Pseudoxanthoma Elasticum Papillary Dermal Elastolysis: A Case Report

PXE-PDE is a rare clinicopathological entity with few cases reported. It affects more often elderly women and is characterized by asymptomatic bilateral and symmetrical yellowish papules localized predominantly on the neck and supraclavicular regions. It is clinically similar to Pseudoxanthoma Elast...

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Main Authors: Rubina Alves, Lurdes Ferreira, Esmeralda Vale, Olívia Bordalo
Format: Article
Language:English
Published: Wiley 2010-01-01
Series:Dermatology Research and Practice
Online Access:http://dx.doi.org/10.1155/2010/352724
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author Rubina Alves
Lurdes Ferreira
Esmeralda Vale
Olívia Bordalo
author_facet Rubina Alves
Lurdes Ferreira
Esmeralda Vale
Olívia Bordalo
author_sort Rubina Alves
collection DOAJ
description PXE-PDE is a rare clinicopathological entity with few cases reported. It affects more often elderly women and is characterized by asymptomatic bilateral and symmetrical yellowish papules localized predominantly on the neck and supraclavicular regions. It is clinically similar to Pseudoxanthoma Elasticum. The authors report a case of a 64-year-old woman presenting asymptomatic, yellowish, non-follicular papules, affecting the occipital and the posterior region of the neck for 1 year. The patient denied pruritic or inflammatory changes, marked solar exposition or trauma on the affected areas. Routine laboratory studies: thoracic x-ray and ophthalmologic examination were normal. The histopathologic examination of a biopsy of one of the cutaneous lesions showed an absence of elastic fibers in the papillary dermis.The diagnosis of Pseudoxanthoma Elasticum—like Papillary Dermal Elastolysis (PXE-PDE) was made. Of great importance is the differential diagnosis with Pseudoxanthoma elasticum (PXE), but we have also to consider other elastolytic disorders: mid-dermal elastolysis (MDE), linear focal elastosis (LFE) and white fibrous papulosis of the neck (WFPN). Until know, there is no effective treatment for this pathology.
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institution Kabale University
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spelling doaj-art-63fe7f61e658404a850375ab89b8ae5d2025-02-03T05:58:04ZengWileyDermatology Research and Practice1687-61051687-61132010-01-01201010.1155/2010/352724352724Pseudoxanthoma Elasticum Papillary Dermal Elastolysis: A Case ReportRubina Alves0Lurdes Ferreira1Esmeralda Vale2Olívia Bordalo3Department of Dermatology, Hospital Central of Funchal, Estrada dos Marmeleiros Monte, 9054-535 Funchal, Madeira, PortugalDepartment of Dermatology, Centro Médico-Cirúrgico de Lisboa, Rua José Estevão, 35, 1600-100 Lisboa, PortugalDepartment of Dermatology, Centro Médico-Cirúrgico de Lisboa, Rua José Estevão, 35, 1600-100 Lisboa, PortugalDepartment of Dermatology, Centro Médico-Cirúrgico de Lisboa, Rua José Estevão, 35, 1600-100 Lisboa, PortugalPXE-PDE is a rare clinicopathological entity with few cases reported. It affects more often elderly women and is characterized by asymptomatic bilateral and symmetrical yellowish papules localized predominantly on the neck and supraclavicular regions. It is clinically similar to Pseudoxanthoma Elasticum. The authors report a case of a 64-year-old woman presenting asymptomatic, yellowish, non-follicular papules, affecting the occipital and the posterior region of the neck for 1 year. The patient denied pruritic or inflammatory changes, marked solar exposition or trauma on the affected areas. Routine laboratory studies: thoracic x-ray and ophthalmologic examination were normal. The histopathologic examination of a biopsy of one of the cutaneous lesions showed an absence of elastic fibers in the papillary dermis.The diagnosis of Pseudoxanthoma Elasticum—like Papillary Dermal Elastolysis (PXE-PDE) was made. Of great importance is the differential diagnosis with Pseudoxanthoma elasticum (PXE), but we have also to consider other elastolytic disorders: mid-dermal elastolysis (MDE), linear focal elastosis (LFE) and white fibrous papulosis of the neck (WFPN). Until know, there is no effective treatment for this pathology.http://dx.doi.org/10.1155/2010/352724
spellingShingle Rubina Alves
Lurdes Ferreira
Esmeralda Vale
Olívia Bordalo
Pseudoxanthoma Elasticum Papillary Dermal Elastolysis: A Case Report
Dermatology Research and Practice
title Pseudoxanthoma Elasticum Papillary Dermal Elastolysis: A Case Report
title_full Pseudoxanthoma Elasticum Papillary Dermal Elastolysis: A Case Report
title_fullStr Pseudoxanthoma Elasticum Papillary Dermal Elastolysis: A Case Report
title_full_unstemmed Pseudoxanthoma Elasticum Papillary Dermal Elastolysis: A Case Report
title_short Pseudoxanthoma Elasticum Papillary Dermal Elastolysis: A Case Report
title_sort pseudoxanthoma elasticum papillary dermal elastolysis a case report
url http://dx.doi.org/10.1155/2010/352724
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AT lurdesferreira pseudoxanthomaelasticumpapillarydermalelastolysisacasereport
AT esmeraldavale pseudoxanthomaelasticumpapillarydermalelastolysisacasereport
AT oliviabordalo pseudoxanthomaelasticumpapillarydermalelastolysisacasereport