Dermatology Clinic, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy.
Dermatology Clinic, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy.
Clin Dermatol. 2021 Mar-Apr;39(2):206-210. doi: 10.1016/j.clindermatol.2020.10.018. Epub 2020 Oct 16.
Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis (PDE) is an acquired, rare, elastic tissue disorder that presents with multiple asymptomatic, nonfollicular yellowish or normochromic papules, coalescing into symmetrical cobblestone plaques, with a predilection for the neck of postmenopausal women. The condition develops slowly, in months to years, and, although clinically similar to PXE, it is devoid of any systemic implication, being only of esthetic concern. The etiology is unknown, but it is speculated that there is a multifactorial pathogenesis, with the contribution of intrinsic aging, exposure to ultraviolet radiation, abnormal elastogenesis, and genetic factors. The diagnosis is confirmed by histopathology, with special stains specific for elastic fibers revealing loss of elastic plexus in the papillary dermis and the presence of melanophages. Its prevalence is probably underestimated, which reinforces the importance of better clinical and histologic identification to distinguish it from inherited PXE and to avoid extensive systemic investigations and unnecessary medical visits for the patient.
弹性假黄瘤样乳头真皮弹力纤维松解症（PDE）是一种获得性、罕见的弹性组织疾病，表现为多发性无症状、非滤泡性黄色或正常色素丘疹，融合成对称的鹅卵石斑块，好发于绝经后妇女的颈部。该病进展缓慢，历时数月至数年，尽管临床上与弹性假黄瘤相似，但无任何系统性影响，仅与美观有关。病因不明，但推测存在多种发病机制，包括内在老化、紫外线辐射、异常弹力生成和遗传因素的共同作用。诊断通过组织病理学确认，弹力纤维特殊染色显示乳头真皮中弹性丛缺失和黑色素细胞存在。其患病率可能被低估，这强调了更好的临床和组织学识别的重要性，以将其与遗传性弹性假黄瘤区分开来，并避免对患者进行广泛的系统检查和不必要的医疗访问。
