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      Elastosis Perforans Serpiginosa

      case-report

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          Abstract

          Elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination of abnormal elastic fibers. This condition classically presents as small papules arranged in serpiginous or annular patterns on the neck, face, arms, or other flexural areas. While these lesions may spontaneously resolve, they often persist for longer periods of time. Though numerous treatment modalities have been described in the literature, most are not very effective. A 35-year-old man presented to Gangnam Severance Hospital with a 2-year history of skin eruptions on his neck, which were refractory to topical antifungal or steroid treatment. On examination, the patient showed multiple crusted and erythematous papules arranged in an annular pattern with central clearing. A biopsy specimen revealed acanthosis with notable transepidermal elimination of nuclear debris and eosinophilic degenerated elastic fibers from the dermis via an epidermal channel. Verhoeff-van Gieson staining showed dense clumps of altered elastic fibers in the papillary dermis. Based on these findings, a diagnosis of elastosis perforans serpisinosa was made. Treatment with topical 0.05% tretinoin application for 6 months resulted in no improvement.

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          Most cited references20

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          Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes.

          Elastic fibers in the extracellular matrix are an integral component of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin may be attributed to the network of elastic tissue. Advances in our understanding of elastic tissue physiology provide a foundation for studying the pathogenesis of elastic tissue disorders. Many acquired disorders are nevertheless poorly understood due to the paucity of reported cases. Several acquired disorders in which accumulation or elastotic degeneration of dermal elastic fibers produces prominent clinical and histopathologic features have recently been described. They include elastoderma, linear focal elastosis, and late-onset focal dermal elastosis and must be differentiated from better-known disorders, among them acquired pseudoxanthoma elasticum, elastosis perforans serpiginosa, and Favré-Racouchot syndrome. Learning objective At the conclusion of this learning activity, participants should understand the similarities and differences between acquired disorders of elastic tissue that are characterized by an increase in elastic tissue, as well as the spectrum of solar elastotic dermatoses.
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            Elastosis perforans serpiginosa and associated disorders.

            We report elastosis perforans serpiginosa (EPS) arising in three patients with Ehlers-Danlos syndrome, osteogenesis imperfecta and Down's syndrome. These cases illustrate some of the rare but well-recognized disease associations with EPS. The other causes of EPS are reviewed.
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              Elastosis perforans serpiginosa Associated with Pseudo-Pseudoxanthoma elasticum during Treatment of Wilson’s Disease with Penicillamine

              Background: Elastosis perforans serpiginosa (EPS) is a reactive perforating dermatosis characterized by the elimination of abnormal elastic fibers from the upper 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 described in 1972 in a patient treated for Wilson’s disease. Subsequently, cutaneous changes resembling pseudoxanthoma elasticum (PXE) were observed in patients treated with DPA and were reported as pseudo-PXE. Case Report: We report herein the clinical, pathological and ultrastructural study of 2 new cases of DPA-induced EPS and pseudo-PXE. These patients had been treated for Wilson’s disease since 14 and 16 years, respectively. Characteristic abnormal elastic fibers were found on histopathological examination of both EPS and pseudo-PXE skin and confirmed by an ultrastructural study. There was no ABCC6 mutation. Discussion: Penicillamine is able to induce widespread, cutaneous and systemic, elastic fiber damage. Our patients present typical features of DPA-induced elastosis, presenting as EPS and pseudo-PXE. ABCC6 mutation is associated with PXE and, as expected, it was absent in our cases of pseudo-PXE. This elastopathy has been related to morphologic changes in elastic fibers secondary to prolonged therapy in most cases. DPA may interfere with elastin cross-linking through inhibition of the enzyme lysyl oxidase, or by formation of complexes with the cross-linked precursors, impairing a normal maturation of elastic fibers. However, no fatal complication of DPA-induced elastopathy has been reported so far. An improvement of the cutaneous lesions is expected after the drug discontinuation.
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                Author and article information

                Journal
                Ann Dermatol
                Ann Dermatol
                AD
                Annals of Dermatology
                Korean Dermatological Association; The Korean Society for Investigative Dermatology
                1013-9087
                2005-3894
                February 2014
                17 February 2014
                : 26
                : 1
                : 103-106
                Affiliations
                Department of Dermatology, Gangnam Severance Hospital, The Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.
                Author notes
                Corresponding author: Soo-Chan Kim, Department of Dermatology, Gangnam Severance Hospital, The Cutaneous Biology Research Institute, Yonsei University College of Medicine, 211 Eonju-ro, Gangnam-gu, Seoul 135-720, Korea. Tel: 82-2-2019-3362, Fax: 82-2-3463-6136, kimsc@ 123456yuhs.ac
                Article
                10.5021/ad.2014.26.1.103
                3956773
                24648695
                018bdc3c-d8a7-475d-b523-a4a884305d61
                Copyright © 2014 The Korean Dermatological Association and The Korean Society for Investigative Dermatology

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 13 July 2010
                : 26 September 2011
                : 21 October 2011
                Categories
                Case Report

                Dermatology
                elastosis perforans serpiginosa,therapeutics
                Dermatology
                elastosis perforans serpiginosa, therapeutics

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