Role of Steroid Therapy inPseudoxanthoma Elasticum-like Papillary Dermal Elastolysis

Abstract

Pseudoxanthoma Elasticum-Iike Papillary Dermal Elastolysis (PXE-PDE) is a peculiar idiopathic elastolytic disorder that clinically resembles Psudoxanthoma elasticum (PXE). It is histologically characterized by a total or partial loss of elastic fibers in the papillary dermis. It more often affects elderly women and is characterized by asymptomatic and symmetrical yellowish papules localized predominantly on the neck, supraclavicular regions, and flexural areas. After analyzing a series of cases and the recent literature suggesting that glucocorticoids may down-regulate the elastin gene expression and elastin mRNA, in cultured human skin fibroblasts, we think that high dose and prolonged steroid therapy may contribute to the appearance of PXE-PDE lesions.

Two cases of periumbilical perforating pseudoxanthoma elasticum with dermoscopic and histopathologic features

“…Pseudoxanthoma elasticum‐like papillary dermal elastolysis is a rare idiopathic disorder affecting elderly women . Asymptomatic yellowish papules are situated symmetrically on the flexures, neck and supraclavicular areas and abdomen where it may resemble PXE. Dermoscopy reveals linear vessels and white‐yellow papules .…”

Pseudoxanthoma Elasticum–Like Papillary Dermal Elastolysis: A Single Case Report

“…Approximately 40 cases of PXE-PDE have been reported in the literature. 1-9 PXE-PDE, an uncommon acquired skin disorder, exists on a spectrum of fibroelastolytic disease of the skin that includes variants such as mid-dermal elastolysis, upper dermal elastolysis, and white fibrous papulosis of the neck. A degree of clinical and histologic overlapping exists between the variants, making histopathology essential for diagnosis.…”

“…Reports of PXE-PDE developing during therapy with methylprednisolone or triamcinolone for rheumatologic conditions highlight the potential effect of glucocorticoids in down-regulating the elastin gene expression and elastin messenger ribonucleic acid. 7,8…”

Pseudoxanthoma Elasticum–like Papillary Dermal Elastolysis

“…1,5,8 Another hypothesis holds that PXE-PDE is the result of abnormal elastic fiber formation, given the observation of immature fibers in the upper reticular dermis or evidence of activated fibroblastomas. 1,5 Pranteda et al 9 demonstrated that glucocorticoids may be involved in the pathogenesis of PXE-PDE, given their ability to reduce the expression of the elastin gene and its mRNA in human fibroblast cultures. 9 Even though PXE-PDE is an acquired condition, the case of 2 sisters 10 and the absence of cases in males suggests a genetic, hereditary, or hormonal factor that has not yet been identified.…”

“…1,5 Pranteda et al 9 demonstrated that glucocorticoids may be involved in the pathogenesis of PXE-PDE, given their ability to reduce the expression of the elastin gene and its mRNA in human fibroblast cultures. 9 Even though PXE-PDE is an acquired condition, the case of 2 sisters 10 and the absence of cases in males suggests a genetic, hereditary, or hormonal factor that has not yet been identified.…”