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 occur...
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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 ***6 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.
Solar elastosis is a recognized manifestation of chronic sun exposure affecting mainly Caucasian skin. Recognizable clinical manifestations of solar elastosis include cutis rhomboidalis nuchae,Favre-Racouchotsyndrome,...
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Solar elastosis is a recognized manifestation of chronic sun exposure affecting mainly Caucasian skin. Recognizable clinical manifestations of solar elastosis include cutis rhomboidalis nuchae,Favre-Racouchotsyndrome,actiniccomedonalplaque, nodular forms such as elastomas, elastic nodule of the ear and elastotic bands, collagenous plaques of the hand, and colloid milia. We report two patients with an unusual presentation of vesicular lesions clinically and histopathologically situated in zones of severe solar elastosis that has been previously referred to as bullous solar elastosis.
Background: Fibulin- 5 was recently found as a secreted extracellular matrix protein that functions as a scaffold for elastic fibres. However, the distribution of fibulin- 5 in human skin and its changes during the ag...
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Background: Fibulin- 5 was recently found as a secreted extracellular matrix protein that functions as a scaffold for elastic fibres. However, the distribution of fibulin- 5 in human skin and its changes during the ageing process are not known. Objectives: To explore the involvement of fibulin- 5 in skin ageing, the age- dependent changes in fibulin- 5 localization in human skin were examined compared with those of other elastic fibre components including elastin, fibrillin- 1 and fibulin- 2. Methods: The distribution of elastin, fibrillin- 1, fibrillin- 2, fibulin- 2 and fibulin- 5 was investigated by means of immunohistochemistry using their specific antibodies. Skin samples were recovered from 12 healthy subjects undergoing plastic surgery. Ultraviolet (UV) B- irradiated or control nonirradiated buttock skin samples were obtained from two healthy volunteers at 2 days after the irradiation at 2 minimal erythemal doses. Results: In the reticular dermis of young sun- protected skin from the upper arm, fibulin- 5 colocalized with the other elastic fibre components, while in the papillary dermis fibulin- 5 showed candelabra- like structures perpendicular to the epidermiswith an unstained area just beneath the epidermis, which was similar to that of elastin but not fibrillin- 1. Fibulin- 5 in the reticular dermis decreased and disappeared with age even in sun- protected skin from the thigh, abdomen and upper arm. In sun- exposed skin, fibulin- 5 was extremely reduced in the dermis of cheek skin even from a 20- year- old man. UVB irradiation reduced fibulin- 5, fibulin- 2 and elastin markedly, moderately and weakly, respectively, compared with levels in control nontreated skin. Interestingly, the deposition of fibulin- 5 was increased in solar elastosis, like that of other elastic fibre components. Conclusions: These results suggest that fibulin- 5 is a good marker of skin ageing and that the earlier loss of fibulin- 5 may involve age- dependent changes in other elastic
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