced elastosis, presentinga s EPS and pseudo-PXE.ABCC6 mutation is associated with PXEand,asexpected,it was absent in our cases of pseudo-PXE. This elastopathy has been related to morpho logic changes in elastic fibers secondary to prolonged therapy in most cases. DP A may interfere with elastin cross-linking through inhibition of the enzyme lys yl oxidase, or by formation of complexes with the cross-linked precursors, impa iring a normal maturation of elastic fibers. However, no fatal complication of D PA-induced elastopathy has been reported so far. An improvement of the cutaneou s lesions is expected after the drug discontinuation.
Background: Elastosis perforans serpiginosa (EPS) is a reactive perforating de rmatosis characterized by the elimination of abnormal elastic fibers from the up per dermis through the epidermis. In a few cases, it occurs as a side effect of treatment by D-penicillamine (DPA The first case of EPS induced by DPA was decribed in 1972 in a patient treated for Wilson’s disease. Case Report: We report herein the Clinical, pathological and ultrastructural study of 2 new cases of DPA-induce d EPS and pseudo-PXE. These patients had been treated for Wilson’s disease sin ce 14 and 16 years, respectively. Characteristic abnormal elastic fibers were fo und on histopathological examination of both EPS and pseudo-PXE skin and confir med by an ultrastructural study. There was no ABCC6 mutation. Discussion: Penici llamine is able to induce widespread, cutaneous Our systemic presentational features of DPA-induced elastosis, presentinga s EPS and pseudo-PXE. has related to morpho logic changes in elastic fibers secondary to prolonged therapy in most cases. DP A may interfere with elastin cross-linking through inhibition of the enzyme lys yl oxidase, or by formation of complexes with the cross-linked precursors, impa iring a However, no fatal complication of D PA-induced elastopathy has been reported so far. An improvement of the cutaneou s lesions is expected after the drug discontinuation.