PSEUDOXANTHOMA ELASTICUM-LIKE PAPILLARY DERMAL ELASTOLYSIS - A SUBDIAGNOSIS ENTITY

  • Leonor Lopes Interno da Formação Específica em Dermatovenereologia/Resident, Dermatology and Venereology, Serviço de Dermatovenereologia, Centro Hospitalar de Lisboa Norte – Hospital de Santa Maria, Lisboa, Portugal
  • Miguel Duarte Reis Assistente Hospitalar de Dermatovenereologia/Consultant, Dermatology and Venereology, Serviço de Dermatovenereologia, Centro Hospitalar de Lisboa Norte – Hospital de Santa Maria, Lisboa, Portugal
  • Ana Isabel Gouveia Interno da Formação Específica em Dermatovenereologia/Resident, Dermatology and Venereology, Serviço de Dermatovenereologia, Centro Hospitalar de Lisboa Norte – Hospital de Santa Maria, Lisboa, Portugal
  • João Pedro Vasconcelos Interno da Formação Específica em Dermatovenereologia/Resident, Dermatology and Venereology, Serviço de Dermatovenereologia, Centro Hospitalar de Lisboa Norte – Hospital de Santa Maria, Lisboa, Portugal
  • Luís Soares Almeida Professor Doutor do Serviço de Dermatologia e Venereologia/Professor of Dermatology and Venereology, Serviço de Dermatovenereologia, Centro Hospitalar de Lisboa Norte – Hospital de Santa Maria, Lisboa, Portugal
Keywords: Dermis, Elastic tissue, Pseudoxanthoma elasticum, Skin diseases

Abstract

Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is an acquired clinical entity, of unknown etiology, characterized by partial or total absence of elastic fibers in the papillary dermis. We describe the case of a 62-year-old Caucasian woman, Fitzpatrick III skin phototype, with a dermatosis characterized by multiple, asymptomatic, yellowish, monomorphic, rounded papules, with 2-3mm in diameter, located on the posterior and lateral sides of the neck with 2 years of evolution. Histopathological examination revealed a marked reduction of elastic fibres in the papillary dermis confirming PXE-PDE diagnosis. We consider that this entity, exclusively cutaneous, is under-diagnosed because it is an asymptomatic disease, in which histopathological diagnosis is difficult, if it is not clinically considered.

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References

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Published
2014-09-19
How to Cite
Lopes, L., Reis, M. D., Gouveia, A. I., Vasconcelos, J. P., & Almeida, L. S. (2014). PSEUDOXANTHOMA ELASTICUM-LIKE PAPILLARY DERMAL ELASTOLYSIS - A SUBDIAGNOSIS ENTITY. Journal of the Portuguese Society of Dermatology and Venereology, 72(2), 223-226. https://doi.org/10.29021/spdv.72.2.259
Section
Case Reports