Pseudoxanthoma elasticum‐like papillary dermal elastolysis developed in early middle age

Abstract

Pseudoxanthoma elasticum-like papillary dermal elastolysis is a rare acquired elastolytic disorder characterized by papules that resemble pseudoxanthoma elasticum, and it typically affects elderly women. Histopathological examination shows atrophic epidermis and band-like loss of elastic tissue in the papillary dermis. The pathogenesis is assumed to be related to intrinsic aging because it affects elderly people and shows the loss of elastic tissue. We report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis in early middle age presenting typical clinical and histopathological findings. The patient was a 41-year-old woman who had had her lesions for 10 years. We propose that younger patients, hitherto unknown, can be affected by this disorder and suggest that mechanisms other than intrinsic aging are involved in its pathogenesis.

Pseudoxanthoma elasticum–like papillary dermal elastolysis: A large case series with clinicopathological correlation

“…An argument often cited against intrinsic aging is that PXElike PDE affects only women rather than both sexes. 8 Electron microscopic examination of PXElike PDE has shown immature elastic fibers in the mid dermis and activated fibroblasts with dilated cisternae and rough endoplasmic reticulum as a sign of abnormal elastogenesis. 9 Tajima et al 7 found elastotic change independent of solar elastosis in the subpapillary dermis of PXE-like PDE and assumed that elastotic change may be following the initial elastolytic change reflecting the process of repairing tissue damage.…”

“…3 confirmed by our series, PXE-like PDE affects only women, after the fifth decade, although one case in early middle age has been reported. 8 Some authors explain the exclusive female predominance with the likelihood of women to seek medical care more often for cosmetic concerns. 5 The pathogenesis of PXE-like PDE is still obscure.…”

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 .…”

“…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.…”

Role of Steroid Therapy inPseudoxanthoma Elasticum-like Papillary Dermal Elastolysis

“…Intrinsic aging seems to be an attractive explanation, especially considering that PXE-PDE mostly occurs in women over 50, however a 41-year-old patient who had had her lesions for 10 years has been described (15) and although men are also subjected to intrinsic ageing, they are not affected. Moreover, an immunohistochemical study by Ohnishi et al (11) showed the absence of both elastin and fibrillin-l in the dermis of PXE-PDE patients, while in the aged control subjects only the absence of fibrillar-l was detectable and elastin was normal.…”