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Dermatology Research and Practice
Volume 2010, Article ID 352724, 4 pages
http://dx.doi.org/10.1155/2010/352724
Case Report

Pseudoxanthoma Elasticum Papillary Dermal Elastolysis: A Case Report

1Department of Dermatology, Hospital Central of Funchal, Estrada dos Marmeleiros Monte, 9054-535 Funchal, Madeira, Portugal
2Department of Dermatology, Centro Médico-Cirúrgico de Lisboa, Rua José Estevão, 35, 1600-100 Lisboa, Portugal

Received 22 May 2010; Accepted 11 July 2010

Academic Editor: Franco Rongioletti

Copyright © 2010 Rubina Alves et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

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.