PSEUDOXANTHOMA ELASTICUM-LIKE PAPILLARY DERMAL ELASTOLYSIS - A SUBDIAGNOSIS ENTITY

• Autor(a) principal: Lopes, Leonor
• Data de Publicação: 2014
• Outros Autores: Reis, Miguel Duarte, Gouveia, Ana Isabel, Vasconcelos, João Pedro, Almeida, Luís Soares
• Tipo de documento: Artigo
• Idioma: por
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: https://doi.org/10.29021/spdv.72.2.259
• topic: Dermis; Elastic tissue; Pseudoxanthoma elasticum; Skin diseases; Derme; Doenças da pele; Pseudoxantoma elástico; Tecido elástico

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.