Pseudoxanthoma Elasticum Papillary Dermal Elastolysis: A Case Report

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.

Elastic fibres in health and disease

“…Papillary dermal elastolysis is a rare condition, similar to PXE, which usually affects elderly women, and is associated with elastolytic papules on the neck and supraclavicular sites (Ref. 179).…”

Pseudoxanthoma elasticum-like papillary dermal elastolysis

“…PKE'nin aksine bu hastalıkta sistemik tutulum yoktur. Bilinen etkili bir tedavisi yoktur [1][2][3][4][5][6][7][8] . Topikal tretinoinin etkisiz olduğunu gösteren rapor bulunmaktadır 14 .…”

“…Topikal tretinoinin etkisiz olduğunu gösteren rapor bulunmaktadır 14 . Güneşten korunma önerilebilir [2][3][4]8 .…”

“…Klinik olarak çok sayıda genellikle asemptomatik sarı veya deri renginde yumuşak, non-foliküler kaldırım taşına benzer papüllerle karakterizedir. Etiyopatogenezi tam olarak bilinmemekle beraber ultraviyole hasarı, intrinsik yaşlanma ve elastik doku yıkımı ile ilişkili olabileceği düşünülmektedir [1][2][3][4][5][6][7][8] . Biz burada 60 yaşında PKE-PDE tanısı olan kadın hastamızı hastalığın oldukça nadir görülmesi nedeniyle sunuyoruz.…”

Early‐onset pseudoxanthoma elasticum‐like papillary dermal elastolysis

“…The aetiopathogenetical factors considered are intrinsic skin ageing, ultraviolet radiation and genetics. [1][2][3] A 31-year-old female presented with asymptomatic skin changes on the neck that have been developed during the first years of life. On examination, there was a yellowish cobblestone-appearing plaque predominantly at the left side of her neck ( Fig.…”